Ella Speichinger, MD
Christine H. Holschneider, MD
The incidence of surgical disease is the same in pregnant and nonpregnant patients. A total of 1.5–2% of all pregnancies undergo nonobstetric surgical intervention. Presenting symptoms of surgical diseases are often similar in pregnant and nonpregnant patients. The most common surgical disorders in pregnancy are appendicitis, cholecystitis, intestinal obstruction, adnexal torsion, trauma, and cervical and breast disease. Limited imaging can be performed during pregnancy if results would significantly alter management. The second trimester is the preferred time for nonurgent surgery. Surgery should not be delayed in any trimester if systemic infection or severe disease is suspected, as this is associated with higher risk to mother and fetus. Whenever possible, regional anesthesia should be performed. Pregnancy does not change prognosis, which depends largely on the extent of disease at diagnosis. A multidisciplinary approach with maternal–fetal medicine, surgery, anesthesia, and neonatology during treatment planning is invaluable to ensure optimal outcomes for both the mother and fetus.
Surgical interventions other than caesarean section are performed in 1.5–2.0% of all pregnancies. Altered anatomy and physiology and potential risks to the mother and fetus make diagnosis and management of surgical disorders more difficult during pregnancy. The interests of mother and fetus are best served by the obstetrician’s active participation with the anesthesiologist, neonatologist, and general surgeon throughout the mother’s diagnosis and management of a nonobstetric surgical disorder. It is imperative that the obstetrician be well informed about the ways in which surgical disorders influence pregnancy and vice versa, the risks of diagnostic and therapeutic procedures to the fetus, and appropriate management of preterm labor in the immediate postoperative period.
Surgical disorders can be either incidental to or directly related to the pregnancy. Diagnostic evaluation requires gentle, sensitive elicitation of physical signs, at times without sophisticated diagnostic aids that involve risk to the developing fetus. Good judgment regarding the timing, methods, and extent of treatment is important. In the absence of peritonitis, visceral perforation, or hemorrhage, surgical disorders during gestation generally have little effect on placental function and fetal development.
MATERNAL CONSIDERATIONS
Pregnancy is accompanied by physiologic and anatomic changes that alter the evaluation and management of the surgical patient. The 30–50% increase in plasma volume during pregnancy affects cardiac output and may alter drug distribution and laboratory test results. Red cell mass increases but not as much as the plasma volume, resulting in a slight physiologic anemia. Colloid osmotic pressure is decreased during pregnancy. Increased interstitial fluid is seen as mild edema, particularly in the lower extremities. Systemic vascular resistance decreases during pregnancy. Systolic and diastolic blood pressures characteristically drop during the early second trimester, with a gradual return to baseline by term. Functional pulmonary residual capacity decreases due to limitation of diaphragmatic excursion. Minute ventilation increases due to increased tidal volume and respiratory rate. A compensated mild respiratory alkalosis exists. Increased renal blood flow is evidenced by increased glomerular filtration rate and decreased serum creatinine and blood urea nitrogen values. Gastrointestinal motility is diminished, resulting in delayed gastric emptying and constipation. The enlarging uterus may alter the anatomic relation among the different organs. When the patient is in the supine position, the enlarged uterus may compress the inferior vena cava and result in the hypotensive vena cava compression syndrome.
ACOG Committee Opinion No. 284: Nonobstetric surgery in pregnancy. Obstet Gynecol 2003;102:431. PMID: 12907126.
Price LC, Slack A, Nelson-Piercy C. Aims of obstetric critical care management. Best Pract Res Clin Obstet Gynaecol 2008;22:775–799. PMID: 18693071.
FETAL CONSIDERATIONS
Optimal care of the pregnant surgical patient requires that potential hazards to the fetus be minimized. This includes risks associated with the maternal disease, diagnostic radiologic procedures, therapeutic drugs, anesthesia, and surgery. Assessment of the risks and benefits to the mother is relatively easy but less so for the fetus because of its relative inaccessibility.
A number of imaging modalities are available for diagnosis during pregnancy, including ultrasound (US), magnetic resonance imaging (MRI), computed tomography (CT), and x-ray.
Radiation Exposure
Although no definite harmful effects from the diagnostic use of US and MRI during pregnancy are reported, exposure to radiation is associated with fetal risks. Limited diagnostic CT or x-ray procedures can be undertaken with care in the pregnant patient. The fetus should be shielded whenever possible. The risk of adverse fetal effects associated with radiation exposure changes with gestational age and is related to the radiation dose to the fetus. These risks fall principally into 2 categories: teratogenicity and carcinogenicity. For example, within 2 weeks of fertilization, the embryo is most susceptible to implantation failure. If implantation was not affected, teratogenicity is extremely unlikely. Before 8 weeks, the fetus is at risk for radiation-induced growth restriction. At 8–15 weeks, the embryo is the most susceptible to mental retardation, with an approximately 4% risk on exposure at 10 cGy and 60% at 150 cGy. Teratogenic effects are unlikely in embryos older than 20 weeks. The most common fetal defects seen with direct fetal irradiation of 10 cGy or more are microcephaly, mental retardation, intrauterine growth restriction, and eye abnormalities. Current evidence suggests no increased structural or developmental fetal risk with radiation doses less than 5 cGy. The second set of concerns exists regarding in utero radiation exposure and its association with an increase in childhood neoplasms. The risk appears to be dose-related. Natural background fetal radiation exposure is estimated at 0.1 cGy. Fetal exposure to 2–5 cGy is estimated to translate into a relative risk of 1.5–2.0 for fatal childhood cancer, recognizing that the absolute risk is still very low (2 in 2000). Table 25–1 outlines estimates of fetal radiation exposure with various diagnostic procedures.
Table 25–1. Estimated fetal radiation exposure from common diagnostic radiologic procedures.
Exposure to Contrast
Traditionally, it has been recommended that use of iodine contrast be avoided during pregnancy. Although in vivo animal studies have not documented teratogenesis, ionic contrast when instilled directly into the amniotic sac during amniofetography has provoked neonatal hypothyroidism. Intravenous use of nonionic contrast media has been reported to have no effect on neonatal thyroid function. Given the existing data, the American College of Radiology states that definitive conclusions regarding the safety of intravascular iodinated contrast use cannot be made and recommends its use in pregnancy only if necessary. Before administration, it is important to weigh information that will be obtained by the addition of iodine contrast and to obtain informed consent. It is essential that infants whose mothers have received iodine contrast have thyroid function tested postnatally.
Gadolinium crosses the placenta and is thought to be excreted by the fetal kidneys into the amniotic fluid. There is a theoretical concern for toxicity related to persistence of free gadolinium. The American College of Radiology discourages its use in pregnancy, stating it should be used only if absolutely essential and following informed consent. However, the US Food and Drug Administration classifies gadolinium as a class C drug, and the European Society of Radiology states that based on available evidence, the use of gadolinium in pregnancy appears to be safe.
In summary, routine preoperative radiologic procedures are not justified. However, if clinical management of the pregnant patient would be significantly altered based on the findings of a judiciously performed radiologic procedure, the limited fetal exposure risk is generally warranted. Gadolinium should be considered only if the diagnostic information gained from the study is essential for the health of the mother. When multiple diagnostic images are required, consultation with a dosimetry expert may be helpful in calculating estimated fetal dose.
Surgical and Anesthesia Risks
Fortunately, most women who require surgery during pregnancy are otherwise relatively healthy and undergo an uneventful postoperative course. Generally, the safety of nonobstetric surgery in pregnancy and general anesthesia has been well established. Nevertheless, some increased risks are associated with surgery and anesthesia during pregnancy, and purely elective surgical procedures should be postponed until after pregnancy. Individual studies have suggested a possible increase in neural tube defects; other registries have documented adverse effects including low birth weight, prematurity, intrauterine growth restriction, and early neonatal death, but they are thought to correlate with the underlying condition that necessitates the surgical procedure. A review of 54 articles documenting pregnancy outcomes of nonobstetric surgical procedures between 1966 and 2002 found a low rate of miscarriage, congenital abnormalities, and preterm birth, but firm conclusions cannot be made, as no suitable control group existed. Thus, despite the general safety of anesthetic agents in pregnancy, some concern remains regarding teratogenicity in early gestation, and all but truly emergent surgery should be postponed until the second trimester. The second trimester is the preferred surgery time over the third trimester, as the risk of preterm labor and spontaneous abortion is lowest at that time. Whenever possible, regional anesthesia should be performed. No known reproductive toxicity is associated with currently used local anesthetic agents at recommended dose ranges. Short-term postoperative use of narcotic analgesic agents, frequently in combination with acetaminophen or nonsteroidal anti-inflammatory drugs before 32 weeks, generally appears to produce no adverse fetal effects.
Because intrauterine asphyxia is a major risk to the fetus consequent to maternal surgery, monitoring and maintaining maternal oxygen-carrying capacity, oxygen affinity, arterial PO2, and placental blood flow throughout the preoperative, operative, and postoperative periods are important. For gestations greater than 18 weeks, attention should be given to providing uterine displacement to prevent venocaval compression when the patient is in the supine position. Supplemental oxygen administration and maintenance of circulating volume also assist fetal oxygenation. A reduction in maternal blood pressure can lead directly to fetal hypoxia. Greater reductions in uteroplacental perfusion by direct vascular constriction and an increase in uterine tonus are noted in association with the use of vasopressors, especially those with predominantly α-adrenergic activity. Ephedrine, with its peripheral beta-adrenergic effect, produces much less vasospasm and has traditionally been the vasopressor of choice in the pregnant patient, especially for treating hypotensive complications of regional anesthesia. More recent data find phenylephrine a good alternative with no untoward fetal effects.
To ensure fetal well-being, continuous electronic fetal heart rate monitoring should be used when maternal surgery is performed after 24 weeks as long as the monitoring device can function outside the sterile surgical field. In some cases, intraoperative electronic fetal monitoring may be considered in previable pregnancies to facilitate maternal positioning and oxygenation. At minimum, if the fetus is considered to be viable, electronic fetal and contraction monitoring is advised both before and after the procedure to assess fetal well-being and to evaluate for signs of preterm labor.
The severity of the inflammatory response associated with the disease requiring surgery appears to be more important in determining pregnancy outcome than is the use of anesthesia or the surgical procedure itself. Premature labor does not appear to be a common result of procedures such as exploratory laparotomy unless visceral perforation and peritonitis are encountered or a low pelvic procedure is performed with significant uterine manipulation. Prophylactic use of tocolytics in this setting is controversial. Often, a single dose of a beta-adrenergic agent such as terbutaline is sufficient to arrest contractions. Use of indomethacin may be preferred if significant inflammation is present; however, patency of fetal ductus arteriosus and amniotic fluid index should be monitored if used for more than 48 hours. If possible, uterine activity should be monitored after surgery to detect preterm labor and allow for early intervention. There are no studies to guide the decision to administer prophylactic glucocorticoids at the time of nonobstetric surgery. In the absence of systemic maternal infection, glucocorticoids should be considered if the gestation is between 24 and 34 weeks to reduce perinatal morbidity and mortality if preterm delivery occurs. Additionally, there is no literature to support progesterone supplementation perioperatively unless the corpus luteum is removed before 12 weeks of gestation.
