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Spigelian hernia-cryptorchidism syndrome

ORPHA:314432· ICD-10 K43.6

Definition

Spigelian hernia-cryptorchidism syndrome is a rare developmental defect during embryogenesis characterized by a ventral, uni- or bilateral protrusion of extraperitoneal fat, peritoneum and/or intra-abdominal organs through a defect in the spigelian fascia (Spigelian hernia), associated with ipsi- or bilateral undescended testis (usually found within or just beneath the hernial sac) in male neonates. The gubernaculum and/or inguinal canal may be absent.

Prevalence
<1 / 1 000 000
Inheritance
Not applicable
Age of onset
Infancy, Neonatal