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| Year : 2000 | Volume
: 5
| Issue : 3 | Page : 138-140 |
Beckwith Wiedemann Syndrome.
G Chalapathi, SK Chowdhary, KL Narasimhan, KLN. Rao
Asst. Professor, Deptt. of Pediatric Surgery, Advanced Pediatric Centre, PGIMER, Chandigarh-160012
Correspondence Address:
G Chalapathi
Asst. Professor, Deptt. of Pediatric Surgery, Advanced Pediatric Centre, PGIMER, Chandigarh-160012
 Source of Support: None, Conflict of Interest: None  | Check |
ABSTRACT: Beckwith Wiedemann syndrome is characterized by Exomphalos, macroglossia and gigantism. It is associated with life threatening hypoglycemia in the neonatal period and an increased risk of solid organ tumors in the long term. Although exomphalos is a common condition seen in our country, this entity has not been previously reported from India. Authors report such a case and its outcome. Exomphalos is the commonest abdominal wall defect seen in our country. Although there is no published series on the outcome of exomphalos in neonates from our country, exomphalos major has a high risk of operative mortality in the neonatal period. Beckwith Wiedemann syndrome is an association of examphalos, gigantism and macroglossia. Life threatening complication of hypoglycemia in the neonatal period and a long term risk of solid organ tumors are further problems.
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