Two unusual cases of neurocutaneous melanosis are presented. Both patients had congenital giant hairy nevi and both developed hydrocephalus, seizures, and myelopathy. The first patient displayed multicentric cerebral and spinal cord melanosis, as opposed to the more commonly described basilar leptomeningeal involvement. The second patient had total spinal leptomeningeal involvement, and ventriculoperitoneal shunting for hydrocephalus produced peritoneal metastasis of melanoma. An individual born with a congenital giant hairy nevus or marked generalized cutaneous pigmentation should be closely observed for the development of malignant melanoma of the nervous system.
The authors describe the case of a prematurely born infant who developed hydrothorax after ventriculoperitoneal (VP) shunt placement for treatment of posthemorrhagic communicating hydrocephalus. Prior to shunt placement a bout of necrotizing enterocolitis created intense abdominal and peritoneal scarring. The authors postulate that the scarring created poor peritoneal absorption capacity of cerebrospinal fluid (CSF), with preferential flow of CSF from the peritoneal to the pleural cavity. A (99m)Tc-diethylenetriamine pentaacetic acid radionucleotide study enabled the authors to rule out shunt malfunction, and preferential transdiaphragmatic flow of CSF from the abdomen to the thoracic cavity was demonstrated. The hydrothorax resolved after conversion of the VP shunt to a ventriculoatrial shunt. Respiratory distress after VP shunt placement should be considered an unusual but important sentinel symptom in the differential diagnosis of postoperative shunt complications.
We have observed two patient groups with seizure activity as the primary presentation of shunt malfunction. Eight patients had a first-time seizure, and seven patients had a history of prior seizures, none more recently than 1 year prior to the seizure recurrence. Five of seven patients with prior seizures who seized were on anticonvulsant medication, three of these seven patients had therapeutic anticonvulsant levels. Shunt malfunction was diagnosed via a combination of tests. The electroencephalograms of nine patients were diffusely abnormal with regions of slowing and focal spike activity. Seizure activity stopped in all patients after preoperative stabilization with anticonvulsant medication and shunt revision. These 15 patients represent 2% of all patients treated for shunt malfunction during a 3-year period. The assessment of new or recurrent seizure activity in a previously stable shunted patient should include evaluation of shunt function whatever the anticonvulsant levels.
We report a 3-month-old infant who became paraplegic from an epidural hematoma caused by a diagnostic lumbar puncture for work-up of sepsis. The differential diagnosis of the cause of paraplegia was epidural hematoma formation versus spinal abscess. Hemophilia A was diagnosed when coagulation studies were discovered to be abnormal, and non-contrast CT scan revealed an epidural mass with spinal cord displacement. The coagulopathy was rapidly corrected preoperatively with an infusion of cryoprecipitate. A medially limited bilateral T8-L4 laminectomy allowed complete evacuation of the hematoma with maximum preservation of normal bone tissue, but no clinical improvement resulted. Coagulopathy should be highly suspect in an infant who becomes paraplegic after lumbar puncture. The coagulopathy may be rapidly corrected with deficient factor replacement, allowing major spinal surgery to be performed safely.
Early surgery to repair sciatic nerve injury possibly promotes significant pain reduction, reduces narcotic usage and facilitates a long rehabilitation process. Allograft nerve placement is not associated with serious complications. A follow-up period longer than 3 yr would be required and is ongoing to assess the efficacy of our treatment of patients with combat-acquired sciatic nerve injury.
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