Chen MM, Coakley FV, Kaimal A, Laros RK. Guidelines for computed tomography and magnetic resonance imaging use during pregnancy and lactation. Obstet Gynecol 2008;112:333–340. PMID: 18669732.
Cohen-Kerem R, Railton C, Oren D, et al. Pregnancy outcome following nonobstetric surgical intervention. Am J Surg 2005;190:467–473. PMID: 16105538.
McCollough CH, Schueler BA, Atwell TD, et al. Radiation exposure and pregnancy: When should we be concerned? Radiographics 2007;27:909–917.
The American College of Obstetricians and Gynecologists. Committee Opinion: Nonobstetric surgery during pregnancy. Obstet Gynecol 2011;117:420–421. PMID: 21252774.
DIAGNOSTIC CONSIDERATIONS
History
Clues to the cause of surgical disorders in pregnancy are often found in a careful review of the medical history. The stage and status of pregnancy are also relevant.
Pain
Pain is the most prominent symptom encountered with acute abdominal conditions complicating pregnancy. Generalized abdominal pain, guarding, and rebound strongly suggest peritonitis secondary to bleeding, exudation, or leakage of intestinal contents. Peritoneal signs can be less obvious in pregnancy as the uterus may displace the infected organ from contact with the parietal peritoneum. Cramping with lower central abdominal pain suggests a uterine disorder. Lower abdominal pain on either side suggests torsion, rupture, or hemorrhage of an ovarian cyst or tumor. Right lower or midabdominal pain suggests appendicitis. Disorders of the descending and sigmoid colon with left lower quadrant pain are infrequently encountered because of the relatively young age of obstetric patients. Midabdominal pain early in gestation suggests an intestinal origin. Upper abdominal pain is often related to the liver, spleen, gallbladder, stomach, duodenum, or pancreas. Constipation is a common problem but is rarely associated with other symptoms.
Other Symptoms
Abdominal pain associated with nausea and vomiting after the first trimester usually suggests a gastrointestinal disorder. Nausea and vomiting associated with the inability to pass gas or stool points to an intestinal obstruction. Diarrhea is seldom encountered in association with acute surgical problems except as a symptom of recurrent ulcerative colitis.
Syncope associated with pain and signs of peritoneal irritation usually indicate an acute abdominal emergency with rupture of a viscus, ischemia, or hemorrhage. A temperature of 38 °C (100.4 °F) or greater suggests infection, which may be localized by other clinical findings. Fever can also be associated with later stages of visceral necrosis in the cases of torsion or intestinal ischemia. Vaginal bleeding usually points to an intrauterine or cervical problem. Urinary tract infection is often accompanied by urinary frequency and urgency.
Physical Examination
The patient with an acute abdomen should undergo careful assessment of the reproductive organs, and her vital signs and general condition should be noted as well as the presence or absence of bowel sounds, abdominal rigidity or rebound tenderness, and the presence or absence of a mass. The fewest possible number of abdominal examinations should be gently performed without haste and with adequate explanation, using the flat part of the hand and starting in an asymptomatic area.
Laboratory Studies
Several laboratory studies routinely used in the evaluation of surgical disease have altered normal values during pregnancy; they are discussed where appropriate for the specific disease entity. The white blood cell count is considered elevated if the value is above 16,000/μL in any trimester. An interval of several hours usually passes between onset of hemorrhage and detection of lowered hematocrit values.
ANESTHESIA
The type of anesthesia is determined primarily by the planned surgical procedure. All general anesthetic agents cross the placenta but are not thought to be teratogenic. Regional anesthesia minimizes fetal exposure but may either not be appropriate for the surgical procedure or the maternal condition. If general anesthesia is anticipated, it is important to consider the physiologic changes in pregnancy, such as increased oropharyngeal swelling and decreased glottic opening, which can complicate intubation and ventilation. Obesity and preeclampsia can exacerbate these difficulties, leading to aspiration, failed intubation, and subsequent maternal and fetal hypoxia. Despite this, successful general anesthesia optimizes maternal and fetal oxygenation and reduces intraoperative uterine irritability. During either regional or general anesthesia, liberal oxygen supplementation should be employed to avoid maternal and fetal hypoxia.
Cheek TG, Baird E. Anesthesia for nonobstetric surgery: Maternal and fetal considerations. Clin Obstet Gynecol 2009;52:535–545. PMID: 20393407.
Lynch J, Scholz S. Anaesthetic-related complications of caesarean section. Zentralbl Gynakol 2005;127:91–95. PMID: 15800840.
PRINCIPLES OF SURGICAL MANAGEMENT
Delay in diagnosis and performance of surgery is the factor primarily responsible for increased maternal morbidity rates and perinatal loss, especially with maternal abdominal trauma. Immediate surgical exploration is generally indicated in the presence of unmistakable signs of peritoneal irritation, evidence of strangulating intestinal obstruction with possible gangrene, or intraabdominal hemorrhage. In subacute conditions, care should be used in deciding to proceed with surgery. Surgery that is not urgent and can be delayed is best deferred until the second trimester or puerperium. Surgical techniques usually are not altered because of the pregnancy. Essentials of good preoperative care include adequate hydration, availability of blood for transfusion, and appropriate preoperative medication that will not decrease oxygenation for mother and fetus. Gestational age, uterine size, the specific surgical disorder, and the anticipated type of surgery to be performed are important factors in the selection of the abdominal incision. At operation, the least extensive procedure necessary should be performed with as little manipulation of the uterus as possible. Unless an obstetric indication is present or the uterus interferes with performance of a procedure, it usually is best not to perform a caesarean delivery during an abdominal operation.
Postoperative care depends on the gestational age and the operation performed. For patients whose gestation has reached viability, electronic monitoring of fetal heart rate and uterine activity should be continued in the immediate postoperative period, with staff capable of performing an emergent caesarean section readily available. Oversedation and fluid or electrolyte imbalance are to be avoided. Encouragement of early maternal activity and resumption of normal food intake are generally recommended.
THROMBOPROPHYLAXIS
Both pregnancy and surgery increase the risk of venous thromboembolism (VTE). Beginning in early pregnancy, vitamin K coagulation factors and type-1 plasminogen activator inhibitor increase, whereas protein S levels decrease. Surgery increases venous stasis and causes endothelial damage. Both mechanical and pharmacologic thromboprophylaxis reduce the incidence of symptomatic VTE. Pneumatic compression devices have few contraindications and should be considered for all pregnant women undergoing surgery. Pharmacologic thromboprophylaxis should be weighed against the patient’s risk of thrombosis versus perioperative bleeding. Pregnant women who have an inherited or acquired thrombophilia, prolonged immobilization, past history of VTE, malignancy, age older than 35 years, multiple gestation, systemic illness, or obesity are at increased risk for VTE.
Bates SM, Greer IA, Pabinger I, et al. Venous thromboembolism, thrombophilia, antithrombotic therapy, and pregnancy: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Ed.). Chest2008;133:844S–886S. PMID: 18574280.
Dargaud Y, Rugeri L, Vergnes MC, et al. A risk score for the management of pregnant women with increased risk of venous thromboembolism: A multicentre prospective study. Br J Haematol 2009;145:825–835. PMID: 19388925.
LAPAROSCOPY IN PREGNANCY
Over the past 2 decades, laparoscopy has been increasingly used during pregnancy in the management of a variety of surgical disorders, most commonly for the exploration and treatment of adnexal masses, for appendectomy, and for cholecystectomy, but also for more technical surgical procedures such as nephrectomy, splenectomy, or retroperitoneal lymphadenectomy. The major advantages are decreased postoperative morbidity, less pain, shorter hospital stay, and postoperative recovery time. There may also be benefits specific to pregnancy, such as less uterine manipulation and better visualization around the enlarged uterus. Possible drawbacks are the risk of injury to the pregnant uterus, technical difficulty with exposure because of the enlarged uterus, increased carbon dioxide absorption, and decreased uterine blood flow secondary to excessive intraabdominal pressure. Knowledge of the short- and long-term effects of laparoscopy on the human fetus is limited. Laparoscopy has been performed during all trimesters. During the first half of pregnancy, the risks inherent to the laparoscopic procedure do not appear to be substantially increased compared with the risks in nonpregnant patients. The largest population-based study of 2181 laparoscopies and 1522 laparotomies before 20 weeks’ gestation did not find any differential impact of laparoscopy versus laparotomy on perinatal outcome. Fetal loss appears to be associated with maternal disease severity rather than with operative technique. Risks of uterine injury can be mitigated by placing a supraumbilical port 6 cm above the fundus using the open (Hasson’s) technique; others recommend inserting the Veres needle in the left upper quadrant. Trocar placement under ultrasound guidance has also been described. Because there is uncertainty regarding the possible adverse effects of pneumoperitoneum and potential for fetal acidosis, attempts should be made to keep intraabdominal pressure between 8 and 12 mm Hg and not to exceed 15 mm Hg. Intraoperative CO2 monitoring should be used to maintain end-tidal CO2 between 32 and 34 mm Hg.
Corneille MG, Gallup TM, Bening T, et al. The use of laparoscopic surgery in pregnancy: Evaluation of safety and efficacy. Am J Surg 2010;200:363–367. PMID: 20800715.
Guidelines Committee of SAGES. Guidelines for diagnosis, treatment, and use of laparoscopy for surgical problems during pregnancy. Surg Endosc 2008;22:849–861. PMID: 18288533.
GASTROINTESTINGAL DISEASES & DISORDERS
Early accurate diagnosis of serious abdominal surgical disease during pregnancy is more difficult for the following reasons: (1) altered anatomic relationships, (2) impaired palpation and detection of nonuterine masses, (3) depressed symptoms, (4) symptoms that mimic the normal discomforts of pregnancy, and (5) difficulty in differentiating surgical and obstetric disorders. In general, elective surgery should be avoided during pregnancy, but operation should be performed promptly for definite or probable acute disorders. The approach to surgical problems in pregnant or puerperal patients should be the same as in nonpregnant patients, with prompt surgical intervention when indicated. The risk of inducing labor with diagnostic laparoscopy or laparotomy is low, provided unnecessary manipulation of the uterus and adnexa is avoided. Spontaneous abortion is most likely to occur if surgery is performed before 14 weeks’ gestation or when peritonitis is present.
APPENDICITIS
ESSENTIALS OF DIAGNOSIS
Symptoms include abdominal pain, usually localized to right lower or mid quadrant, with nausea, vomiting, and/or anorexia.
Patients may have an elevated white blood cell count with a left shift.
Ultrasound or CT scan demonstrates enlargement or inflammation of the appendix.
Clinical Findings
Acute appendicitis is the most common extrauterine complication of pregnancy for which surgery is performed. Suspected appendicitis accounts for nearly two-thirds of all nonobstetric exploratory celiotomies performed during pregnancy; most cases occur in the second and third trimesters.
Appendicitis occurs in 0.1–1.4 per 1000 pregnancies. Although the incidence of disease is not increased during gestation, rupture of the appendix occurs 2–3 times more often during pregnancy secondary to delays in diagnosis and operation. Maternal and perinatal morbidity and mortality rates are greatly increased when appendicitis is complicated by peritonitis.
