Adam S. Brinkman
Dennis P. Lund
Presentation
An 8-day-old male infant presents to the emergency department accompanied by his mother with concerns for feeding intolerance, weight loss, and several episodes of emesis. He was a full-term baby, born to a G1P1 healthy 29-year-old female. The labor was prolonged without distress to the fetus, but meconium was present at birth. He was discharged home on day of life two, afebrile, tolerating breast milk, having regular bowel movements with a discharge weight of 6 lb 14 oz. Over the past 2 days, he has had increasing episodes of nonprojectile emesis, slightly green in color both during and in between feedings. The infant’s vital signs are temperature: 38.0, heart rate: 146, respiratory rate: 26, and systolic blood pressure: 86 mm Hg. He is alert but fussy and easily falls to sleep in his mother’s arms with gentle rocking. His current weight is 6 lb 10 oz.
Differential Diagnosis
When evaluating a newborn with feeding intolerance and bilious emesis, the working diagnosis should be malrotation with midgut volvulus until proven otherwise. The classic presentation of malrotation consists of a previously healthy infant with sudden-onset bilious emesis, feeding intolerance, and failure to thrive. The differential diagnosis is included in Table 1. A newborn with feeding intolerance, irritability, and bilious emesis should be approached as a surgical emergency as outcome is largely time dependent.
TABLE 1. Differential Diagnosis for Bilious Emesis in a Neonate
Workup
While the true incidence of malrotation remains unknown, autopsy studies estimate it as high as 1% of the total population. Greater than 75% of patients present within the first month of life and 90% within the first year. Key factors to note during the history include a thorough prenatal and delivery history, discharge weight, feeding habits and duration, recent emesis (including amount and color), bowel movements, as well as other congenital anomalies. Bilious emesis remains the cardinal sign of malrotation with midgut volvulus. Of particular note, abdominal pain and distention are not prominent early features of malrotation; however, as volvulus develops with associated mesenteric compromise, pain, irritability, and abdominal tenderness become apparent. As malrotation is a primary developmental problem, it is not surprising that other congenital anomalies are observed in 30% to 60% of patients with malrotation as illustrated in Table 2.
TABLE 2. Congenital Anomalies Associated with Malrotation
While majority of patients with malrotation are diagnosed within the first month of life, a significant number of patients continue through childhood, adolescence, and even adulthood without diagnosis. Individuals >2 years old with undiagnosed malrotation often report intermittent abdominal cramps, poor weight gain, and, in advanced cases, malnutrition.
Initial assessment should begin with evaluation of age-specific vital signs, fluid status, and interaction with providers. Fever, tachycardia, lethargy, and dehydration all indicate severe illness requiring urgent intervention. A scaphoid abdomen is not uncommon although signs of distention, abdominal wall erythema, and focal/diffuse peritonitis are worrisome signs and often represent intestinal ischemia. Initial management should begin with stabilization/resuscitation, pain control, nasogastric tube insertion for proximal decompression, basic laboratory studies, and foley catheter placement.
An unstable child suspected of having malrotation with an acute abdomen requires no imaging modalities, but rather prompt transportation to the operating room. In a stable patient, initial imaging should begin with plain radiographs of the abdomen evaluating for signs of bowel obstruction, “double bubble,” free intraperitoneal air, and gas/stool in the rectum.
The upper gastrointestinal series (UGIS) is the diagnostic test of choice to confirm the diagnosis of malrotation. Typically performed with barium or water-soluble contrast and placement of a nasogastric tube, contrast is slowly instilled into the stomach and its course through the foregut is monitored. Assistance of a pediatric radiologist and understanding of intestinal development will eliminate equivocal examinations and false interpretations, which occur in 15% of all upper GI studies performed for malrotation.
