Single or double bubble: Sign of trouble! Congenital pyloric atresia: Report of two cases and review of literature

Abstract

Congenital pyloric atresia is a very rare anomaly. It can occur as an isolated anomaly and can be associated with other conditions such as aplasia cutis congenital/epidermolysis bullosa or multiple intestinal atresias. We present two cases of congenital pyloric atresia, solitary air bubble on X-ray abdomen being pathognomonic sign of pyloric atresia. However, it might present with an intermittent double bubble sign.

Pyloric Atresia: An Unusual Presentation

“…Patients usually present soon after birth due to non-bilious vomiting [2,10], which is the most common clinical feature at presentation (100% of cases) in addition to epigastrium distension (68% of cases) [5]. In a case series by Llce et al, the condition was often associated with a low birth weight [1].…”

“…In a case series by Llce et al, the condition was often associated with a low birth weight [1]. Prenatally the diagnosis is often suspected due to polyhydramnios (63% of cases) [5]. Fetal ultrasound also often shows a dilated stomach and at times fluid distension of esophagus [6,8,9].…”

“…Fetal ultrasound also often shows a dilated stomach and at times fluid distension of esophagus [6,8,9]. Postnatal diagnosis also relies on imaging, with abdominal radiograph showing the characteristic 'single-bubble' sign in up to 98% of the cases [5]. Postnatal ultrasound can also be performed and may show an elongated and echogenic pylorus with no patent channel [2,6].…”

“…The usual presenting symptoms include non-bilious vomiting and abdominal distension [3][4][5]. Delay in diagnosis may occur since the non-bilious vomiting may be attributed mistakenly to gastroesophageal reflux.…”

Angeborene Pylorusagenesie Typ C

“…Müller et al [11] zeigten, dass bei den betroffenen Neugeborenen in 100% der Fäl-le nichtgalliges Erbrechen und in 68% ein geblähtes Abdomen vorhanden war [10,11,13]. Hinweisend für die Diagnose Pylorusatresie ist das "single bubble"-Phänomen in der Abdomenübersichtsaufnahme, das in 98% der Fälle besteht [11,16]. Wie auch bei unserer Patientin erkennt man eine große Magenblase ohne distale Luftfüllung, sodass von einer Stenose der gastralen Ausflussbahn auszugehen ist.…”