A. Symptoms & Signs
The diagnosis of appendicitis in pregnancy is challenging. Signs and symptoms often are atypical and not dramatic. Right lower quadrant or middle quadrant pain almost always is present when acute appendicitis occurs in pregnancy but may be ascribed to so-called round ligament pain or urinary tract infection. In nonpregnant women, the appendix is located in the right lower quadrant (65%), in the pelvis (30%), or retrocecally (5%). Traditionally it was taught that pregnancy displaces the appendix upwardly. However, some retrospective studies suggest that there is only minimal appendiceal migration throughout pregnancy.
The most consistent clinical symptom encountered in pregnant women with appendicitis is vague pain on the right side of the abdomen, although atypical pain patterns abound. Muscle guarding and rebound tenderness are much less demonstrable as gestation progresses. If pain changes from localized tenderness to a more diffuse nature, appendiceal perforation should be suspected. Rectal and vaginal tenderness are present in 80% of patients, particularly in early pregnancy. Nausea, vomiting, and anorexia usually are present, as in the nonpregnant patient. During early appendicitis, the temperature and pulse rate are relatively normal. High fever is not characteristic of the disease, and 25% of pregnant women with appendicitis are afebrile.
B. Laboratory Findings
The relative leukocytosis of pregnancy (normal 6000–16,000/μL) clouds interpretation of infection. Although not all patients with appendicitis have white blood cell counts above 16,000/μL, at least 75% show a left shift in the differential. Urinalysis may reveal significant pyuria (20%) as well as microscopic hematuria. This is particularly true in the latter half of pregnancy, when the appendix migrates closer to the retroperitoneal ureter.
C. Imaging
In the nonpregnant patient, CT of the abdomen with and without contrast has become an important tool aiding in the diagnosis of appendicitis. To avoid the risk of radiation to the fetus, US has a distinct role as the first-line imaging modality in pregnancy (Fig. 25–1). Graded compression ultrasonography has been found to have a high positive predictive value but average sensitivity in diagnosing appendicitis. MRI is helpful in further aiding the diagnosis in patients for whom sonographic findings are nondiagnostic. If US is nondiagnostic and MRI is not available, CT may be appropriate. A noncompressible appendix on ultrasound is abnormal, whereas MRI or CT may demonstrate an enlarged, fluid-filled appendix with or without a fecalith. An appendix measuring > 6 mm should be considered abnormal.
Figure 25–1. Acute appendicitis diagnosed by graded compression ultrasonography. A: Longitudinal image of the right lower quadrant demonstrates the appendix as a blind-ending, thick-walled tubular structure. B: Transverse images with and without compression demonstrate this structure remains at least 6 mm thick with compression. (Images used, with permission, from Dr. Maitraya Patel, Olive View-UCLA Medical Center, Sylmar, CA.)
Differential Diagnosis
Pyelonephritis is the most common misdiagnosis in patients with acute appendicitis in pregnancy. The differential diagnosis of appendicitis includes gastrointestinal disorders such as gastroenteritis, small bowel obstruction, diverticulitis, pancreatitis, mesenteric adenitis, diverticulitis, and neoplasm; also possible are gynecologic and obstetric disorders such as ruptured corpus luteum cyst, adnexal torsion, ectopic pregnancy, placental abruption, early labor, round ligament syndrome, chorioamnionitis, degenerating myoma, or salpingitis.
Complications
Postoperative preterm labor has been reported to occur in 25% of second-trimester and as high as 50% of third-trimester patients. Most preterm deliveries occur within the first postoperative week. Perinatal loss may occur in association with preterm labor and delivery or with generalized peritonitis and sepsis, occurring in 0–1.5% of uncomplicated appendicitis cases. Twenty-five percent of pregnant women with appendicitis will progress to perforation; this risk is greatest when surgery is delayed more than 24 hours. With appendiceal rupture, fetal loss rates are reportedly as high as 30%, and maternal mortality rates as high as 4% are reported. This is of particular concern because appendiceal rupture occurs most frequently in the third trimester.
Treatment
Immediate surgical intervention is indicated once the diagnosis of appendicitis is made. In the setting of active labor, the surgery should be performed immediately postpartum. Delaying treatment increases the risk of perforation, which in turn increases the risk of fetal loss. Under appropriate conditions, laparoscopic appendectomy may be as safe as open appendectomy. A systematic review of 637 cases of laparoscopic appendectomy showed a significantly higher rate of fetal loss (6% vs. 3.1%), though equal or lesser rates of preterm delivery compared with open appendectomy. Large series report a negative surgical exploration rate between 13% and as high as 55%, likely due to the many processes that may mimic appendicitis in pregnancy. When the appendix appears normal at laparotomy, careful exploration for other nonobstetric and obstetric conditions is important.
Treatment of nonperforated acute appendicitis complicating pregnancy is appendectomy. A single dose of preoperative prophylactic antibiotics should be routinely given. In the setting of perforation, peritonitis, or abscess formation, broad-spectrum intravenous antibiotics should be continued until culture and sensitivities can narrow antibiotic choice. If drainage is necessary for generalized peritonitis, drains should be placed transabdominally and not transvaginally. During the first trimester, a transverse incision at McBurney’s point or over the area of maximal tenderness is generally considered appropriate. If the diagnosis is not certain, a vertical midline incision can be made. Laparoscopy is an alternate surgical approach used with increasing frequency, especially in the first half of pregnancy. In the late second or third trimester, a muscle-splitting incision centered over the point of maximal tenderness usually provides optimal appendiceal exposure. As a rule, appendiceal disease is managed and the pregnancy is left alone. A Smead-Jones combined mass and fascial closure with secondary wound closure 72 hours later may be advisable when the appendix is gangrenous or perforated or in the presence of peritonitis or abscess formation.
Induced abortion is rarely indicated. Depending on the gestational age and expert neonatal care available, abdominal delivery occasionally is performed when peritonitis, sepsis, or a large appendiceal or cul-de-sac abscess occurs. Data are limited, so making definitive recommendations regarding the use of prophylactic tocolytics is difficult. It appears unnecessary in uncomplicated appendicitis but may be appropriate with advanced disease. Caution is indicated because of reports that tocolytics are associated with an increased risk of pulmonary edema in women with sepsis. Labor that follows shortly after surgery in the late third trimester should be allowed to progress because it is not associated with a significant risk of wound dehiscence. At times, the large uterus may help wall off an infection, which after delivery may become disrupted, leading to an acute abdomen within hours postpartum.
Prognosis
Better fluid and nutritional support, use of antibiotics, safer anesthesia, prompt surgical intervention, and improved surgical technique have been important elements in the significant reduction of maternal mortality from appendicitis during pregnancy. Similarly, the fetal mortality rate has significantly improved over the past 50 years. Perinatal loss is low and maternal mortality negligible in cases of uncomplicated appendicitis, but increase significantly in the setting of peritonitis or appendiceal rupture. Thus it is imperative to avoid surgical delay. A higher negative laparotomy or laparoscopy rate may be an acceptable trade-off for a lower fetal mortality rate.
Oto A, Ernst RD, Shah R, et al. Right-lower-quadrant pain and suspected appendicitis in pregnant women: Evaluation with MR imaging–initial experience. Radiology 2005;234:445–451. PMID: 15591434.
Pates JA, Avendanio TC, Zaretsky MV, McIntire DD, Twickler DM. The appendix in pregnancy: Confirming historical observations with a contemporary modality. Obstet Gynecol 2009;114:805–808. PMID: 19888038.
Walsh CA, Tang T, Walsh SR. Laparoscopic versus open appendectomy in pregnancy: A systematic review. Int J Surg 2008;6:339–344. PMID: 18342590.
CHOLECYSTITIS & CHOLELITHIASIS
ESSENTIALS OF DIAGNOSIS
Patients usually present with abdominal pain in the right upper quadrant or epigastric region.
Serum laboratories may demonstrate an elevation in the white blood cell count and/or elevated liver enzymes.
Ultrasound of the right upper quadrant of the abdomen is usually diagnostic in these cases.
Clinical Findings
Gallbladder disease is one of the most common medical conditions and the second most common surgical disorder during pregnancy. Gallstones are responsible for 90% of cholecystitis in Western countries; parasitic infections are a less common cause. Acute cholecystitis occurs in 1 in 1600 to 1 in 10,000 pregnancies. Well-described risk factors for cholelithiasis are age, female sex, fertility, obesity, and family history. It has been estimated that at least 3.5% of pregnant women harbor gallstones. Multiparas are at increased risk of gallbladder disease. Both progesterone and estrogen increase bile lithogenicity; progesterone decreases gallbladder contractility. These changes are seen by the end of the first trimester of pregnancy.
A. Symptoms and Signs
Signs and symptoms are similar to those seen in the non-pregnant state and include anorexia, nausea, vomiting, dyspepsia, and intolerance of fatty foods. Biliary tract disease may cause right upper quadrant, epigastric, right scapular, shoulder, and even left upper quadrant or left lower quadrant pain that tends to be episodic. Biliary colic attacks often are of acute onset, seemingly are triggered by meals, and may last from a few minutes to several hours. Fever, right upper quadrant pain, and tenderness under the liver with deep inspiration (Murphy’s sign) are often present in patients with acute cholecystitis. In severe cases the patient may have mild jaundice or appear septic.
B. Laboratory Findings
An elevated white blood cell count with an increase in immature forms is seen with acute cholecystitis. Aspartate transaminase (AST) and alanine transaminase (ALT) levels are often increased. Modest increases in the alkaline phosphatase and bilirubin levels are anticipated very early in cholecystitis or common duct obstruction. However, a more characteristic pattern of relatively normal AST and ALT levels with elevated alkaline phosphatase and bilirubin levels is generally found after the first day of the attack. These changes are not diagnostic and do not signify common bile duct stone or obstruction alone, but when present they serve to support the diagnosis. Elevated lipase and amylase support the diagnosis of an associated pancreatitis.
C. Imaging
US findings of gallbladder stones, a thickened gallbladder wall, fluid collection around the gallbladder, a dilated common bile duct, or even swelling in the pancreas are suggestive of cholelithiasis and cholecystitis. The diagnostic accuracy of US for detecting gallstones in pregnancy is 95%, making it the diagnostic test of choice.
Differential Diagnosis
The major diagnostic difficulty imposed by pregnancy is differentiating between cholecystitis and appendicitis. In addition to its association with gallstones, cholecystitis can be infectious secondary to Salmonella typhi or parasites. A number of other lesions of the biliary tract occur rarely during gestation, including choledochal cysts, which are seen as a spherical dilatation of the common bile duct with a very narrow or obstructed distal end. Associated pancreatitis may be present. Severe preeclampsia with associated right upper quadrant abdominal pain and abnormal liver function tests; hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome; acute fatty liver of pregnancy; and acute viral hepatitis are in the differential diagnosis. The presence of proteinuria, nondependent edema, hypertension, and sustained increases in AST and ALT levels compared with alkaline phosphatase level are clinical and laboratory features usually associated with preeclampsia. Peptic ulcer disease, myocardial infarction, and herpes zoster also have overlapping symptoms.
