Spontaneous bile duct perforation with cystic fibrosis and meconium ileus

Abstract

a b s t r a c tSpontaneous bile duct perforation in the newborn is rare, with less than 150 cases reported. A term male newborn presented with abdominal distention and respiratory distress on the first day of life. Paracentesis revealed 420 mL of bile without succus or meconium, and laparoscopic washout and drainage was performed. A follow-up contrast enema several days later revealed a microcolon. The second operation confirmed the spontaneous bile duct perforation, but also revealed meconium ileus with ileal perforation and thick meconium. A sweat chloride study was abnormal and the patient was homozygous for the delta-F508 mutation. This is the first reported case of spontaneous bile duct perforation in association with meconium ileus or cystic fibrosis. Ó 2015 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). Case reportA newborn 37 6/7weeks gestation Caucasian male infant weighing 3075 g presented at birth with abdominal distention. He was born via cesarean section with no significant antenatal history and a normal newborn screening panel to a G2P2 mother. Shortly after birth he developed apnea and respiratory distress associated with worsening abdominal distention requiring intubation and chest compressions. He was subsequently transferred for a higher level of care. On exam the patient had severe abdominal distention noted with thin skin and visible veins. He had not passed any meconium. An abdominal radiograph demonstrated non-dilated, centralized lops of bowel with no gas in the expected location of the colon (Fig. 1). Abdominal sonography demonstrated fluid with calcifications.An urgent bedside paracentesis was done for respiratory compromise and 420 mL of viscous, clear goldenegreen bile, with no succus or meconium, was drained. The excessive quantity of clear bile lead us to make the presumptive diagnosis of spontaneous bile perforation. He was then taken to the operating room for laparoscopic washout and drain placement.At laparoscopy, chronic bile peritonitis was encountered as evidenced by bile-stained fibrinous exudate completely covering the bowel and liver which caused the bowel to be completely adherent to itself. As there was no meconium spillage or identification of small bowel perforation, the loops of bowel were not completely separated during this operation. A laparoscopic-guided intraoperative cholangiogram was preformed via the gallbladder with a micro-puncture needle. This study was technically difficult due to the bile peritonitis and a discrete site of leakage was not identified. The puncture site on the gallbladder was closed and the abdomen was irrigated at the end of the procedure. A closed suction drain was left in the right upper quadrant and the patient was transferred back to the neonatal intensive care unit.Over the next few days there was no bilious drain output and no re-accumulation of fluid by ultrasound. The patient continued to be distended without any clinical signs o...

Perforación espontánea de la vía biliar en un neonato: un hallazgo inesperado. Caso clínico

“…Sólo el 20% de los pacientes se presentan con cuadro agudo de distensión abdominal, fiebre, vómito, irritabilidad y signos de severa peritonitis fulminante que lleva al shock séptico y muerte 2,13,14 . Por la presentación clínica aguda de nuestra paciente en ausencia de ictericia, se pensó inicialmente en enterocolitis necrosante perforada, diagnóstico por el cual fue llevada a cirugía.…”