A normal UGIS illustrates a gentle C-shaped configuration of the duodenum, which crosses the midline and ascends to the level of the pylorus to form the duodenojejunal junction at the ligament of Treitz as illustrated in Figure 1. In patients with malrotation, the C-shape of the duodenum does not cross the midline and takes a caudal trajectory toward the right lower quadrant. In addition, the fourth portion of the duodenum does not rise to the level of the pylorus and the entire small bowel remains on the right side of the abdomen as illustrated in Figure 2. The key findings on the upper GI study include (1) abnormal position of the duodenojejunal junction, (2) corkscrew or spiral course of the distal duodenum and jejunum, and (3) location of the proximal jejunum in the right abdomen.
FIGURE 1 • Normal upper gastrointestinal series S, stomach; P, pylorus; LOT, Ligament of Treitz.
FIGURE 2 • Abnormal upper gastrointestinal series illustrating malrotation. S, stomach; P, pylorus.
In addition to plain radiographs and upper GI studies, cross-sectional imaging and ultrasound examination can also be utilized. In patients with malrotation, the superior mesenteric vein lies to the left of the superior mesenteric artery and as midgut volvulus develops a “whirlpool” sign can be visualized. The use of ultrasound in children is noninvasive and easy to perform, but unfortunately neither specific nor sensitive to diagnose malrotation alone. Atypical vascular orientation or a “whirlpool” sign should alert the surgeon to the possibility of malrotation, not used to confirm the diagnosis.
Diagnosis and Treatment
In a patient with tachycardia, hypotension, lethargy, peritonitis and acidosis emergent transportation to the operating room is needed. Preparation for operative intervention in patients without signs of systemic toxicity should include resuscitation, correction of electrolyte disturbances, and administration of intravenous antimicrobial therapy prior to skin incision. Patients diagnosed with malrotation, regardless of symptoms, should be scheduled for operative intervention, as they are susceptible to midgut volvulus. Several studies have attempted to determine the risk for developing midgut volvulus and have found it difficult to predict the risk based on radiographic imaging.
Surgical Approach
The surgical procedure for correction of malrotation, commonly known as Ladd’s procedure, was first described by Dr. William Ladd in 1932 and now performed both open and laparoscopically as illustrated in Figure 3. The open technique should be utilized in patients with acute midgut volvulus, peritonitis, hematemesis, abdominal wall erythema or by a surgeon with a lack of comfort with advanced laparoscopic techniques. The procedure consists of five key steps illustrated in Table 3. A right upper-quadrant transverse incision is the preferred incision although a midline minilaparatomy may also be considered to gain entrance to the peritoneal cavity. The bowel should be eviscerated and carefully inspected for evidence of ischemia or perforation, and detorsed in a counterclockwise direction, often requiring one to three complete turns to fully reduce the volvulus. To assure complete detorsion and division of all coloduodenal bands, the proximal jejunum is grasped in the left hand and ileocecal junction in the right hand. The entire small bowel should then hang below the surgeon’s hands in a “U” shape. The base of the mesentery should be widened by division of the anterior mesenteric leaflet. The small bowel is returned to the abdominal cavity and placed along the right lateral gutter and the colon along the left lateral gutter. Prophylactic appendectomy is also performed to prevent a future diagnostic dilemma. Potential pitfalls during the open Ladd’s procedure are listed in Table 3. It is unnecessary to fix the bowel to the lateral abdominal wall with sutures after proper Ladd’s procedure, as the bowel is rotated as far clockwise as possible and therefore not able to rotate any further. Several studies have clearly illustrated that fixation of the bowel to the lateral abdominal wall may actually increase the incidence of recurrent obstructions and offers no significant benefit to the patient.
FIGURE 3 • Ladd’s procedure.
