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Congenital pyloric atresia and associated anomalies: a case series
Rahul Gupta1, Varsha Soni1, Praveen Mathur1
1Department of Paediatric Surgery, SPMCHI, SMS Medical College, Jaipur, India.
Insights
Congenital pyloric atresia (CPA) is a rare surgical condition. This study found an 81.8% survival rate in eleven patients, with sepsis being the cause of mortality in two cases.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Medical Genetics
Background:
- Congenital pyloric atresia (CPA) is an extremely rare congenital anomaly of the gastric outlet.
- It presents a significant surgical challenge due to its rarity and potential for associated anomalies.
Purpose of the Study:
- To retrospectively analyze the clinical presentation, management, and outcomes of patients diagnosed with congenital pyloric atresia.
- To identify associated anomalies and evaluate the effectiveness of different surgical interventions.
Main Methods:
- Retrospective case series of eleven patients with CPA.
- Data collected included age at diagnosis, sex, symptoms, associated anomalies, operative findings, surgical procedures, and patient outcomes.
- Review of surgical records and patient charts.
Main Results:
- Eleven patients (8 male: 3 female) were studied, with diagnosis ranging from 1 day to 3 years.
- Associated anomalies were present in 36.6% of cases, including epidermolysis bullosa, esophageal atresia, and colonic atresia.
- Type 1 CPA was most common (54.5%), followed by Type 2 (36.3%) and Type 3 (9%).
- Surgical procedures included Heineke-Mickulicz pyloroplasty, Finney's pyloroplasty, and gastro-duodenostomy.
- An overall survival rate of 81.8% was achieved, with two deaths attributed to sepsis.
Conclusions:
- Congenital pyloric atresia requires prompt surgical intervention.
- Associated anomalies are common and impact management and prognosis.
- While surgical outcomes can be favorable, sepsis remains a significant risk factor for mortality.
Abstract:
Congenital pyloric atresia (CPA) is a very rare surgical condition. Eleven patients with the diagnosis of CPA treated at our hospital were retrospectively studied for the age at diagnosis, sex, presenting symptoms, associated anomalies, operative findings, treatment and outcome. Male: Female is 8:3. The age at diagnosis ranged from one day to three years. Associated anomalies were seen in four (36.6%). These included epidermolysis bullosa (EB) in two, oesophageal atresia with distal trachea-oesophageal fistula in one, colonic atresia in one, sensorineural deafness and dysplastic kidney in one patient. All three types of CPA were observed; six (54.5%) had type 1, four (36.3%) had type 2 and one (9%) had type 3 [(core)]. Different procedures performed were Heineke-Mickulicz pyloroplasty, Finney's pyloroplasty and gastro-duodenostomy. Post-operatively, nine out of eleven did well while other two died giving an overall survival of 81.8%. Sepsis was the cause of death in both of them.
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