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Congenital para esophageal hernia: a 10 year experience from Saudi Arabia.
Indian J Pediatr. 2009 May; 76(5):489-93.IJ

Abstract

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.

Authors+Show Affiliations

Department of Pediatric Surgery, Assir Central Hospital, Abha, KSA, Saudi Arabia.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

19390801

Citation

Jetley, Nishith Kumar, et al. "Congenital Para Esophageal Hernia: a 10 Year Experience From Saudi Arabia." Indian Journal of Pediatrics, vol. 76, no. 5, 2009, pp. 489-93.
Jetley NK, Al-Assiri AH, Al Awadi D. Congenital para esophageal hernia: a 10 year experience from Saudi Arabia. Indian J Pediatr. 2009;76(5):489-93.
Jetley, N. K., Al-Assiri, A. H., & Al Awadi, D. (2009). Congenital para esophageal hernia: a 10 year experience from Saudi Arabia. Indian Journal of Pediatrics, 76(5), 489-93. https://doi.org/10.1007/s12098-009-0084-3
Jetley NK, Al-Assiri AH, Al Awadi D. Congenital Para Esophageal Hernia: a 10 Year Experience From Saudi Arabia. Indian J Pediatr. 2009;76(5):489-93. PubMed PMID: 19390801.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Congenital para esophageal hernia: a 10 year experience from Saudi Arabia. AU - Jetley,Nishith Kumar, AU - Al-Assiri,Ali Hassan, AU - Al Awadi,Dawood, Y1 - 2009/04/23/ PY - 2008/02/12/received PY - 2008/08/08/accepted PY - 2009/4/25/entrez PY - 2009/4/25/pubmed PY - 2009/8/14/medline SP - 489 EP - 93 JF - Indian journal of pediatrics JO - Indian J Pediatr VL - 76 IS - 5 N2 - 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. SN - 0973-7693 UR - https://www.unboundmedicine.com/medline/citation/19390801/Congenital_para_esophageal_hernia:_a_10_year_experience_from_Saudi_Arabia_ L2 - https://dx.doi.org/10.1007/s12098-009-0084-3 DB - PRIME DP - Unbound Medicine ER -