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Accessory arm--dysraphism or disparity? Case report.

The case of a 3-month-old infant with an accessory third arm is reported. The extra appendage was attached at the midcervical region and was associated with posterior cervical dysraphism and a cervical cord lipoma. Possible theories of origin are examined.

Arm↗

Cutaneous signs of neural tube dysraphism.

Cutaneous markers have a crucial role in the detection and diagnosis of occult neural tube dysraphism. Recognition of these stigmata and appropriate radiologic imaging decrease the long-term morbidity of this condition.

Child, Preschool↗

Hydrocephalus in the child with dysraphism.

Hydrocephalus develops in approximately 85% of patients with myelomeningoceles and appears most frequently to result from obstruction to cerebrospinal fluid (CSF) flow within the posterior fossa subarachnoid space and at the tentorial hiatus, due to posterior fossa crowding from the Chiari malformation. The presenting features of hydrocephalus are legion; although signs and symptoms of intracranial hypertension are most common, many patients exhibit more subtle and confusing signs, such as intellectual deterioration or behavioral changes, or those that mimic brainstem compression from the Chiari malformation or spinal cord dysfunction due to tethering or syringomyelia. Prompt recognition of these signs and symptoms will direct appropriate therapy toward shunt revision and will help the clinician avoid potentially unnecessary and more dangerous procedures.

Central Nervous System↗

The renal axes in spina bifida: issues of confusion and fusion.

The occurrence of horseshoe kidneys in myelodysplasia has been suggested to be overestimated because of the concurrent prevalence of kyphotic spine in the spina bifida population. Pseudohorseshoe kidneys result from the actual medial migration and apposition of the lower renal poles in the deep fossa created by the gibbus deformity. The presence of a lumbosacral kyphosis, however, does not imply there is not a true horseshoe kidney. In our myelodysplasia population of 189 patients, occurrence of true horseshoe kidneys (13 patients) and pseudohorseshoe kidneys (14 patients) was increased. The finding of lumbosacral kyphosis was twice as common in the pseudohorseshoe population as in the true horseshoe population. True horseshoe kidneys were three times more commonly seen in association with congenital vertebral anomalies cephalad to the dysraphic spine. Diagnosis in this study was predominantly based on renal cortical scintigraphy with the posterior pin-hole magnification technique.

Child↗

Meningocele manqué: radiologic findings with clinical correlation.

PURPOSE: To determine whether meningocele manqué can be detected by neuroimaging techniques in dysraphic patients. METHODS: We reviewed the records and imaging studies of 16 patients with surgically proved meningocele manqué seen at our institution between 1989 and 1990. Both CT and MR imaging techniques were used. CT of the spine was performed immediately following contrast myelography. RESULTS: Nine of 16 patients (CT, four; and MR, five) showed evidence of meningocele manqué which corresponded to intraoperative findings. Fourteen of 16 patients were found to have diastematomyelia, eight with medium septum and six without a septum. Associated findings included syrinx (six), lipoma (five), dermoid cyst (one), and neuroenteric cyst (one). After completing this review, we were able to prospectively diagnose dorsal bands in two new patients; these bands were confirmed at surgery. CONCLUSION: Dorsal bands can be detected in dysraphic patients with CT or MR using operative findings as a road map.

Adolescent↗