Lethal multiple pterygium syndrome

Lethal multiple pterygium syndrome (LMPS) is a lethal sub type of the multiple pterygium syndrome.

It is primarily characterised by the presence of pterygia (or wings) present in two or more body areas overlying predominantly the large joints. Accompanying joint contractures are almost always present.

Genetics

The majority of cases carry an autosomal recessive inheritance while a small proportion of cases may carry an X linked recessive inheritance.

Associations
Antenatal ultrasound

Described features are wide and include

Ancillary sonographic features that may be present include

As the name implies, the condition is fatal with fetal death occurring usually by mid-gestation 3.

Consider
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Article Information

rID: 13483
Synonyms or Alternate Spellings:
  • Lethal multiple pterygium syndrome (LMPS)

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