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Diaphragmatic defect, limb deficiency, skull defect syndrome (disorder)
Diaphragmatic defect, limb deficiency, skull defect syndrome
Froster Huch syndrome
Syndrome with the association of classical diaphragmatic hernia (Bochdalek type), severe lung hypoplasia and variable associated malformations. It has been reported only once in four successive fetuses (two females and two males) born to a nonconsanguineous couple. The spectrum of malformations is wide and includes omphalocele (one case), severe limb hypoplasia (two cases), syndactyly of the toes (two cases), extra spleen (one case) and an ossification defect of the skull (one case).
syndroom van diafragmadefect, hypoplasie van ledematen en schedeldefect
syndroom van Froster-Huch
Id721095007
StatusPrimitive
Associated morphologyHernia
Finding siteAbdomen
OccurrenceCongenital
Associated morphologyHernial opening
Finding siteStructure of foramen of Bochdalek
OccurrenceCongenital
Pathological processPathological developmental process
Associated morphologyDefect
Finding siteDiaphragm structure
OccurrenceCongenital
Pathological processPathological developmental process
Associated morphologyHypoplasia
Finding siteLung structure
OccurrenceCongenital
Pathological processPathological developmental process
DHD Diagnosis thesaurus reference set
ICD-10 complex map reference set
TargetQ87.8
RuleTRUE
AdviceALWAYS Q87.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified