Cervical spinal cord infarction after posterior fossa surgery: a case-based update.

BACKGROUND: Several positions are currently utilized for operating patients with posterior fossa lesions. Each individual position has its own risks and benefits, and none has demonstrated its superiority. A dreaded, and probably underreported, complication of these procedures is cervical cord infarction with quadriplegia. DISCUSSION: We reviewed eight previous reported instances of this devastating complication aimed at ascertaining its pathogenesis to suggest preventive strategies. Several hypotheses have been put forward to explain the occurrence of this complication. Some factors involved in the production of cervical cord infarction include patient's position (seated or prone), hyperflexion of the neck, excessive spinal cord traction, canal stenosis, and systemic arterial hypotension. We hypothesize that spinal cord infarction in our patient might have resulted from compromised blood supply to the midcervical cord caused by tumor infiltration of the cervical leptomeninges in addition to a brief episode of arterial hypotension during venous air embolism. CASE REPORT: We treated an 8-year-old girl who developed quadriplegia after surgery for a fourth ventricular ependymoma. Postoperative magnetic resonance imaging demonstrated cervical cord infarction. Evoked potentials confirmed the diagnosis. CONCLUSIONS: With this report, we want to draw the attention of neurosurgeons to the possibility of the occurrence of this dreadful complication during posterior fossa procedures. Retrospectively, the only measures that might have helped to avoid this complication in our patient would have been using the prone position and intraoperative monitoring of evoked potentials.

CSF overdrainage in shunted intracranial arachnoid cysts: a series and review.

BACKGROUND: Cerebrospinal fluid (CSF) overdrainage in hydrocephalus is well recognized. Overshunting following cysto-peritoneal (CP) drainage in patients with arachnoid cysts (AC) is rarely documented. AIM: We report five patients with acquired Chiari malformation I and three with posterior fossa overcrowding due to excessive CSF drainage in shunted intracranial ACs. We review our observations and discuss the current knowledge on the pathogenesis and management of this complication. PATIENTS AND METHODS: The medical records of the eight patients were analyzed in regard to clinical manifestation, cyst and shunt characteristics, management, and outcomes. RESULTS: Mean age of the patients was 5.5 years. After an average interval of 5 years, five patients developed symptoms related to hindbrain herniation and three to severe shunt overdrainage following CP shunting. Several management modalities were utilized that achieved a good result in seven instances. DISCUSSION: Some shunted ACs may evolve with overdrainage syndromes. Posterior fossa overcrowding and tonsillar herniation constitute their most severe forms. CSF hypotension, bone changes, venous engorgement, and probably cerebral chronic edema at the posterior fossa constitute the main factors involved in the pathogenesis of this entity. We also review previous instances of acquired Chiari malformation originating after AC shunting. CONCLUSIONS: Posterior fossa overcrowding and acquired Chiari I malformation can develop after excessive CSF drainage of intracranial ACs. Overshunting manifestations require prompt recognition and management. Preventive measures consist of making a stringent selection of cases being considered for surgery, avoiding CP drainage, and placing of a programmable valve as initial treatment of intracranial ACs if shunting is considered.

Functional assessment of intracranial arachnoid cysts with TC99 m-HMPAO SPECT: a preliminary report.

BACKGROUND: Many arachnoid cysts (AC) are detected incidentally in asymptomatic patients. Current neuroimaging methods provide only morphological details of the cysts, but they do not give information about cerebral function. While surgery is indicated in symptomatic patients, the management of asymptomatic individuals, who present with large cysts, is controversial. STUDY OBJECTIVE: To ascertain the value of cerebral 99 mTc-HMPAO single photon emission computed tomography (SPECT) for detecting brain dysfunction in cases of intracranial ACs, aimed at allocating the patients for surgical or for conservative treatment. PATIENTS AND METHODS: We studied prospectively 11 patients diagnosed with sylvian fissure ACs. The subjects underwent neurological examination, EEG, neuroimaging studies, neuropsychological testing, and cerebral perfusion studies with 99 mTc-HMPAO SPECT. RESULTS: The patients' ages ranged from 2 to 42 years (median 16 years). The study group consisted of ten symptomatic patients with ACs and one patient with an incidental cyst. Seven patients showed diminished regional cerebral blood flow (rCBF) in their initial cerebral SPECT. Four individuals underwent surgery. Seven patients showed normalization of rCBF after surgical or conservative treatment. CONCLUSIONS: Cerebral SPECT demonstrated impaired brain perfusion in 70% of symptomatic patients. The zone of decreased rCBF corresponded well with clinical symptoms and with neuroimaging findings. Patients exhibiting normal rCBF in SPECT studies remained or became asymptomatic during the follow-up time. Cerebral SPECT constitutes a valuable adjunct tool for correlating regional function with brain anatomy, and may be of help to allocate patients with ACs for surgical treatment or clinical observation. Further research on this field is warranted.

