Congenital para esophageal hernia: a 10 year experience from Saudi Arabia.

OBJECTIVE: Para esophageal hiatal hernia is a rare childhood condition and reported series have had scant number of children which makes diagnosis a challenge. The authors sought to study the presentation and the outcome of treatment of congenital para esophageal hernias (CPEH) over a period of 10 years from a single tertiary care hospital in Saudi Arabia.
METHODS: The records of 9 patients presenting between 1997 and 2007, were retrospectively analyzed for demographics, presenting features, referral diagnoses, investigations, management including operative procedures, their outcome and follow-up.
RESULTS: Nine patients (3 males and 6 females) aged between 8 days to 34 months were seen. Respiratory distress (n=6), vomiting (n=5) and frequent respiratory tract infections (n=3) were the most common presentations. Cyanosis (n=2), cough and excessive crying were the other important symptoms. The referral diagnoses in these patients included congenital Bochdalek's hernias, lung abscess, bronchogenic cyst, pneumatocoele, bronchiolitis, and pneumonias which reflected a misinterpretation of their clinical findings and chest X-rays. Seven of these patients had other associated congenital anomalies. Three had cardiovascular abnormalities and 2 had lesions of the central nervous system. A pair of siblings had Marfan's syndrome. All the patients had abnormal chest C-rays and an UGS (upper GI series) proved to be diagnostic in 8 patients. The CT scans done in 4 patients corroborated the findings of the UGS. A laparotomy was done on most patients (n=8) which comprised of reduction of the stomach, resection of the hernial sac, tightening of the hiatus and a gastropexy or a gastrostomy. One patient, who underwent thoracotomy died of surgical complications. Two others died of causes unrelated to the surgery. The remaining six operated patients have been followed up for a median of 3.5 years and are doing well.
CONCLUSION: CPEH is uncommon in children, presented with respiratory tract symptoms and vomiting, and may be associated with Martan's syndrome. It should be considered in the workup of a child with vomiting or frequent chest infections. Abnormal chest X-rays may indicate the diagnosis and a subsequent UGS, is confirmatory. The present study found the aparotomya good approach for repair of the wide hiatus. A gastropexy and a floppy fundoplication were added to prevent reherniation and post operative reflux though given the small numbers it is not possible to determine the place of either of these procedures. CPEH may be frequently associated with other congenital problems which may impact survival.