[Severe respiratory distress due to ileal duplication cyst in the newborn]

Differential diagnosis of neonatal respiratory distress includes pulmonary and systemic disorders and anatomic problems compromising respiratory system. We report on a 2770-g female born to a 29-year-old gravida 3, para 2 woman after 34 weeks of gestation. Antenatal ultrasound performed in week 8 and 21 was normal. The infant was delivered by cesarean section after amniotic membranes had been ruptured for less than 12 hours due to signs of fetal distress. The Apgar score was 3 and 3 at 1 and 5 minutes, respectively. The infant was intubated and resuscitated, and transferred immediately to the neonatal intensive care unit. She had an extremely protuberant and cyanotic abdomen. Dilated cutaneous collateral vessels were apparent in the periumbilical region. Abdominal sonography showed cystic multiloculated tumorous mass filled with dense, flocculent content at the level of hepatic portal. The tumorous mass occupied the majority of the abdomen with caudal extension toward the pelvis and dorsally toward the spine. The liver was displaced high under the diaphragm with the left liver lobe in the left hemiabdomen. On x-ray the lung were collapsed due to a large abdominal mass in the right hemiabdomen that displaced the right diaphragm and intestines contralaterally. She soon developed bilateral pneumothoraces. Drainage and continuous suction were started. The infant failed to improve despite all attempts and died. On autopsy, an extremely large, mobile, multichambered, solitary cyst was found. It was attached to the mesenteric side of the ileum by its own thin peduncular stalk and had no communication with the remainder of the gut. It occupied the majority of the abdomen. Histologic section revealed a well-developed smooth muscle wall and inner mucosa of small bowel type. Respiratory distress is a common problem in premature infants. The majority of cases are due to pulmonary disorders (e. g., hyaline membrane disease, meconium aspiration syndrome, pneumonia), hypothermia, metabolic acidosis, anemia, and congenital heart disease. Anatomic problems including space occupying lesions are less common. Duplications of the alimentary tract in infants and children are rare congenital anomalies. Although symptoms can occur at any age, they usually present during the first year. In our patient, intraabdominal mass caused severe respiratory distress and respiratory failure in the first hours of postnatal life. This had been seen before only as a complication of intrathoracic lesions extending into the abdominal cavity. Pathology revealed spherical intestinal duplication that was completely separated from the alimentary tract. Embryologically, it was a localized duplication. Respiratory distress in our patient was refractory to all means of mechanical ventilation. Poor lung compliance was the consequence of prenatal lung hypoplasia and inadequate postnatal lung expansion due to the duplication cyst space occupying character and its compressive effect. Prenatal diagnosis was the child's only chance for survival but it was not made. Duplications of the alimentary tract can present a diagnostic challenge even in the first hours of life. They should be included in the differential diagnosis of severe respiratory distress, especially in premature infants in which timely prenatal diagnosis cannot be always made. We propose their inclusion among other space occupying lesions that might be the cause of severe respiratory distress even in the earliest neonatal period.