A rare case of giant terminal lipomyelocystocele with partial sacral agenesis

Abstract

Terminal myelocystoceles (TMCs) are a rare form of occult spinal dysraphism. They constitute approximately 5% of skin covered lumbosacral masses. The TMC is composed of a low-lying conus medullaris with cystic dilatation of caudal central canal, a surrounding meningocele and a lipoma that extends from the conus to a subcutaneous fat collection. A 6-month-old female child presented with a large lumbosacral mass (14 cm × 12 cm × 10 cm) and weakness at the ankle joints since birth. Magnetic resonance imaging and computed tomography scan revealed a low-lying terminal lipomyelocystocele with holocord syrinx and partial sacral agenesis. The child was operated on successfully. This is an interesting and rare case of giant terminal lipomyelocystocoele with syrinx and a partial sacral agenesis. Terminal lipomyelocystocele should be included in the differential diagnosis of congenital lesions presenting as a lumbosacral mass and operated early.

Giant terminal myelocystocele: A case report

“…The patient was paraplegic at time of presentation. Patients with giant TMC, as described in other case reports,[ 1 9 10 11 ] also had motor deficits in lower limb but none of them were paraplegic. Neurological deficits were reported in 47% patients by Gupta[ 12 ] in their series of 17 cases with TMC.…”

“…Patients with giant TMC, as described in other case reports,[ 1 9 10 11 ] also had motor deficits in lower limb but none of them were paraplegic. Neurological deficits were reported in 47% patients by Gupta[ 12 ] in their series of 17 cases with TMC. Interestingly, Pang[ 5 ] in their study of 10 patients with TMC (four newborns and six older children) found that all the newborns were neurologically normal, whereas all the older children had neurological deficits.…”

"Giant" terminal myelocystocele: A rare variant of spinal dysraphism

“…"Giant" variant of TMC was not described by Pang et al [10] but reported by others. [2,3] Magnetic resonance imaging is characterized by a "trumpet like" flaring of the distal cord central canal into an ependymal lined terminal cyst. Abnormalities of the vertebral column may occur and include lordosis, scoliosis, and agenesis of the sacral parts.…”

“…[ 2 ] Here, we present an interesting case of “giant” TMC, only rarely reported in the literature. [ 2 3 ]…”