Complications
Secondary infection with enteric flora such as Escherichia coli, Klebsiella, or Streptococcus faecalis complicates one-fifth of cases. Pancreatitis may frequently accompany cholecystitis during pregnancy. Removal of the gallbladder and gallstones may be preferred over conservative medical therapy when pancreatitis is concurrent, as it is associated with fetal loss in 3–20% of pregnant patients. Other uncommon complications of cholecystitis during gestation are retained intraductal stones, gangrenous cholecystitis, galbladder perforation with biliary peritonitis, cholecystoenteric fistulas, and ascending cholangitis.
Treatment
The initial management of symptomatic cholelithiasis and cholecystitis in pregnancy is nonoperative with bowel rest, intravenous hydration, correction of electrolyte imbalances, and analgesics. If antibiotics are not routinely given, they should be administered if no improvement is seen in 12–24 hours or if systemic symptoms are noted. This therapy results in resolution of acute symptoms in most patients. Surgical intervention is indicated if symptoms fail to improve with medical management, for recurrent episodes of biliary colic, and for complications such as recurrent cholecystitis, choledocholithiasis, and gallstone pancreatitis. Because recurrence rates for symptomatic biliary disease during pregnancy may be as high as 60–92%, active surgical management, especially in the second trimester, has been advocated in recent years. Recent literature has demonstrated the safety of open and laparoscopic cholecystectomy during pregnancy. Endoscopic retrograde cholangiopancreatography (ERCP) with endoscopic sphincterotomy may be an alternative for selected patients with common bile duct stones. Operative therapy for uncomplicated cholecystitis performed during the second and third trimesters does not appear to be associated with an appreciable increase in morbidity and mortality rates or fetal loss.
Prognosis
The outcomes for mother and fetus after uncomplicated gallbladder surgery are excellent. Morbidity and mortality rates increase with maternal age and extent of disease.
Andriulli A, Loperfido S, Napolitano G, et al. Incidence rates of post-ERCP complications: A systematic survey of prospective studies. Am J Gastroenterol 2007;102:1781–1788. PMID: 17509029.
Date RS, Kaushal M, Ramesh A. A review of the management of gallstone disease and its complications in pregnancy. Am J Surg 2008;196:599–608. PMID: 18614143.
Jackson H, Granger S, Price R, et al. Diagnosis and laparoscopic treatment of surgical diseases during pregnancy: An evidence-based review. Surg Endosc 2008;22:1917–1927. PMID: 18553201.
ACUTE PANCREATITIS
ESSENTIALS OF DIAGNOSIS
Patients usually present with epigastric pain that may radiate to the back.
Serum amylase and lipase levels are elevated, findings diagnostic of pancreatitis.
Ultrasound may demonstrate an enlarged pancreas and fluid within the peritoneal cavity.
Clinical Findings
The incidence of acute pancreatitis in pregnancy reportedly ranges from 1 in 1000 to 1 in 5000 deliveries. Pancreatitis occurs most frequently in the third trimester and puerperium. The mortality rate associated with acute pancreatitis may be higher during pregnancy because of delayed diagnosis. The ultimate cause of pancreatitis is the presence of activated digestive enzymes within the pancreas. Many cases of pancreatitis are idiopathic. As in the nonpregnant state, cholelithiasis is the most commonly identified cause, followed by alcoholism, lipidemia, viral and drug-induced pancreatitis, familial pancreatitis, structural abnormalities of the pancreas or duodenum, severe abdominal trauma, vascular disease, and preeclampsia-associated pancreatitis.
A. Symptoms & Signs
Gravidas with pancreatitis usually present with severe, steady epigastric pain that often radiates to the back in general approximation of the retroperitoneal location of the pancreas. Often exacerbated by food intake, its onset may be gradual or acute and is frequently accompanied by nausea and vomiting. During gestation, patients may present primarily with vomiting with little or no abdominal pain. Although physical examination is rarely diagnostic, several findings of note may be present, including a low-grade fever, tachycardia, and orthostatic hypotension. The latter finding may be present with hemorrhagic pancreatitis in addition to Cullen’s sign (periumbilical ecchymosis) and Turner’s sign (flank ecchymosis). Epigastric tenderness and ileus also may be present.
B. Laboratory Findings
The cornerstone of diagnosis is the determination of serum amylase and lipase levels. Interpretation of serum amylase levels in pregnancy is difficult at times because of the physiologic, up to 2-fold rise in serum amylase level during pregnancy. A laboratory serum amylase level that is more than 2 times above the upper limit of normal suggests pancreatitis. However, an elevated serum amylase level is not specific for pancreatitis because cholecystitis, bowel obstruction, hepatic trauma, or a perforated duodenal ulcer can cause similar serum amylase level elevations. Serum amylase levels usually return to normal within a few days of an attack of uncomplicated acute pancreatitis. Serum lipase level is a pancreas-specific enzyme and lipase elevation can guide the differential diagnosis toward pancreatitis. In severe pancreatitis, hypocalcemia develops as calcium is complexed by fatty acids liberated by lipase.
C. Imaging
Sonographic examination may demonstrate an enlarged pancreas with a blunted contour, peritoneal or peripancreatic fluid, and abscess or pseudocyst formation. Ultrasonography allows for the diagnosis of cholelithiasis, which may be etiologic for pancreatitis. The mere presence of gallstones, however, does not demonstrate etiologic relevance. US is also helpful for evaluating other differential diagnostic considerations.
Differential Diagnosis
Especially pertinent in the differential diagnosis of pancreatitis in pregnancy are hyperemesis gravidarum, preeclampsia, ruptured ectopic pregnancy (often with elevated serum amylase levels), perforated peptic ulcer, intestinal obstruction or ischemia, acute cholecystitis, ruptured spleen, liver abscess, and perinephric abscess.
Complications
Although all of the usual complications of pancreatitis can occur in parturients, there is no special predisposition to complications during pregnancy. Acute complications include hemorrhagic pancreatitis with severe hypotension and hypocalcemia, acute respiratory distress syndrome, pleural effusions, pancreatic ascites, abscess formation, and liponecrosis.
Treatment
Treatment of acute pancreatitis is aimed at correcting any underlying predisposing factors and treating the pancreatic inflammation. In pregnancy, acute pancreatitis is managed as it is in the nonpregnant state, except that nutritional supplementation is considered at an earlier point in treatment to protect the fetus, either via nasojejunal tube feeding of an elemental formula or total parenteral nutrition. Treatment is primarily medical and supportive, including bowel rest with or without nasogastric suction, intravenous fluid and electrolyte replacement, and parenteral analgesics. Antibiotics are reserved for cases with evidence of an acute infection. In patients with gallstone pancreatitis, consideration is given to early cholecystectomy or ERCP after the acute inflammation subsides. In pancreatitis not caused by gallstones, surgical exploration is reserved for patients with pancreatic abscess, ruptured pseudocyst, severe hemorrhagic pancreatitis, or pancreatitis secondary to a lesion that is amenable to surgery. Pregnancy does not influence the course of pancreatitis.
Prognosis
Maternal mortality rates as high as 37% were reported before the era of modern medical and surgical management. Respiratory failure, shock, need for massive fluid replacement, and severe hypocalcemia are predictive of disease severity. Most recent single-institution series reflect a reduced maternal mortality rate of less than 1%; perinatal death ranges from 3–20%, depending on severity of disease. Preterm labor appears to occur in a high proportion of patients with acute pancreatitis in later gestation.
Eddy JJ, Gideonsen MD, Song JY, Grobman WA, O’Halloran P. Pancreatitis in pregnancy. Obstet Gynecol 2008;112:1075–1081. PMID: 18978108.
Luminita CS, Steidl ET, Rivera-Alsina ME. Acute hyperlipidemic pancreatitis in pregnancy. Am J Obstet Gynecol 2008;98:e57. PMID: 18359475.
PEPTIC ULCER DISEASE
ESSENTIALS OF DIAGNOSIS
Patients typically present with epigastric discomfort.
Endoscopy is diagnostic of peptic ulcer disease.
Pathogenesis
Pathogenesis
Pregnancy appears to be somewhat protective against the development of gastrointestinal ulcers, as gastric secretion and motility are reduced and mucus secretion is increased. Close to 90% of women with known peptic ulcer disease experience significant improvement during pregnancy, but more than half will have recurrence of symptoms within 3 months postpartum. Thus peptic ulcer disease occurring as a complication of pregnancy or diagnosed during gestation is encountered infrequently, although the exact incidence is unknown. Infection with Helicobacter pylori is associated with the development of peptic ulcer disease.
Clinical Findings
Signs and symptoms of peptic ulcer disease in pregnancy can be mistakenly dismissed as being a normal part of the gravid state. Dyspepsia is the major symptom of ulcers during gestation, although reflux symptoms and nausea are also common. Epigastric discomfort that is temporally unrelated to meals is often reported. Abdominal pain might suggest a perforated ulcer, especially in the presence of peritoneal signs and systemic shock. Endoscopy is the diagnostic method of choice for these patients if empiric clinical therapy, including lifestyle and diet modifications, antacids, antisecretory agents, and treatment for H pylori when positive, fail to improve symptoms.
Differential Diagnosis
Gastroesophageal reflux disease and functional or nonulcer dyspepsia are common occurrences in pregnancy and may result in symptoms very similar to those of peptic ulcer disease. Biliary colic, chronic pancreatitis, Mallory-Weiss tears, and irritable bowel syndrome must also be considered. In recent years the diagnosis of persistent hyperemesis gravidarum has been linked to H pylori infection. Women with jaundice, persistent symptoms of dysphagia or odynophagia, weight loss, occult gastrointestinal bleeding, a family history of gastrointestinal cancers or unexplained anemia postpartum should be assessed for malignancy. A history of prior gastric surgery should prompt an evaluation for surgical complications. Ulcer perforation should be suspected in the setting of sudden, severe, diffuse abdominal pain followed by tachycardia and peritoneal signs.
Complications
Fewer than 100 parturients with complications of peptic ulcer disease, such as perforation, bleeding, and obstruction, have been reported. Most of these cases have occurred in the third trimester of pregnancy. Gastric perforation during pregnancy has an exceedingly high mortality rate, partly because of the difficulty in establishing the proper diagnosis. Other causes of upper gastrointestinal bleeding in pregnancy are reflux esophagitis and Mallory-Weiss tears. Surgical intervention is indicated for significant bleeding ulcerations. In patients requiring surgery for complicated peptic ulcers late in the third trimester, concurrent caesarean delivery may be indicated to enhance operative exposure of the upper abdomen and to prevent potential fetal death or damage from maternal hypotension and hypoxemia.
Treatment
Dyspepsia during pregnancy first should be treated with dietary and lifestyle changes, supplemented with antacids or sucralfate. When symptoms persist, H2-receptor antagonists or, in severe cases, proton pump inhibitors can be used. Administration of triple-drug therapy for H pylori during pregnancy is controversial; because complications from peptic ulcer disease during pregnancy are low and there are theoretical concerns of teratogenicity from treatment, it is often deferred until postpartum. Empiric treatment of H pylori without testing is not recommended.
Chen YH, Lin HC, Lou HY. Increased risk of low birthweight, infants small for gestational age, and preterm delivery for women with peptic ulcer. Am J Obstet Gynecol 2010;202:164. e1–164.e8. PMID: 20113692.
Engemise S, Oshowo A, Kyei-Mensah A. Perforated duodenal ulcer in the puerperium. Arch Gynecol Obstet 2009;279: 407–410. PMID: 18642012.
