Pseudoainhum syndrome L94.6

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Amniotic band syndrome; Amniotic rupture sequence

History
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Portal 1685; Streeter 1930; Neumann 1953

Definition
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Constriction or constriction of limbs (especially fingers or toes) by inelastic, fibrotic, scarred strands of the amniotic sac as a rare phenomenon of numerous underlying diseases (in contrast to the idiopathic Ainhum syndrome). Can lead to spontaneous amputation.

Occurrence/Epidemiology
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Clinical features
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No uniform constellation. Various malformations may be present at birth, e.g. constrictions or amputations of fingers, toes or whole extremities. Furthermore rudimentary fingers and toes, syndactylia, clubfoot, cleft lip and jaw. Rarely thorax, head, soft tissue or umbilical cord are affected.

Differential diagnosis
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Ainhum Syndrome (not congenital!)

Therapy
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If possible, plastic reconstructive surgical operations.

Prophylaxis
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Prenatal diagnostics by means of high-resolution ultrasound.

Note(s)
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Ainhum = East African naga language, for sawing or cutting.

Literature
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  1. Ena P, Pinna A (2003) Lamellar ichthyosis associated with pseudoainhum of the toes and eye changes. Clin Exp Dermatol 28: 493-495
  2. Neumann A (1953) Pseudoainhum: report of a congenital case involving several fingers and the elft wrist. Arch Dermatol Syphiol 68: 412-427
  3. Stinco G et al (2003) Complications of pseudoainhum. dermatologist 54: 163-166
  4. Wollina U et al (2001) Pseudoainhum of all fingers associated with Reynolds' syndrome and breast cancer: report of a case and review of the literature. J Am Acad Dermatol 44: 381-384

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020