Case Files Pediatrics, (LANGE Case Files) 4th Ed.

CASE 49

A full-term 1-week-old boy presents with bilious vomiting and lethargy. His mother notes a normal prenatal course and uncomplicated delivery. On physical examination he is noted to have significant abdominal distension and blood in his diaper.

Image What is the most likely diagnosis?

Image What is the best management for this condition?

ANSWERS TO CASE 49: Malrotation

Summary: A full-term 1-week-old boy presents with bilious vomiting and lethargy. He is noted to have significant abdominal distension and blood in his diaper.

• Most likely diagnosis: Malrotation with volvulus.

• Best treatment: Surgical intervention to remove any necrotic bowel and to ensure adequate blood supply to surviving intestine.

ANALYSIS

Objectives

1. Know the presentation of malrotation with volvulus.

2. Understand the treatment of malrotation.

3. Be familiar with the differential diagnosis of acute abdominal pain in children.

Considerations

In this neonate with bilious emesis, a variety of etiologies are possible (Table 49-1). The clues to the diagnosis are bilious emesis due to intestinal obstruction, abdominal distension, blood per rectum, and lethargy. The most important next step is surgical intervention to prevent death and loss of viable intestine.

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Table 49-1 • COMMON ETIOLOGIES OF ACUTE ABDOMINAL PAIN IN INFANTS AND YOUNG CHILDREN

APPROACH TO:

Malrotation

DEFINITIONS

VOLVULUS: Twisting of the mesentery of the small intestine leading to decreased vascular perfusion, which results in ischemia and ultimately bowel necrosis.

INTUSSUSCEPTION: A condition in which a proximal portion of the gastrointestinal tract telescopes into an adjacent distal portion. The most common location is ileocolic portion of the bowel.

CLINICAL APPROACH

Malrotation occurs when intestinal rotation is incomplete during fetal development. During normal fetal development in the first trimester, the growing intestine exits the abdominal cavity, elongates, and ultimately rotates 270° in a counterclockwise manner before returning into the abdomen. Following normal intestinal rotation, the duodenojejunal junction (ligament of Treitz) is fixed to the posterior body wall to the left of the spine. In cases of malrotation, the ligament of Treitz is located on the right side and the intestine may use the small portion of attached mesentery as axis to turn (volvulus) leading to ischemia and possible necrosis.

Although individuals with intestinal malrotation may present from birth to adulthood, the classic presentation is that of an infant with bilious vomiting due to intestinal obstruction. With prolonged ischemia the bowel becomes necrotic and the patient may have melena or hematemesis, and may develop peritonitis, acidosis, and sepsis. Without surgical intervention, risk of mortality is significant. Patients with malrotation and either partial or intermittent volvulus may present with recurrent abdominal pain or lymphatic congestion leading to failure to thrive because of malabsorption or chylous ascites. Individuals may also have asymptomatic malrotation as an incidental finding.

Abdominal radiographs may be normal or have nonspecific findings in cases of volvulus; thus an upper gastrointestinal contrast series is generally indicated. The characteristic finding in cases of volvulus is a “corkscrew” pattern of the duodenum or “bird’s beak” of the second or third portions of the duodenum. In cases of malrotation with or without volvulus, abnormal position (right sided) of the ligament of Treitz or malposition of the colon may be noted with contrast radiography.

Prior to emergent surgical intervention, the initial management of patients with malrotation and volvulus includes appropriate evaluation of fluid status as patients may have significant fluid loss with electrolyte abnormalities. In the ill-appearing infant, placement of a nasogastric tube to aid gastrointestinal decompression, and initiation of parenteral antibiotic, in order to address potential sepsis are indicated. Exploratory laparotomy is performed and bowel viability assessed. Areas of necrotic bowel are removed and Ladd procedure of disengaging bowel with anomalous fixation and appendectomy are performed. Complications include short gut syndrome if a significant portion of necrotic bowel is removed, and adhesions may develop leading to obstruction. Because of the significant mortality and morbidity associated with volvulus, asymptomatic patients with malrotation require surgical intervention.

COMPREHENSION QUESTIONS

49.1 Malrotation with volvulus is most likely to be present in which of the following patients?

A. A healthy 15-month-old with severe paroxysmal abdominal pain and vomiting

B. A 15-year-old sexually active girl with lower abdominal pain

C. A 3-day-old term infant with bilious emesis, lethargy, and abdominal distension

D. A 4-day-old premature baby (33-week gestation) who has recently started nasogastric feeds; he now has abdominal distention, bloody stools, and thrombocytopenia

E. A 7-year-old non-toxic-appearing girl with abdominal pain, vomiting, fever, and diarrhea

49.2 A 3-day-old boy presents with 12 hours of bilious vomiting, abdominal pain, and abdominal distension. Which of the following is the most appropriate next step in management?