Talley N, Vakil N. Guidelines for the management of dyspepsia. Practice Parameters Committee of the American College of Gastroenterology. Am J Gastroenterol 2005;100:2324–2337. PMID: 16181387.
ACUTE INTESTINAL OBSTRUCTION
ESSENTIALS OF DIAGNOSIS
Patients typically present with the classic triad of abdominal pain, vomiting, and obstipation.
The diagnosis is confirmed with abdominal x-ray series.
Pathogenesis
Intestinal obstruction is an infrequently encountered complication of pregnancy that is estimated to occur in approximately 1–3 of every 10,000 pregnancies. However, it is the third most common nonobstetric reason for laparotomy during pregnancy (following appendicitis and biliary tract disease). It occurs most commonly in the third trimester. The most common causes of mechanical obstruction are adhesions (60%) and volvulus (25%), followed by intussusception, hernia, and neoplasm. Volvulus is much more prevalent during pregnancy; the risk is greatest when uterine size rapidly changes (eg, second trimester and immediately postpartum).
Clinical Findings
The same classic triad of abdominal pain, vomiting, and obstipation is observed in pregnant and nonpregnant women with intestinal obstruction. Pain may be diffuse, constant, or periodic, occurring every 4–5 minutes with small-bowel obstruction or every 10–15 minutes with large-bowel obstruction. Bowel sounds are of little value in making an early diagnosis of obstruction, and tenderness to palpation typically is absent with early obstruction. Vomiting occurs early with small-bowel obstruction. Guarding and rebound tenderness are observed in association with strangulation or perforation. Late in the course of disease, fever, oliguria, and shock occur as manifestations of massive fluid loss into the bowel, acidosis, and infection. The classic findings of bowel ischemia include fever, tachycardia, localized abdominal pain, marked leukocytosis, and metabolic acidosis. Unfortunately, most laboratory abnormalities arise once bowel ischemia has progressed to bowel necrosis.
The diagnosis usually is confirmed by radiologic studies, which should be obtained when intestinal obstruction is suspected. A single abdominal series (upright and supine abdominal film) is nondiagnostic in up to 50% of early cases, but serial films usually reveal progressive changes that confirm the diagnosis. Volvulus should be suspected when a single, grossly dilated loop of bowel is seen. A volvulus primarily occurs at the cecum but may also be seen at the sigmoid colon. Occasionally, more extensive radiologic imaging is indicated, given the high risk of fetal death with delayed treatment.
Differential Diagnosis
The diagnosis of hyperemesis gravidarum in the second and third trimesters should be viewed with caution and made only after gastrointestinal causes of the symptoms including pancreatitis have been excluded. Mesenteric ischemia, adynamic ileus of the colon, and acute colonic pseudo-obstruction (Ogilvie’s syndrome) are included in the differential diagnosis but are rarely seen during pregnancy.
Prognosis
Intestinal obstruction in pregnancy is associated with a maternal mortality rate of 6%, often secondary to infection and irreversible shock. Early diagnosis and treatment are essential for an improved outcome. Perinatal mortality is approximately 20% and usually results from maternal hypotension and resultant fetal hypoxia and acidosis.
Treatment
The management of bowel obstruction in pregnancy is essentially no different from treatment of nonpregnant patients. The cornerstones of therapy are bowel decompression, intravenous hydration, correction of electrolyte imbalances, and timely surgery when indicated. The patient’s condition must be rapidly stabilized. The amount of fluid loss often is underestimated and may be 1–6 L by the time obstruction is identified on a scout film. Aggressive hydration is needed to support both the mother and the fetus. A nasogastric tube should be placed. Colonoscopy has been used successfully in the reduction of volvulus averting laparotomy. Ogilvie’s syndrome can be managed with bowel rest, rehydration, and a rectal tube for large bowel decompression. Surgery is mandatory if perforation or gangrenous bowel is suspected or when the patient’s symptoms do not resolve with medical management. A vertical midline incision on the abdomen provides the best operative exposure and can be extended as needed. Surgical principles for intraoperative management apply similarly to pregnant and nonpregnant patients. Caesarean delivery is performed first if the large uterus prevents adequate exposure of the bowel in term pregnancies or if indicated obstetrically. The entire bowel should be examined carefully because there may be more than 1 area of obstruction or limited bowel viability.
Dietrich CS 3rd, Hill CC, Hueman M. Surgical diseases presenting in pregnancy. Surg Clin North Am 2008;88:403–419. PMID: 18381120.
Parangi S, Levine D, Henry A, Isakovich N, Pories S. Surgical gastrointestinal disorders during pregnancy. Am J Surg 2007;193:223–232. PMID: 17236852.
INFLAMMATORY BOWEL DISEASE
(See Chapter 29, Gastrointestinal Disorders in Pregnancy, for more details.)
ESSENTIALS OF DIAGNOSIS
Crohn’s disease is one subcategory, characterized by insidious onset; episodes of low-grade fever, diarrhea, and right lower quadrant pain; and perianal disease with abscess and fistulas formed. Radiographic evidence of ulceration, structuring, or fistulas of the small intestine or colon. May involve any segment of the gastrointestinal tract from the mouth to the anus.
Ulcerative colitis is the other subcategory of inflammatory bowel disease, manifesting with bloody diarrhea, lower abdominal cramps, fecal urgency, anemia, and low serum albumin. It is diagnosed with sigmoidoscopy and only involves the colon.
Clinical Findings
Inflammatory bowel disease (IBD) (Crohn’s disease and ulcerative colitis) often affects women in their childbearing years; however, initial presentation of IBD during pregnancy is rare. IBD presents with crampy abdominal pain and diarrhea stained with blood or mucus. More rarely the patient has weight loss and fevers.
Differential Diagnosis
Because some of the early IBD symptoms are found in normal pregnancies, diagnosis can be delayed, leading to poorer outcome. Initial evaluation should begin with ultrasound, which can also evaluate gallbladder, pancreas, and adnexa. Bowel wall thickening or abscess formations may be seen. Gold standard for diagnosis is endoscopy with biopsy, which can be safely performed during pregnancy.
Treatment
Initial management includes dietary modifications or bulking agents. Other medications that have been safely used in pregnancy are sulfasalazine, prednisone, and occasionally antibiotics. Patients receiving sulfasalazine should be given folate supplementation because sulfasalazine inhibits its absorption. Patients taking corticosteroids should receive stress-dose steroids during delivery or in surgery. Safety data of immunosuppressant use such as cyclosporine and anti–tumor necrosis factor-α in pregnancy are limited, but these agents have been used for persistent flares. Surgery during pregnancy is indicated for intestinal obstruction, megacolon, perforation, hemorrhage, abscess formation, and failed medical management. Delivery route should be based on obstetric indications except for patients who have active perianal disease; those with an ileal pouch and anastomosis may consider caesarean section to prevent injury to the anal sphincter.
Prognosis
The impact of IBD on pregnancy outcomes is controversial, though in general maternal and fetal outcomes are improved if IBD is in remission before conception. Postpartum flare is more common in Crohn’s disease than ulcerative colitis.
Ilnyckyj A. Surgical treatment of inflammatory bowel diseases and pregnancy. Best Pract Res Clin Gastroenterol 2007;21:819–834. PMID: 17889810.
Reddy D, Murphy SJ, Kane SV, et al. Relapses of inflammatory bowel disease during pregnancy: In-hospital management and birth outcomes. Am J Gastroenterol 2008;103:1203–1209. PMID: 18422816.
HEMORRHOIDS
ESSENTIALS OF DIAGNOSIS
Patients with hemorrhoids typically present with complaints of painless bleeding, prolapse, pain, pruritus, and/or fecal soilage.
Hemorrhoids are visible on physical examination or anoscopy.
Pathogenesis
Pregnancy is the most common cause of symptomatic hemorrhoids. Approximately 9–35% of pregnant and postpartum women suffer from hemorrhoids. Higher incidences of constipation, increased blood volume, and venous congestion secondary to the enlarging uterus contribute to hemorrhoid formation.
Clinical Findings
Patients with hemorrhoids typically present with complaints of painless bleeding, prolapse, pain, pruritus, and/or fecal soilage. On physical examination, hemorrhoids are visualized as a protrusion into or out of the anal canal. Internal hemorrhoids may require anoscopy for visualization.
Treatment
The current management approach to hemorrhoid disease is conservative, with simple outpatient treatment preferred, particularly during pregnancy and the puerperium. Medical therapy with dietary changes, avoidance of excessive straining, fiber supplementation, stool softeners, and hemorrhoidal analgesics often is the only requirement for nonthrombosed hemorrhoids. Often 6 weeks or longer are needed to perceive improvement. If conservative treatments fail, rubber-band ligation, infrared coagulation, or sclerotherapy appear to be safe during pregnancy. Hemorrhoidectomy is the best means of definitive therapy for hemorrhoidal disease but is rarely necessary during pregnancy. It should be considered postpartum if the patient continues to fail to respond to conservative measures, if hemorrhoids are severely prolapsed and require manual reduction, or if associated pathology such as ulceration, severe bleeding, fissure, or fistula is present. Thrombosis or clots in the vein lead to severe symptoms. If thrombosed external hemorrhoids remain tender and persist despite conservative treatment, surgical excision under local anesthesia is preferred over clot extraction, as this results in a high rate of clot recurrence.
Longo SA, Moore RC, Canzoneri BJ, Robichaux A. Gastrointestinal conditions during pregnancy. Clin Colon Rectal Surg 2010;23:80–89. PMID: 21629625.
SPONTANEOUS HEPATIC & SPLENIC RUPTURE
ESSENTIALS OF DIAGNOSIS
Patients with spontaneous hepatic or splenic rupture typically present with severe abdominal pain and the rapid onset of shock.
Pathogenesis
intraabdominal hemorrhage during pregnancy has diverse causes, including trauma, preexisting splenic disease, and preeclampsia–eclampsia. Often, the exact cause cannot be determined preoperatively. Spontaneous hepatic rupture may be associated with severe preeclampsia–eclampsia. (See Chapter 26, Hypertension in Pregnancy, for more details on preeclampsia–eclampsia.)
Clinical Findings
Spontaneous hepatic or splenic rupture is usually manifested by severe abdominal pain and shock, with thrombocytopenia and low fibrinogen levels.
Treatment
Exploratory celiotomy in conjunction with aggressive transfusion of blood products including packed red blood cells, fresh-frozen plasma, and platelets should be undertaken immediately, as this has been associated with improved survival rates.
Bleeding from a lacerated or ruptured spleen does not cease spontaneously and requires immediate surgical attention. Evidence of a hemoperitoneum on imaging studies or a hemorrhagic peritoneal lavage in association with a falling hematocrit level and abdominal pain establish the presence of a hemoperitoneum.
RUPTURED SPLENIC ARTERY ANEURYSM
ESSENTIALS OF DIAGNOSIS
Women with ruptured splenic artery aneurysm typically present with epigastric, left upper quadrant, or left shoulder pain.
The diagnosis is usually confirmed by abdominal radiography or ultrasound.
