Laparoscopic gastroduodenal anastomosis (Billroth I) for the management of type II pyloric atresia in Carmi syndrome: a case report

Pyloric atresia accounts for less than 1 % of all congenital intestinal obstructions, with an estimated incidence of 1:100,000 live births. Its association with epidermolysis bullosa, known as Carmi syndrome, is rare but well documented. Minimally invasive techniques can be employed in the surgical management of complex neonatal conditions. A male neonate was delivered vaginally at 35 weeks of gestation to a 16-year-old mother, with reported consanguinity between the parents. At birth, he presented with widespread bullous skin lesions involving the extremities, thorax, and face, along with a positive Nikolsky sign, findings that raised strong suspicion for epidermolysis bullosa. Birth weight was 2000 g, and no resuscitative maneuvers were required. Following the initiation of enteral feeding, the patient developed non-bilious vomiting. Abdominal radiography showed a single gastric bubble with absence of distal gas. A contrast study confirmed gastric outlet obstruction. Endoscopy on day three revealed complete pyloric occlusion and mucosal fragility. A laparoscopic procedure was performed, confirming type II pyloric atresia. A Laparoscopic gastroduodenostomy (Billroth I) was successfully carried out without intraoperative complications. Postoperative contrast imaging confirmed adequate anastomotic patency. Unfortunately, the patient developed sepsis due to extensive skin involvement and died on postoperative day three. Minimally invasive gastroduodenal anastomosis (Billroth I) seems to be technically feasible in newborns with pyloric atresia.