TABLE 3. Key Technical Steps and Potential Pitfalls for the Ladd’s Procedure
In 1995, van der Zee and colleagues describe the first laparoscopic Ladd’s procedure. A laparoscopic approach is not only safe and effective for the correction of malrotation but also leads to decreased hospital stay, decreased intravenous narcotic requirements, and earlier enteral feeding in both children and adults. Traditionally, the surgeon stands at the foot of the operating room table and three or four 5-mm laparoscopic ports are placed to aid in dissection. The steps to the laparoscopic technique are identical to those of the open technique as described above. An extracorporeal appendectomy can be performed through a port site to avoid placing a larger port, which would be needed for the laparoscopic stapler. The most difficult steps during the laparoscopic approach include the entry into the peritoneal cavity, confirmation of malrotation by identification of abnormally positioned anatomy, and detorsion of the small bowel. The surgeon should always have a low threshold for conversion should there be uncertainty regarding anatomy, acute midgut volvulus with fragile bowel, or failure to resolve the malrotation.
Special Intraoperative Considerations
If the bowel appears healthy upon entrance to the abdominal cavity, Ladd’s procedure should be performed. However, if compromised bowel is encountered after the reduction of the volvulus, the surgeon has three main options including (1) resection of clearly necrotic bowel with primary anastomosis as illustrated in Figure 4, (2) return of compromised bowel to peritoneal cavity without resection, temporary abdominal closure with planned second-look laparatomy, and (3) resection of necrotic bowel with creation of stomas and planned additional resection if stomas become necrotic or elective stoma closure in several months. When the surgeon is faced with potential bowel resection, the ultimate goal should be preservation of the maximal length of intestine to prevent the serious complication of short gut syndrome. If repeat laparatomy is planned, it is recommended that a form of temporary abdominal closure be utilized to keep intra-abdominal pressure as low as possible.
Postoperative Management
With an uncomplicated patient, attention to providing appropriate analgesia, monitoring fluid status and resisting the urge for immediate enteral feeds is needed. Patients should have frequent vital sign measurements during the first 24 hours and in some cases may benefit from brief stay in an intensive care unit, especially if the patient had hemodynamic instability during the operative exploration or are <12 months old. Return of bowel function largely depends on degree and duration of preoperative obstruction and overall health of the patient, but generally enteral feeds can be started within 2 or 3 days. In general, patients who underwent laparoscopic Ladd’s procedure can be fed 1 or 2 days earlier as compared to the open technique.
On the other hand, patients who have undergone Ladd’s procedure for malrotation and were discovered to have had midgut volvulus requiring bowel resection are often critically ill and require admission to a critical care unit for support (Figure 4). Parenteral nutrition is necessary if the neonate is to survive. Repeat laparatomy should be accomplished within the first 24 to 48 hours. If however the patient begins to decompensate as evident by hemodynamic instability, persistent acidosis, or necrotic-appearing stomas, return to the operating room should be much sooner than initially planned. The true incidence of recurrent midgut volvulus after Ladd’s procedure is unknown but believed to be quite low, <1%. It must be kept in mind that these patients are susceptible to adhesive bowel obstruction as well.
FIGURE 4 • A 21-month-old male with malrotation and midgut volvulus.
TAKE HOME POINTS
· All children with bilious emesis should be suspected of having malrotation with midgut volvulus until proven otherwise.
· Older children and adults with malrotation often complain of chronic intermittent abdominal pain, cramps, nausea, and vomiting.
· A thorough history and physical examination are absolutely necessary with particular attention to vital signs, weight, fluid status, and evidence of peritonitis.
· A hypotensive neonate with bilious emesis and peritonitis should immediately be taken to the operating room for exploration.
· The upper gastrointestinal series with documentation of the duodenojejunal position is the imaging modality of choice.
· Ladd’s procedure is the procedure of choice for correction of malrotation.
· Laparoscopic Ladd’s procedure is safe and effective but requires advanced laparoscopic training, and one should have a low threshold to convert to open technique.
· Bowel resection, second-look laparotomy, and an open abdomen may be necessary depending on the viability of the bowel.
· Parenteral nutrition is necessary postoperatively in neonates who underwent significant bowel resection.
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