Pathogenesis
Autopsy data suggest that splenic artery aneurysm occurs in 0.1% of adults and appear to be more common in women. It is estimated that 6–10% of lesions will rupture, with portal hypertension and pregnancy being the main risk factors. Twenty-five to 40% of ruptures occur during gestation, especially in the last trimester, and are a major cause of intraperitoneal hemorrhage. Pregnant women who develop ruptured splenic artery aneurysm have a 75% mortality rate, with an even higher fetal mortality rate of up to 95%. Most patients with this condition are thought preoperatively to have placental abruption or uterine rupture.
Clinical Findings
Before rupture, the presenting symptoms may be completely absent or vague. The most common symptom is vague epigastric, left upper quadrant, or left shoulder pain. In approximately 25% of patients a 2-stage rupture is seen, with a smaller primary hemorrhage into the lesser sac, which may allow for temporary tamponade of the bleeding until complete rupture into the peritoneal cavity occurs, causing hemorrhagic shock. A bruit may be audible. A highly diagnostic finding on flat x-ray film of the abdomen is demonstration in the upper left quadrant of an oval calcification with a central lucent area. In stable clinical situations, angiography can provide positive confirmation and is the gold standard for diagnosis. In pregnancy, however, ultrasonography and pulsed-wave Doppler studies are preferred in order to minimize fetal radiation exposure.
Treatment
A splenic artery aneurysm in a woman of childbearing age should be treated in a timely manner, even during pregnancy, because of the increased risk of rupture and associated mortality. The elective operative mortality rate reportedly ranges between 0.5% and 1.3%.
He MX, Zheng JM, Zhang S, et al. Rupture of splenic artery aneurysm in pregnancy: A review of the literature and report of two cases. Am J Forensic Med Pathol 2010;31:92–94. PMID: 20032776.
Parangi S, Levine D, Henry A, Isakovich N, Pories S. Surgical gastrointestinal disorders during pregnancy. Am J Surg 2007;193:223–232. PMID: 17236852.
PELVIC DISEASES & DISORDERS
OVARIAN MASSES
ESSENTIALS OF DIAGNOSIS
Most ovarian masses during pregnancy present as an incidental finding on routine obstetrical ultrasound to evaluate the fetus.
Some women may experience pelvic pain or discomfort due to the mass.
Pathogenesis
The incidental finding of an adnexal mass in pregnancy has become more common with the routine use of ultrasonography. As many as 1–4% of pregnant women are diagnosed with an adnexal mass. The majority of the masses are functional or corpus luteum cysts and spontaneously resolve by 16 weeks’ gestation. More than 90% of unilateral, non-complex masses less than 5 cm in diameter that are noticed in the first trimester are functional and resolve spontaneously. Patients who undergo assisted reproduction present a special subgroup, as their ovaries frequently have ovarian cysts in the first trimester due to ovarian hyperstimulation. Pathologic ovarian neoplasms tend not to resolve. The most common pathologic ovarian neoplasms during pregnancy are benign cystic teratoma, serous or mucinous cystadenoma, and cystic corpus luteum. Of the adnexal masses that persist, 1–10% will be malignant.
Clinical Findings
Most adnexal masses discovered during pregnancy are found as incidental findings at time of ultrasound performed for evaluation of the fetus. Thus most women are asymptomatic for the ovarian mass. However, some women may experience pelvic pain or discomfort related to the mass.
Differential Diagnosis
Ovarian masses must be differentiated from lesions of the colon, pedunculated leiomyomas, pelvic kidneys, and congenital abnormalities of the uterus. If ultrasound cannot distinguish between leiomyoma and ovarian neoplasm, MRI can improve diagnostic precision.
Treatment
The 3 main reasons for advising surgery for an adnexal mass in pregnancy are the risks of rupture, torsion, and malignancy. Determination of the actual risk of rupture or torsion of a benign-appearing adnexal mass in pregnancy remains an unsettled issue. It is estimated that only approximately 2% of such masses will rupture during gestation, and the incidence of torsion in recent published series ranges from 0–15%. The challenge to the clinician is to weigh for each individual patient these risks against the risks of abdominal surgery during pregnancy, including miscarriage, rupture of membranes, and preterm labor. If adnexal masses diagnosed in the first trimester require surgery in pregnancy, it is generally advisable to perform the operation via laparotomy or laparoscopy in the second trimester unless signs or symptoms suggestive of torsion or highly aggressive malignancy indicate the need for more immediate intervention. Similarly, asymptomatic ovarian masses that are initially noted in the third trimester of pregnancy can be followed until the time of delivery or postpartum because the size of the uterus may present access problems and because preterm labor may be inadvertently induced.
The risk of malignancy can be largely gauged by the ultrasonographic characteristics of the mass. Ultrasonography usually facilitates delineation of the size and morphology of adnexal masses. If the mass is unilateral, mobile, and cystic, anaplastic elements are less likely and operation can be deferred.
Any adnexal lesion that is present after 14 weeks’ gestation, is growing in size on serial ultrasonographic evaluations, contains solid and complex components or internal papillae, is fixed, is surrounded by abdominal ascites, or is symptomatic warrants surgical exploration and pathologic diagnosis.
Marret H, Lhomme C, Lecuru F, et al. Guidelines for the management of ovarian cancer during pregnancy. Eur J Obstet Gynecol Reprod Biol 2010;149:18–21. PMID: 20042265.
Schmeler KM, Mayo-Smith WW, Peipert JF, Weitzen S, Manuel MD, Gordinier ME. Adnexal masses in pregnancy: Surgery compared with observation. Obstet Gynecol 2005;105: 1098–1103. PMID: 15863550.
Schwartz N, Timor-Tritsch IE, Wang E. Adnexal masses in pregnancy. Clin Obstet Gynecol 2009;52:570–585. PMID: 20393410.
Yen CF, Lin SL, Murk W, et al. Risk analysis of torsion and malignancy for adnexal masses during pregnancy. Fertil Steril 2009;91:1895–1902. PMID: 18359024.
TORSION OF THE ADNEXA
ESSENTIALS OF DIAGNOSIS
Adnexal torsion may be suspected in the woman with an adnexal mass who experiences the sudden onset of pelvic pain, usually severe in nature.
Ultrasound is useful in confirming the presence of an adnexal mass.
Laparoscopy or laparotomy is diagnostic for confirming the presence of torsion.
Pathogenesis
Torsion of the adnexa can involve the ovary, tube, and ancillary structures, either separately or together. The most common time for occurrence of adnexal torsion is between 6 and 14 weeks and in the immediate puerperium. Although torsion of normal adnexa has been described, it commonly is associated with a cystic neoplasm.
Clinical Findings
Symptoms include abdominal pain and tenderness that usually are sudden in onset and result from occlusion of the vascular supply to the twisted organ. Shock and peritonitis may ensue. Ultrasonography frequently demonstrates an adnexal mass and altered blood flow on Doppler studies. The diagnosis of torsion is ultimately made at surgery.
Treatment
Prompt operation is necessary to prevent tissue necrosis, pre-term labor, and potential perinatal death. Laparoscopy appears to be as safe as laparotomy for mother and fetus. The right ovary is involved more frequently than is the left ovary. Benign cystic teratomas and cystadenomas are the most common histologic findings in ovaries that have undergone torsion. Traditional thinking has been that ovarian cysts that have undergone torsion must not be untwisted before pedicle clamping because of the concern for potential fatal thromboembolic complications. However, recent series on both nonpregnant and pregnant patients demonstrate that adnexa that had undergone torsion can safely be untwisted, followed by the appropriate removal of the mass (eg, cystectomy). Oophoropexy may be performed to prevent future re-occurrence of the torsion. These adnexa are capable of recovering and being functional. Salpingo-oophorectomy can be reserved for the management of active bleeding or suspicious neoplasms. If cystectomy includes corpus luteum before 12 weeks’ gestation, progesterone supplementation should be initiated.
CARCINOMA OF THE OVARY
(See also Chapter 50, Premalignant and Malignant Disorders of the Ovaries and Oviducts.)
ESSENTIALS OF DIAGNOSIS
Symptoms of ovarian cancer are often vague and mimic some of the common symptoms associated with pregnancy.
Certain ultrasound findings, although not diagnostic of malignancy, can be suggestive of malignancy.
The diagnosis is confirmed on pathologic examination of surgically excised tissue.
Pathogenesis
Carcinoma of the ovary occurs in less than 0.1% of all gestations and has been encountered in all trimesters. Between 1% and 10% of all ovarian tumors complicating pregnancy are malignant. Consistent with the young age of the pregnant population, most malignant neoplasms are germ cell tumors (dysgerminoma, endodermal sinus tumor, malignant teratoma, embryonal carcinoma, and choriocarcinoma) and tumors of low malignant potential, but cystadenocarcinomas do occur. The majority of ovarian malignancies diagnosed in pregnancy are early-stage disease.
Clinical Findings
Symptoms of ovarian cancer are often vague and include bloating, increasing abdominal girth, and urinary frequency, findings that are common during pregnancy. Most cases of ovarian cancer diagnosed during pregnancy are found when an adnexal mass is seen during routine ultrasound to evaluate the fetus. Ultrasound findings suggestive of ovarian malignancy are a mass with a solid component or thick septations, evidence of flow within the solid component on color Doppler interrogation, and the presence of other masses in the pelvis suggestive of enlarged nodes.
Treatment
Solid and complex ovarian tumors with significant solid components discovered during pregnancy generally should be treated surgically because of the low but significant incidence of cancer (1–10%). The treatment of gestational ovarian cancer follows the same principles as that for the nonpregnant patient. If the adnexal mass is complex, laparoscopy may be considered if intact removal in an endoscopic bag is feasible. If an open approach is taken, the incision needs to be of sufficient size not only to remove the tumor intact, but also to properly explore the abdomen and to reduce uterine manipulation until the definitive surgical course of management is determined. Upon abdominal entry, peritoneal washings should be obtained and the contralateral ovary examined. If abnormal in appearance, biopsy should be performed; otherwise, routine biopsy is unnecessary. Adequate tissue should be obtained for histologic diagnosis on frozen section. If the tumor is benign, residual ovarian tissue should be conserved if possible.
If malignant, staging is performed. Conservative surgery is appropriate for an encapsulated tumor if no evidence of uterine or contralateral ovarian involvement is seen. In more advanced stages, the extent of surgery, including tumor debulking, will depend on gestational age and the patient’s wishes with regard to the pregnancy. Early termination of pregnancy does not improve outcome of ovarian cancer. In some cases, optimal surgical cytoreduction of the tumor to <1cm residual disease can be accomplished with the uterus and pregnancy left in situ. Neoadjuvant chemotherapy may offer an interim treatment for selected patients diagnosed at midgestation to allow for fetal maturity before extensive surgical cyto-reduction. Elevated tumor markers, such as α-fetoprotein, lactate dehydrogenase, β-human chorionic gonadotropin, and cancer antigen-125, during the preoperative workup of an adnexal mass must be interpreted with caution because pregnancy itself may cause an increase in these values.
LEIOMYOMAS
ESSENTIALS OF DIAGNOSIS
The diagnosis of leiomyomas in pregnancy is made based on ultrasound
Clinical Findings
Uterine leiomyomas are found in 0.1–3.9% of pregnancies. Most women with fibroids during pregnancy are asymptomatic. A degenerating leiomyoma or one undergoing torsion is characterized by acute abdominal pain with point tenderness over the site of the leiomyoma. Ultrasonography is of great value to document the location, size, and consistency of leiomyomas in a pregnant uterus. Cystic changes in leiomyomas are often visualized when clinical signs of degeneration are present.