Hydrocephalus in intraspinal dermoids and dermal sinuses: the spectrum of an uncommon association in children.

BACKGROUND: Hydrocephalus associated with intraspinal tumors constitutes a well-documented occurrence. The accepted mechanisms for this association seem to be well established. On the contrary, hydrocephalus in the context of intraspinal dermoids has been rarely recognized and its pathogenetic mechanism appears to be different. PATIENTS AND METHODS: The authors report four pediatric cases of spinal dermoid tumors and dermal sinuses that developed hydrocephalus during the evolution of these congenital lesions of ectodermal origin. DISCUSSION: In two children, the mechanism leading to the development of hydrocephalus consisted of leptomeningeal inflammation due to bacterial meningitis or to spillage of dermoid cyst contents in the cerebrospinal fluid spaces. We hypothesize that ventricular dilatation in the other two might be the result of chemical meningitis occurring during intrauterine life. Two cases developed ventriculomegaly prior to the diagnosis of their spinal cord disease. CONCLUSIONS: The unusual evolution of these cases suggests that neuroimaging studies that include the spine should be performed in cases of childhood "unexplained hydrocephalus".

Positional skull deformities in children: skull deformation without synostosis.

BACKGROUND: Patients with craniosynostosis are readily diagnosed by clinical and neuroimaging findings. Surgical treatment is indicated for preventing neurological deficits and for correcting esthetically unacceptable head deformities. In recent years, we have witnessed a progressive number of neurosurgical consultations for abnormal head shapes unrelated to premature fusion of the cranial sutures, especially of positional plagiocephaly. There have been descriptions of abnormalities in the cerebrospinal fluid (CSF) spaces in children with craniosynostosis. OBJECTIVES: The aim of the present study was to investigate the role of the changes of the CSF spaces in the development of positional skull deformities in children. PATIENTS AND METHODS: The authors reviewed demographic, clinical, and neuroimaging data of 23 patients assessed for some form of nonsynostotic skull deformity (group A). The results were compared with those of a simultaneous group of nine infants diagnosed with benign extracerebral collections of fluid (group B). RESULTS: The study group was composed of 11 boys and 12 girls, aged 3 years or younger. Seventeen children had plagiocephaly, four scaphocephaly, and two brachycephaly. Sixteen children (15 with plagiocephaly and 1 with brachycephaly) exhibited enlarged subarachnoid CSF spaces. In group B, the boy/girl ratio was of 7:2. Infants in group A presented at an older age (mean 12.7 months) than group B (mean age at presentation of 7.17 months). Children with benign extracerebral fluid collections were born with a head circumference (HC) greater than infants with positional skull deformities (p=0.005). The percentile of the children's HC at consultation was also larger for children of group B (p=0.03). The form of clinical presentation differed between the two groups. Most infants of group A were seen because of a type of head deformity, and children in group B were studied for macrocephaly. Long-term follow-up assessment showed better outcomes for patients in group B than for children of group A in regard to regression of initial symptoms (p=0.03). CONCLUSIONS: Most positional head deformities appear to be related with the children's positioning for sleeping. We have not confirmed macrocephaly as a contributing factor for positional deformities. The distribution of extracerebral CSF and the presence of abnormal collections of fluid in children with positional head deformities do not seem to be related with the findings of pericerebral CSF encountered in children with benign extracerebral collections of fluid. In our view, brain pulsations, transmitted to these accumulations of CSF, play an important part in the development of the infants' skull deformities (p=0.02). The findings of enlarged CSF spaces in children with nonsynostotic skull deformation constituted an age-related event, as these collections tended to disappear as the children grew older (p=0.04).