Paraoesophageal hernia (PH) in children is a rare entity, and most reported cases refer to adult patients. Its etiology is not precisely known, but the hypothesis of its congenital origin is widely accepted. Similarly to other congenital diaphragmatic defects, PH follows a sporadic pattern of incidence in most cases. Familial occurrence of sliding hiatal herniae has been reported in more than 20 cases, but only one family with two members affected by PH was described in the literature. We present two pairs of siblings with the paraoesophageal type of hiatus hernia and discuss the clinical presentation of this anomaly in children.

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The authors present their own modification of surgical staged repair of large abdominal wall defects (AWD). 21 newborns with gastroschisis (GSCH) and 8 with omphalocele (OC) are presented. In all patients the protruding viscera were placed in a prosthetic sac with a semi-permeable lining. The sac was suspended using a system with active external traction with a force amounting to 30 - 40 % of the baby's weight. All babies tolerated the applied method well. A progressive stretching of the abdominal wall and enlargement of the abdominal cavity enabled the defect to be closed between the second and sixth postoperative day. In comparison to the classic method, the presented modification of staged repair of AWD makes an earlier reduction of the viscera into the abdomen possible and does not seem to be associated with a higher risk of mechanical or infectious complications.

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