Complications
A large cohort study of obstetric outcomes of women diagnosed ultrasonographically with uterine leiomyomas in pregnancy found an increased risk of caesarean delivery (mostly before labor onset), breech presentation, malposition, preterm delivery, placenta previa, and severe postpartum hemorrhage. Uterine leiomyomas may further complicate pregnancy by undergoing degeneration or torsion, or by causing mechanical obstruction of labor.
Treatment
Conservative treatment with analgesia, reassurance, and supportive therapy almost always is adequate. Occasionally, surgery during pregnancy is indicated for torsion of an isolated, pedunculated leiomyoma. With the exception of a pedunculated leiomyoma on a narrow stalk, myomectomy should not be performed during pregnancy because of the risk of uncontrollable hemorrhage.
Qidwai GI, Caughey AB, Jacoby AF. Obstetric outcomes in women with sonographically identified uterine leiomyomata. Obstet Gynecol 2006;107:376–382. PMID: 16449127.
Vergani P, Locatelli A, Ghidini A, et al. Large uterine leiomyomata and risk of cesarean delivery. Obstet Gynecol 2007;109:410–414. PMID: 17267843.
CANCER IN PREGNANCY
The incidence of cancer in pregnancy is approximately 1 in 1000. The most common malignancies diagnosed during pregnancy are cervical cancer (26%; see also Chapter 48, Premalignant and Malignant Disorders of the Uterine Cervix), breast cancer (26%), leukemias (15%), lymphomas (10%; see also Chapter 34, Hematologic Disorders in Pregnancy), and malignant melanomas (8%). Once cancer is diagnosed during pregnancy, a multidisciplinary team of maternal–fetal medicine specialists, oncologists, surgeons, and radiation oncologists can assist the patient in making difficult decisions regarding treatment timing and continued pregnancy.
CERVICAL CANCER
(See also Chapter 48, Premalignant and Malignant Disorders of the Uterine Cervix.)
ESSENTIALS OF DIAGNOSIS
The diagnosis of cervical cancer during pregnancy is usually made on the basis of cervical biopsy after abnormal pap smear or detection of cervical mass.
Pregnancy should not limit evaluation of abnormal cervical cytology or evaluation of a cervical mass.
Pathogenesis
Invasive cervical cancer complicates approximately 0.05% of pregnancies. Diagnosis during pregnancy occurs more frequently in areas in which routine prenatal cytologic examination is done. Significantly abnormal cervical cytology in pregnancy calls for colposcopic evaluation.
Clinical Findings
As is the case with nonpregnant patients, cervical cancer in pregnancy primarily presents with bleeding, but the diagnosis is frequently missed because the bleeding is assumed to be pregnancy-related rather than due to cancer. The possibility of cancer must be kept in mind, and if a cervical lesion or mass is seen during prenatal care, it must be biopsied.
Treatment
The diagnosis and management of invasive cervical cancer during pregnancy presents the patient and the physician with many challenges. Management is determined by the stage of the cancer, the gestational age, and the patient’s desires regarding the pregnancy. Pregnancy does not appear to affect the prognosis for women with cervical cancer and the fetus is not affected by the maternal disease, but may suffer morbidity from its treatment (eg, preterm delivery).
If the pregnancy is early and the disease is stage I–IIA, radical hysterectomy and therapeutic lymphadenectomy can be performed with the fetus left in situ, unless the patient is unwilling to terminate the pregnancy. Women at a gestational age closer to fetal viability or who decline termination may decide to continue the pregnancy after careful discussion regarding the maternal risks. Delivery in patients with cervical dysplasia and carcinoma in situ may be via the vaginal route. Patients with invasive cervical cancer should be delivered by caesarean section to avoid potential cervical hemorrhage and dissemination of tumor cells during vaginal delivery. A caesarean radical hysterectomy with therapeutic lymphadenectomy is the procedure of choice for patients with stage IA2–IIA2 disease once fetal maturity is established. As in the nonpregnant patient, radiation with concomitant chemotherapy is used for the treatment of more advanced disease. In the first trimester, irradiation may be carried out with the expectation of spontaneous abortion. In the second trimester, interruption of the pregnancy by hysterotomy before radiation therapy should be considered, although some experts advocate proceeding with immediate radiation treatment, again awaiting spontaneous evacuation of the uterus. In selected cases with locally advanced disease in which the patient declines pregnancy termination, consideration may be given to neoadjuvant chemotherapy in an effort to prevent disease progression during the time needed to achieve fetal maturity. Delivery should be by caesarean section. A lymphadenectomy can be performed at the same time. Postpartum the patient should receive chemoradiation following guidelines established for the nonpregnant patient.
BREAST CANCER
ESSENTIALS OF DIAGNOSIS
Women with breast cancer during pregnancy usually present with a breast mass or thickening.
The diagnosis is confirmed with biopsy.
Pregnancy should not limit a thorough evaluation of a breast mass.
Breast cancer is the most common cancer diagnosed in women in the United States. One of every 5 cases occurs in women younger than 45 years, and 2–5% of women with breast cancer are pregnant when the diagnosis is made. In the United States, the incidence of breast cancer in pregnancy is 3 per 10,000 live births. For this reason, careful breast examination should be performed during prenatal and postnatal care, and a family history should be obtained.
Clinical Findings
Pregnancy- and lactation-related changes in the breast increase the frequency and range of breast problems and make the diagnosis of breast cancer more difficult. A painless lump is the most common presentation of gestational breast cancer. Bloody nipple discharge may be a presenting symptom and requires workup. Any mass found by the patient or by the obstetrician should be fully evaluated without undue delay.
Differential Diagnosis
The differential diagnosis is broad and includes lactating adenoma, galactocele, milk-filled cyst, fibroadenoma, abscess, and cancer.
Complications
Management of the pregnant woman with breast carcinoma is difficult because it requires careful consideration of both mother and fetus. The general approach to treatment of breast cancer in pregnancy should be similar to that in nonpregnant patients and should not be delayed because of pregnancy.
Treatment
Initial management of the pregnant patient with a breast mass does not differ significantly from that for nonpregnant women. When a localized lesion is present, breast ultrasonography is the preferred first imaging modality during pregnancy. It is safe and helpful in distinguishing between cystic and solid masses. Although the sensitivity of mammography is diminished by the breast changes in pregnancy, the study still may be helpful for selected patients with inconclusive ultrasound examinations. With low-dose mammography and appropriate shielding, fetal radiation exposure is minimal. Nonetheless, it is generally recommended that the procedure be avoided during the first trimester. Gadolinium-enhanced breast MRI is an imaging technique that may be indicated in selected patients, although there is a paucity of data on MRI features of pregnancy-associated breast cancer. Cystic lesions should be aspirated and the fluid, if bloody, examined cytologically. Malignant cells are rarely found in nonbloody fluid. Fine-needle aspiration, core biopsy, or incisional biopsy can be used in some cases, but surgical excisional biopsy may be most appropriate for clinically suspicious or cytologically equivocal lesions. The increased vascularity of the breasts is associated with a higher rate of bleeding, and the lactating breast is prone to infectious complications, but neither pregnancy nor lactation appears to interfere with excisional biopsy in an outpatient setting.
Breast cancer is classified according to the tumor-node metastasis (TNM) staging system. If a pregnant woman has clinically positive nodes or suspicious symptoms, she should undergo radiographic staging of lung, liver, and bone; asymptomatic women with clinically node-negative early-stage breast cancer do not. This can be done with plain films with abdominal shielding, abdominal ultrasound, MRI, and radionuclide bone scans, which are thought to be safe in pregnancy.
Termination of pregnancy has not been shown to improve survival rates. Modified radical mastectomy is the preferred local management of pregnant patients with breast cancer, with the goal of avoiding the need for adjuvant radiation therapy. Radical mastectomy is well tolerated during pregnancy. Breast-conserving surgery, which must be combined with adjuvant radiation, is limited primarily to patients presenting in the late second and third trimester, for whom surgery is performed during pregnancy and radiation treatment postponed until after delivery. For a patient who desires breast-conserving surgery outside the third trimester, a detailed discussion is imperative. For management of the lymph nodes, axillary dissection has been the traditional treatment of choice. Sentinel lymph node biopsy can be safely performed during pregnancy using colloid, but outcomes data are limited.
Adjuvant chemotherapy is frequently recommended for premenopausal women with breast cancer. The recommendation of chemotherapy for a pregnant woman with breast cancer is a complex decision, but the indications for adjuvant chemotherapy for gestational breast cancer are generally the same as for the nonpregnant patient. Chemotherapy during the first trimester is contraindicated as it is associated with miscarriage and major malformations. The chemotherapeutic agents used in gestational breast cancer are generally the same as those used in nonpregnant patients; cyclophosphamide, doxorubicin, and fluorouracil have been given successfully during the second and third trimesters, with no measurable increase in congenital malformations but an increased incidence of prematurity and intrauterine growth restriction. Neoadjuvant chemotherapy may be a treatment option in select patients with locally advanced or metastatic gestational breast cancer. Chemotherapy should be stopped after 35 weeks to decrease the risk of neonatal neutropenia. Use of radiation and endocrine therapy should be avoided during pregnancy. Breastfeeding should be avoided during chemotherapy, hormone therapy, or radiation. There is no contraindication to breastfeeding after completion of therapy for breast cancer.
The results of treatment are much the same stage for stage as they are in nonpregnant patients, but pregnancy-associated breast cancers tend to be more advanced at diagnosis (larger tumor size, more frequently involved lymph nodes), resulting in an overall worse prognosis for this group of patients as a whole. Diagnostic delay is blamed for more advanced disease at diagnosis.
Prognosis
Subsequent pregnancies need not necessarily be discouraged after a suitable period of recuperation and observation, as subsequent pregnancy does not increase the risk of recurrence or death from breast cancer. For women who are breast cancer antigen (BRCA)-1 or BRCA-2 mutation carriers, there is no evidence that pregnancy decreases their breast cancer risk. With respect to fetal outcomes, there appears to be an increase in preterm birth among women receiving chemotherapy during pregnancy. There are no reported cases of metastatic disease to the fetus.
Amant F, Deckers S, Van Calsteren K, et al. Breast cancer in pregnancy: Recommendations of an international consensus meeting. Eur J Cancer 2010;46:3158–3168. PMID: 20932740.
Azim HA Jr, Pavldis N, Peccatori F. Treatment of the pregnant mother with cancer: A systematic review on the use of cytotoxic, endocrine, targeted agents and immunotherapy during pregnancy. Part II: Hematological tumors. Cancer Treat Rev 2010;36:110–121. PMID: 20018452.
Loibl S, Von Minckwitz G, Gwyn K, et al. Breast carcinoma during pregnancy: International recommendations from an expert meeting. Cancer 2006;106:237–246. PMID: 16342247.
Pereg D, Koren G, Lischner M. Cancer in pregnancy: Gaps, challenges and solutions. Cancer Treat Rev 2008;34:302–312. PMID: 18291591.