A. Order an abdominal ultrasonography.

B. Order a computed tomography scan of the abdomen.

C. Order an upper GI contrast series.

D. Order a barium enema.

E. Order a chest radiograph.

49.3 A 9-year-old boy has 24 hours of persistent abdominal pain and vomiting. His physical examination reveals abdominal guarding and right lower quadrant rebound tenderness. Which of the following is the most likely diagnosis?

A. Appendicitis

B. Gastroenteritis

C. Gastroesophageal reflux

D. Intussusception

E. Pyloric stenosis

49.4 A previously healthy 18-month-old child has vomiting and severe, paroxysmal, writhing abdominal pain (he prefers to have his knees flexed to the chest) alternating with periods of relative comfort with a soft, only mildly tender abdomen. On abdominal examination you find a sausage-like mass. He has not stooled, but you find blood upon digital rectal examination. Which of the following is the best next step in management?

A. Administer morphine for pain control.

B. Order a computed tomography of the abdomen.

C. Obtain an air contrast enema.

D. Obtain serum acetaminophen levels.

E. Begin antibiotics for Escherichia coli 0157:H7.

49.5 A 6-week-old male infant has projectile emesis after feeding. He has an olive-shaped abdominal mass on abdominal examination. Which of the following statements is accurate?

A. He likely has hypochloremic metabolic alkalosis.

B. He likely has metabolic acidosis.

C. This condition is more common in female infants.

D. He should be restarted on feeds when the vomiting resolves.

E. He likely will develop diarrhea.

ANSWERS

49.1 C. The 3-day-old term infant with bilious emesis and abdominal distension has classic presenting features of malrotation with volvulus. The 15-month-old child with paroxysmal abdominal pain is most likely to have intussusception. The adolescent female is evaluated for ectopic pregnancy, pelvic inflammatory disease, appendicitis, ovarian torsion, and ruptured ovarian cyst. The premature infant might have necrotizing enterocolitis, whereas the 7-year-old girl most likely has gastroenteritis.

49.2 C. Order an upper GI contrast series. Fluid and electrolyte status should also be evaluated.

49.3 A. This child most likely has appendicitis based on the clinical presentation.

49.4 C. The case describes the typical presentation of intussusception. Although a clinical diagnosis can be made, the diagnostic “gold” standard and often treatment is contrast enema. Air contrast usually is preferred because the complication risk is lower than with other forms of contrast material. Prior to diagnostic intervention, patients should undergo measurement of serum electrolyte and hemoglobin levels and receive fluid resuscitation. When suspicion for intussusception is high, a pediatric surgeon should be consulted. Classically described “currant jelly stools” are a late finding. Recurrence of intussusception following successful reduction occurs in 5% to 10% of cases.

49.5 A. This infant has the features of pyloric stenosis, a condition four times more common in males and more common in first-born children. Affected infants usually present between the third and eighth week of life with increasing projectile emesis. Abdominal examination may reveal an olive-shaped mass and visible peristaltic waves. Serum electrolyte levels usually reveal hypochloremic metabolic alkalosis. Ultrasonography is useful in confirming the diagnosis.


CLINICAL PEARLS

Image Treatment of malrotation with volvulus includes emergent surgical intervention.

Image Classic features of intussusception are fever, intermittent colicky abdominal pain, currant jelly stools, and a sausage-like abdominal mass.

Image Classic features of pyloric stenosis include projectile vomiting, an olive-shaped abdominal mass, and hypochloremic metabolic alkalosis.


REFERENCES

Aiken JJ. Malrotation and volvulus. In: Rudolph CD, Rudolph AM, Lister GE, First LR, Gershon AA eds. Rudolph’s Pediatrics. 22nd ed. New York, NY: McGraw-Hill; 2011:1417-1419.

Bales C, Liacouras CA. Intestinal atresia, stenosis, and malrotation. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: WB Saunders; 2011:1277-1281.

Brandt ML. Intussusception. In: McMillan JA, Feigin RD, DeAngelis CD, Jones MD, eds. Oski’s Pediatrics: Principles and Practice. 4th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2006: 1938-1940.

Chu A, Liacouras CA. Ileus, adhesions, intussusception, and closed-loop obstructions. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: WB Saunders; 2011:1287-1289.

Hunter AK, Liacouras CA. Hypertrophic pyloric stenosis. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: WB Saunders; 2011:1274-1275.

McEvoy CF. Developmental disorders of gastrointestinal function. In: McMillan JA, Feigin RD, DeAngelis CD, Jones MD, eds. Oski’s Pediatrics: Principles and Practice. 4th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2006:371-375.

Nazarey P, Sato TT. Gastrointestinal obstruction. In: Rudolph CD, Rudolph AM, Lister GE, First LR, Gershon AA eds. Rudolph’s Pediatrics. 22nd ed. New York, NY: McGraw-Hill; 2011:1394-1396.