A 22-month-old girl was seen in the emergency department (ED) after several hours of abdominal pain associated with non-bloody, non-bilious emesis. Over the past 2 months, she has had 7 or 8 similar episodes of abdominal pain followed by emesis 1 to 2 hours later.
Pain appeared severe, causing the patient to cease all activity, clutch her epigastric region, cry, and remain in the fetal position. Her mother was unable to describe quality of pain or whether it radiated.
During all episodes, pain began suddenly and then continued intermittently for several hours. Emesis alleviated the pain. Temporal relationship of the start of abdominal pain to oral intake was not clear because episodes occurred at different times throughout the day. The patient's mother thought the intensity of pain was increasing. Between episodes, patient was playful and energetic, and had good oral intake and a varied diet. However, the mother noted some weight loss during the past month.
Her pediatrician, seen multiple times since the start of these episodes, had diagnosed gastritis. The patient had made 3 ED visits; laboratory study results were normal. Bowel intussusception was suspected but abdominal ultrasonographic findings were normal.
There was no history of sick contacts, trauma, or chemical ingestion. The patient's bowel habits were normal; there was adequate urinary output. Immunizations were up-to-date. There were no drug or food allergies. The patient's development was appropriate for her age.
The family history was not contributory. The patient lived with her mother, father, 4 siblings, and a dog. Six months before current episode, patient was taken to Mexico for 1 month; there was no other recent travel. The patient did not attend day care.
Past medical history was relatively unremarkable. The patient was born at 36 weeks' gestation via "repeat" cesarean section. Her birth weight was 1820 g with moderate intrauterine growth restriction. Antenatal maternal α-fetoprotein (AFP) level was said to be elevated, but further evaluation for fetal anomaly was not carried out. The patient was admitted to the neonatal ICU for 10 days after delivery because of poor oral intake. There were no respiratory problems during neonatal hospitalization.
At age 20 months, incontinentia pigmenti was diagnosed on the basis of hypopigmented lesions on patient's extremities, which had been present since 2 months of age.
The patient was notably thin. Her weight was 8.3 kg (less than the third percentile); height, 83 cm (25th to 50th percentile). She was afebrile, and vital signs were normal. The lungs were clear. There was a regular heart rate and rhythm; no murmurs were heard. The abdomen was soft and non-distended; there was discomfort on palpation when the patient was awake but not while sleeping. No rebound tenderness or guarding was noted. There were decreased bowel sounds in the abdomen. The skin showed hypopigmented linear lesions along the lines of Blaschko on the upper and lower extremities.
A complete metabolic panel and pancreatic enzyme levels were all within normal limits. White blood cell count and differential were normal. Hemoglobin level was low (9.3 g/dL) and hematocrit was level was low (29.9%). The patient was hospitalized for further evaluation.
IMAGING STUDY RESULTS
Intussusception was ruled out by ultrasonography. Abdominal flat plate radiographs—obtained to evaluate for possible intermittent volvulus and bowel obstruction—showed tubular radiolucencies in the upper abdomen extending to the chest toward the midline. Decreased gas was noted in lower abdomen (Figure 1). Chest fluoroscopy showed several rounded lucencies in the midline of the chest on right and left sites, with a tubular structure passing over the gastroesophageal junction. An enlarged cardiac silhouette was also noted (Figure 2).
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