O’Meara AT, Cress R, Xing G, et al. Malignant melanoma in pregnancy: A population-based evaluation. Cancer 2005;103: 1217–1226. PMID: 15712209.
LYMPHOMAS AND LEUKEMIAS
(See also Chapter 34, Hematologic Disorders in Pregnancy.)
ESSENTIALS OF DIAGNOSIS
Most women diagnosed with Hodgkin’s lymphoma during pregnancy present with painless lymphadenopathy.
Biopsy is necessary to make a diagnosis of Hodgkin’s lymphoma.
Patients with leukemia may experience symptoms related to pancytopenia.
Leukemia may be suspected on the basis of circulating blasts on peripheral blood smear.
The diagnosis of leukemia is made on the basis of bone marrow biopsy.
Clinical Findings
The incidence of Hodgkin’s lymphoma in pregnancy is estimated to be 1 in 1000 to 1 in 6000 pregnancies, with non-Hodgkin’s lymphomas being significantly less frequent.
The typical presentation is painless adenopathy, and adequate biopsy is essential for diagnosis. Hodgkin’s lymphoma is curable even in advanced stages, and prognosis and stage distribution in pregnancy is comparable to those in the nonpregnant patient.
The incidence of leukemia in pregnancy is estimated at 1 in 100,000. The acute leukemias are more frequent. The diagnosis is made by examination of bone marrow samples, which can be safely performed during pregnancy. Acute leukemia places pregnant patients at very high risk for bleeding and infection complications.
Treatment
Approximately 70% of patients present with early-stage disease and can be treated with either single-agent chemotherapy or, in selected cases, modified supradiaphragmatic radiation.
Patients in early pregnancy presenting with extensive infradiaphragmatic disease, for which radiation therapy would be a significant component of curative therapy, should consider termination of pregnancy because of the associated significant teratogenic risks. Standard chemotherapy regimens appear moderately safe to use in the second and third trimester.
Treatment of acute leukemia should be started immediately after diagnosis for an attempt at cure. Depending on the gestational age, the management during pregnancy poses many challenges to the patient, her family, and the treating physicians. Chronic myelogenous leukemia can be treated with interferon throughout pregnancy. There are rare reports of lymphoma and leukemia metastases to the fetus.
MALIGNANT MELANOMA
ESSENTIALS OF DIAGNOSIS
Patients with melanoma usually present with a suspicious skin lesion.
The diagnosis is made by biopsy or excision of the lesion.
Approximately 30–35% of women diagnosed with melanoma are of childbearing age, and approximately 0.1–1% of female melanoma patients are pregnant.
Clinical Findings
Most present with stage I disease. Clinical signs of melanoma are the same in pregnant and nonpregnant women. Suspicious lesions are those that have changed in size, color, or shape; bleed; or are ulcerated. The diagnosis is made by excision, allowing for microstaging. Tumor thickness, tumor site, and presence of metastases are the most important prognostic factors.
Treatment/Prognosis
There has been long-standing controversy regarding the prognosis of pregnancy-associated melanoma, but more recent evidence suggests that patients with early primary lesions and wide surgical excision with appropriate margins have a prognosis comparable to that of their nonpregnant counterparts. Moreover, pregnancy termination has not been shown to improve survival. Data on higher stage melanoma diagnosed in pregnancy are limited. Malignant melanoma is the tumor that most frequently metastasizes to the placenta or fetus, accounting for more than half of all tumors with fetal involvement. Postpartum the placenta should be sent for pathologic evaluation.
CARDIAC DISEASE
ESSENTIALS OF DIAGNOSIS
Cardiac disease complicates 1–4% of all pregnancies in the United States.
Rheumatic and congenital heart disease constitute the majority of cases.
Treatment
Patients requiring cardiac surgery should undergo the procedure before becoming pregnant. Nevertheless, the rare patient will require cardiac surgery during pregnancy. Most available reports on cardiac surgery during pregnancy involve closed and open mitral valvuloplasties and mitral or aortic valve replacement.
Cardiac surgery can be performed with good results in pregnancy, although there is maternal and fetal risk. Operations should generally be performed early in the second trimester when organogenesis is complete and there is comparatively less hemodynamic burden and less risk of preterm labor than later in gestation.
Prognosis
Maternal mortality rates average 1–9%, related to the specific procedure performed and the patient’s preoperative cardiovascular status. Percutaneous balloon valvuloplasty should be considered the preferred technique to treat valvular disease during pregnancy. Perinatal mortality is expected in 2–10% of percutaneous balloon valvuloplasties. Perinatal loss is thought to be greater after open valvular or bypass surgery due largely to the nonpulsatile blood flow and hypotension associated with cardiopulmonary bypass. Close fetal surveillance by electronic heart rate and uterine contraction monitoring is essential during any cardiac surgical procedure, whether or not cardiopulmonary bypass is used. During bypass, blood flow to the uterus can be assessed indirectly by changes in the fetal heart rate, and alterations in flow can be made accordingly.
Weiss BM. Managing severe mitral valve stenosis in pregnant patients—Percutaneous balloon valvuloplasty, not surgery, is the treatment of choice. J Cardiothorac Vasc Anesth 2005;19:277–278. PMID: 15868549.
NEUROLOGIC DISEASE
(See also Chapter 33, Nervous System and Autoimmune Disorders in Pregnancy.)
ESSENTIALS OF DIAGNOSIS
The most common neurosurgical emergency to complicate pregnancy is intracranial hemorrhage.
Symptoms and signs of subarachnoid hemorrhage include headache, nausea and vomiting, stiff neck, photophobia, seizures, and a decreasing level of consciousness.
Pathogenesis
Intracranial hemorrhage during pregnancy is rare (1–5 per 10,000 pregnancies) but is associated with significant maternal and fetal mortality and serious neurologic morbidity in survivors. Cerebral aneurysm rupture is responsible for approximately 70% of intracranial hemorrhage; arteriovenous malformations (AVM) cause 25%, and the remaining cases are due to eclampsia, coagulopathy, trauma, and intracranial tumors. During pregnancy the risk of bleeding from an AVM that had not bled previously is 3.5%, which is close to the annual bleeding rate in the nonpregnant patient. However, mortality due to a bleeding AVM in pregnancy is higher (30%) than in the nonpregnant state (10%). The risk of rebleeding from an AVM in the same pregnancy is 27%. Intracranial hemorrhage with associated neurologic damage during pregnancy (limited capacity for decision making, persistent vegetative state, brain death) poses significant medical and ethical challenges in caring for the mother and fetus.
Most commonly, bleeding from an aneurysm occurs in the subarachnoid space, whereas bleeding from an AVM is located within the brain parenchyma.
Clinical Findings
Symptoms and signs of subarachnoid hemorrhage include headache, nausea and vomiting, stiff neck, photophobia, seizures, and a decreasing level of consciousness. The headache usually is very sudden in onset, whereas the headache associated with intraparenchymal bleeding usually is somewhat less severe and is slower in onset. Focal neurologic deficits may be absent in up to 40% of patients. CT or MRI confirm the diagnosis of an intracranial bleed. Cerebral angiography may be needed to identify and characterize an aneurysm or AVM.
Treatment
Early surgical or endovascular intervention after aneurysmal hemorrhage during pregnancy is associated with reduced maternal and fetal mortality. Neurosurgical centers with significant experience in cerebral aneurysm procedures have better outcomes than lower volume centers. For patients with AVM, the decision to treat the lesion during pregnancy is less clear but should follow the same guidelines that apply to nonpregnant patients.
Prognosis
Once the intracranial hemorrhage has been effectively treated, vaginal delivery can proceed according to obstetric indications. For women who have not received definitive treatment, delivery route is controversial. Maternal and fetal mortality appear the same with elective caesarean delivery or instrumental vaginal delivery under regional anesthesia. Aneurysm rupture has been reported during elective caesarean delivery, which is not considered protective. Regardless of delivery route, blood pressure control is imperative.
Qaiser R, Black P. Neurosurgery in pregnancy. Semin Neurol 2007; 27:476–481. PMID: 17940927.
TRAUMA
ESSENTIALS OF DIAGNOSIS
Automobile accidents are the most common nonobstetric cause of death during pregnancy.
The most common cause of fetal death is death of the mother.
Initial treatment focuses on immediate stabilization of the mother followed by evaluation of the fetus.
Pathogenesis
Approximately 7% of pregnancies are complicated by trauma, such as motor vehicle accidents (40%), falls (30%), direct assaults to the maternal abdomen (20%), and other causes (10%). Automobile accidents are the most common nonobstetric cause of death during pregnancy. The most common cause of fetal death is death of the mother. The second most common cause of fetal death is placental abruption. Pregnant women with traumatic injuries may be victims of physical abuse. Suicide also contributes to injury-related death. A pregnancy may increase family stress; therefore, the practitioner should be alert for signs of abuse and/or depression.
Treatment
The primary initial goal in treating a pregnant trauma victim is to stabilize the mother’s condition. Rapid hemorrhage can occur because approximately 600 mL of blood flow is directed to the uterus each minute. To optimize maternal and fetal outcome, an organized team approach to the pregnant trauma patient is essential. Maternal assessment and management are similar to those for the nonpregnant patient, keeping in mind the goal of protecting the fetus from unnecessary drug and radiation exposure. The fetus should be evaluated early during trauma assessment, and after fetal viability is reached, continuous fetal heart rate and uterine activity monitoring should be instituted, as long as it does not interfere with maternal resuscitative efforts. This information becomes critical when making management decisions for mother and fetus. Emergent caesarean section should be initiated if cardiopulmonary resuscitation has been unsuccessful after 4 minutes; this may provide the fetus with a greater chance for intact survival and allow for a successful maternal resuscitation. Caesarean delivery is also indicated if there is a nonreassuring fetal heart rate tracing in the setting of a stable mother, or if the enlarged uterus does not allow for repair of maternal injuries.
After immediate stabilization, the fetal heart rate and uterine contractions should be monitored for posttraumatic placental abruption. This usually occurs quite soon after the injury but rarely manifests as late as 5 days after trauma. Monitoring should continue for at least 4 hours after the trauma unless suspicious findings, including uterine contractions, vaginal bleeding, abdominal or uterine tenderness, postural hypotension, and fetal heart rate abnormalities, are noted. If any of these signs occur or if the trauma was severe, monitoring should be extended to 24–48 hours. Nonreassuring fetal heart rate patterns and fetal death can occur despite mild maternal trauma or pain. Ultrasound is helpful if abruption is visualized, but many are not. There is little evidence that the Kleihauer-Betke test reliably predicts significant fetomaternal hemorrhage, but it is recommended to determine if additional doses of Rho (D) immunoglobulin are needed for Rh-negative patients. Routine coagulation profiles are not clinically helpful in the setting of a stable mother.
Brown HL. Trauma in pregnancy. Obstet Gynecol 2009;114: 147–160. PMID: 19546773.
Katz V, Balderston K, Defreest M. Perimortem cesarean delivery: Were our assumptions correct? Am J Obstet Gynecol 2005;192:1916–1920. PMID: 15970850.
Muench MV, Canterino JC. Trauma in pregnancy. Obstet Gynecol Clin North Am 2007;34:555–583. PMID: 17921015.