VATER Syndrome- A Case Report -
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VATER syndrome is defined as the combinations of three or more of the followings; Vertebral defect, Anal atresia, Esophageal atresia and/or Tracheo-Esophageal fistula, Renal dysplasia, and Radial-ray limb anomalies. A 21-month old female patient was admitted. She had the history of anastomosis of trcheo-esophageal fistula, V-P shunt operation due to hydrocephalus, and spina bifida. Upon admission, physical examinations showed increased deep tendon reflexes of all four extremities with grade 1 spasticity and popliteal angle being 45 degrees, bilaterally. The overall development of the patient was delayed. The imaging study showed hydrocephalus, periventricular leukomalatic change, underdevelopment of the cerebral cortex, atrophy of corpus callosum, Arnold-Chiary malformation, a vascular malformation along the intradural space from lower cervical to nearly entire thoracic vertebral level, multiple vertebral anomalies, and the fusion of multiple ribs. Furthermore hydronephrosis of the left kidney was detected. Follow-up of natural history and management of the patient is needed.
Abstract
VATER syndrome is defined as the combinations of three or more of the followings; Vertebral defect, Anal atresia, Esophageal atresia and/or Tracheo-Esophageal fistula, Renal dysplasia, and Radial-ray limb anomalies. A 21-month old female patient was admitted. She had the history of anastomosis of trcheo-esophageal fistula, V-P shunt operation due to hydrocephalus, and spina bifida. Upon admission, physical examinations showed increased deep tendon reflexes of all four extremities with grade 1 spasticity and popliteal angle being 45 degrees, bilaterally. The overall development of the patient was delayed. The imaging study showed hydrocephalus, periventricular leukomalatic change, underdevelopment of the cerebral cortex, atrophy of corpus callosum, Arnold-Chiary malformation, a vascular malformation along the intradural space from lower cervical to nearly entire thoracic vertebral level, multiple vertebral anomalies, and the fusion of multiple ribs. Furthermore hydronephrosis of the left kidney was detected. Follow-up of natural history and management of the patient is needed.
참고문헌 (6)
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- Lee DH, "Clinical features and musculoskeletal anomalies in VATER association", J Korean Orthop Assoc, 36, pp.593~599 (2001)
- Rudolph CD, "Rudolph's pediatrics", pp.1641~ (2002), McGraw-Hill
- Behrman R, "Nelson textbook of Pediatrics", pp.623~ (2004), Saunders
- Orford JE, "Dose response relationship between adriamycin and birth defects in a rat model of VATER assocation", J Pediatr Surg, 34, pp.392~398 (1999)
- Wheeler PG, "Adults with VATER association: long-term prognosis", Am J Med Genet A, 138, pp.212~217 (2005)