sacral defect with anterior meningocele
Thursday 9 February 2006
Definition: Herniation of the meningeal sac into the extraperitoneal pelvic region through a congenital defect in the sacrum. Sacral defect with anterior meningocele (SDAM) is a type of caudal dysgenesis. The inheritance is autosomal dominant.
SDAM is present at birth and becomes symptomatic later in life, usually because of obstructive labor in females, chronic constipation, rectal fistula and abscess, or meningitis.
The herniated sac is composed of two layers: the outer dural sac, and the inner arachnoid membrane. The sac contains cerebro-spinal fluid with no neural elements. It also called Type 1 extradural meningeal cyst (Occult sacral meningocele) according to Nabors M.C et. al’s classification.
Anterior sacral meningocele involve mostly females with reported incidence of 79%. It represents 2.8% of all presacral tumors in females.
The genetic inheritance pattern has not been ascertained. An X-linked dominant pattern was proposed. In most cases with family recurrence, autosomal dominant transmission is suggested.
The cytogenetic defect site was reported to be linked to the HLA in a location, 7q36, which also contain a gene for holoprosencephaly. However, Chatkupt et al. could not confirm the HLA region as the location for the sacral defect anterior meningocele in members of five generation family with this disorder.
sacral bone defect
proliferation of arachnoid
connective tissue diseases
- Marfan disease
- nerve root avulsion or hemorrhage
Differential diagnosis: presacral cysts
- presacral dermoid cyst
- presacral lipoma
- presacral neuroblastoma
presacrla meuroectodermal cyst
sacral meningeal cyst
- extradural cyst without spinal nerve root fibers
- presacral meningeal cyst / presacral arachnoid cyst
- occult Sacral Meningocele.
- extradural cyst with spinal nerve root fibers
- perineural cyst (Tarlov cst)
- spinal nerve root diverticulum
- intradural cyst (intradural arachnoid cyst)
combination of anterior sacral meningocele , perineural cyst and intrasacral meningeal cyst
gastro intestinal tract:
- rectal dilatation
- anorectal atresia
- Hirschprung Disease
- meconium plug syndrome
renal cyst in pelvic kidney
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