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Diaphragmatic defect-limb deficiency-skull defect syndrome

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Disease definition

A rare genetic multiple congenital anomalies/dysmorphic syndrome characterized by diaphragmatic hernia, lung hypoplasia, ossification defect of the skull, and severe limb hypoplasia. Other clinical features may include, syndactyly, clinodactyly, extra spleen, absence of the femur or pelvic bone, partial intestinal malrotation, omphalocele and testicular atrophy.

ORPHA:2141

Classification level: Disorder

Synonym(s):
  • Froster-Huch syndrome

Prevalence: <1 / 1 000 000

Inheritance: Unknown

Age of onset: Antenatal

ICD-10: Q87.8

OMIM: 601163

UMLS: C4303589

GARD: 2397

A summary on this disease is available in Français, Español, Deutsch, Italiano, Português, Nederlands, Polski
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2020.pdf) - Orphanet Urgences
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