Congenital pyloric atresia (CPA) is a very rare condition that was first described by Calder in 1749.Its incidence is approximately 1 in 100,000 newborns and constitutes about 1% of all intestinal atresias [1,2].
Anatomically, Pyloric Atresia is divided into three differ¬ent anatomical varieties: type A is a prepyloric mem¬brane, type B a solid core of tissue replacing the pyloric canal, and type C an atretic pylorus with a gap between stomach and duodenum .
The diagnosis of pyloric atresia may be suspected from plain abdominal films consistent with a dilated stomach with the typical ‘single bubble’ appearance as seen in our case . Ultrasonography, said to be specific for this condition was not performed in our case.
Pyloric atresia may occur as an isolated condition or associated with other abnormalities, the most common being junctional epidermolysis bullosa (EB), a rare auto¬somal recessive disorder affecting the skin and mucosa [3-6]. However, its association with multiple colonic atresias is not reported to date; though it has been re¬ported in association with multiple small bowel and colonic atresias.
Colonic and pyloric atresias have a different embryologi¬cal basis and different period of occurrence. Colonic atresia is a late event usually occurs beyond 24 weeks of gesta¬tion. Whereas, pyloric atresia is thought to result from developmental arrest between the 5th and 12th weeks of intrauterine life [2,7].
The treatment of CPA is surgical. For pyloric diaphragm or pyloric atresia without a gap, the treatment is exci¬sion of the diaphragm and Heineke-Mikulicz pylorop¬lasty. Intraoperatively it is important to locate the site of obstruction especially in those with a diaphragm, and to obviate missing a windsock diaphragm, a catheter should be passed distally via a small gastrostomy. This is also of importance in case there is more than one diaphragm. For those with pyloric atresia with a gap, if the gap is short, they should be treated with a Finny or Heineke-Mickulicz pyloroplasty, but if the gap is long then a gastroduodenostomy becomes the treatment of choice .
In conclusion, our case is unique because of occurrence of both anomalies in the same patient. No similar report was found in the literature.