Gollop-Wolfgang Syndrome

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Classification according to ICD-10
Q74.8 Other specified congenital malformations of the extremity (s)
ICD-10 online (WHO version 2019)

The Gollop-Wolfgang syndrome is a very rare form of malformation with cleft formation in the hand or foot ( ectrodactyly ) with one-sided cleft hand and one-sided bifurcation of the thigh bone .

The name refers to the first authors of descriptions from 1980 by the Brazilian doctor Thomaz Rafael Gollop and 1984 by the American orthopedic surgeon Gary L. Wolfgang .

X-ray overview on a 2 week old newborn with Gollop-Wolfgang syndrome (left femur)

distribution

The frequency is not known; the inheritance may be autosomal - recessive or dominant .

root cause

The etiology is unknown.

Clinical manifestations

In addition to hand ectrodyctyly and femur bifurcation, there may be hypoplasia or aplasia of the tibia, oligodactyly or monodactyly of the foot. The femoral bifurcation is mostly unilateral. The malformation can be detected prenatally using sonography .

Differential diagnosis

The hypoplastic tibia polydactyly syndrome (Werner Mesomeles syndrome) and the tibia aplasia-ectrodactyly syndrome are to be distinguished .

therapy

Treatment consists of orthopedic prosthetic and reconstructive care in a specialized center.

literature

  • N. Dhanakodi, SK Tripathy: Gollop-Wolfgang syndrome. In: The Indian journal of medical research. Vol. 139, No. 6, June 2014, pp. 963-964, ISSN  0971-5916 . PMID 25109738 .

Individual evidence

  1. ↑ a b c d Gollop-Wolfgang syndrome. In: Orphanet (Rare Disease Database).
  2. ↑ TR Gollop, E. Lucchesi, RM Martins, AS Nione: Familial occurrence of bifid femur and monodactylous ectrodactyly. In: American journal of medical genetics. Vol. 7, No. 3, 1980, pp. 319-322, ISSN  0148-7299 . doi: 10.1002 / ajmg.1320070313 . PMID 7468656 .
  3. ↑ GL Wolfgang: Complex congenital anomalies of the lower extremities: femoral bifurcation, tibial hemimelia, and diastasis of the ankle. Case report and review of the literature. In: The Journal of bone and joint surgery. American volume. Vol. 66, No. 3, March 1984, pp. 453-458, ISSN  0021-9355 . PMID 6699064 .
  4. ^ F. Forzano, V. Viassolo, M. Castagnetta, S. Cavani, L. Battistuzzi, E. Garbati, MC Emiliozzi, A. Cecchi, F. Faravelli, M. Lituania: Prenatal diagnosis of Gollop-Wolfgang Complex. In: Prenatal diagnosis. Vol. 29, No. 7, July 2009, pp. 724-726, ISSN  1097-0223 . doi: 10.1002 / pd.2269 . PMID 19353531 .

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