title: Congenital pyloric atresia, presentation, management, and outcome: a report of 20 cases.
abstract: BACKGROUND
  Congenital pyloric atresia (CPA) is a very rare anomaly. It is usually seen as an isolated condition with excellent prognosis. Few cases are familial. These are usually associated with other hereditary conditions and have a poor prognosis. This is a review of our experience with 20 patients with CPA outlining aspects of diagnosis, associated anomalies and management.
  
  
  METHODS
  This is a retrospective analysis of 20 cases seen over a 22 year period (December 1990 to December 2012). Their records reviewed for: age, sex, presentation, prenatal history, associated anomalies, investigations, treatment, operative findings and the outcome.
  
  
  RESULTS
  20 cases (9 Males, 11 Females) were treated. 7 patients were full term and the remaining 13 were prematures. Their mean birth weight was 2.1 kg (1.1 kg to 3.9 kg). Polyhydramnios was seen in 13 patients (65%). Two were brothers and four were members of the same family. Isolated CPA was seen in 7 (35%); 13 had an associated conditions: epidermolysis bullosa (EB) in 8 (40%) and multiple intestinal atresias (MIA) in 5 (25%). Three patients had associated esophageal atresia. All were operated on except two who died early due to unrelenting sepsis. The variety of pyloric atresias encountered were as follows: pyloric diaphragm in 13 including double diaphragms in 2, pyloric atresia with a gap in 4 and pyloric atresia without gap in 3. Ten died postoperatively giving an overall survival of 40%.
  
  
  CONCLUSIONS
  CPA is a very rare condition. Isolated CPA carries a good prognosis. Association of CPA with other familial and congenital anomalies like EB and MIA carries a poor prognosis.
authors: Department of Pediatric Surgery, Maternity and Children Hospital, Dammam Qatif 31911, Saudi Arabia. Electronic address: ahsalsalem@hotmail.com.
authors: Ahmed H
authors: AH
authors: Al-Salem
authors: Department of Pediatric Surgery, Maternity and Children Hospital, Dammam Qatif 31911, Saudi Arabia. Electronic address: Mohamedramadan_75@yahoo.com.
authors: Mohamed Ramadan
authors: MR
authors: Abdulla
authors: Department of Pediatric Surgery, Maternity and Children Hospital, Dammam Qatif 31911, Saudi Arabia. Electronic address: mukulkothari@gmail.com.
authors: Mukul R
authors: MR
authors: Kothari
authors: Department of Pediatric Surgery, Maternity and Children Hospital, Dammam Qatif 31911, Saudi Arabia. Electronic address: abuyassin09@gmail.com.
authors: Mohamed Ibrahim
authors: MI
authors: Naga
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000601:surgery; D005260:Female; D017219:Gastric Outlet Obstruction / Q000151:congenital* / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications; D011708:Pylorus / Q000002:abnormalities* / Q000601:surgery; D012189:Retrospective Studies; D018805:Sepsis / Q000209:etiology; D016896:Treatment Outcome
keywords: Aplasia cutis congenita; Epidermolysis bullosa; Hereditary multiple intestinal atresia; Pyloric atresia