Discordant encephalocele in monozygotic twins.
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The developmental pathogenesis of cranium bifidum and exencephaly, as well as other forms of the dysraphic state, remains unresolved. The process of neuronal maturation under such conditions is the major definitive factor for functional prognosis and may be the key to the embryopathogenesis. The neuronal maturation processes in cranium bifidum and anencephaly were analyzed in both human neonatal autopsy and surgical specimens, utilizing the technique of immunohistochemical morphological analysis. The results suggest that anencephaly is a form of neuroschisis, a defect in which the neuronal developmental process arrests and results in dys- or rather aplastic cortical architecture. In contrast, although the cortical architecture is often distorted, neuronal maturation in cases of cranium bifidum cysticum is primarily complete, demonstrating normal cerebral cortical layers and NSE positive neural elements. In conclusion, it is strongly suggested that anencephaly is the most severe form of cranium bifidum, as is myeloschisis in spina bifida, without supportable neuronal maturation. Recognizing these points in the developmental stages with regard to the degree of neuronal maturation, a reclassification of cephalic dysraphism is proposed. The embryopathogenetic and epidemiologic aspects of this proposal of reclassification are discussed.
Meningomyelocele/encephalocele with associated ventriculomegaly can be treated as a single-stage procedure (i.e., both lesions treated simultaneous) or as two-stage procedures (i.e., each lesion treated at a separate time). A delay in closure of the meningomyelocele/encephalocele is associated with a higher incidence of ventriculitis/ventricular shunt infection-particularly when closure is performed more than 36 h after birth. In these situations, closure followed by surveillance cultures, appropriate antibiotics, ventricular drainage, and then delayed ventricular shunting seems more reasonable.
A patient with unilateral preauricular appendages and occipital mass is described who died on the fifth day of life. An autopsy demonstrated congenital megabladder and megaureters and dysplastic kidneys. The neuropathological examination revealed cranium bifidum in the occipital region, defective posterior arch of the C1 vertebral body, agenesis of vermis and a large cerebellocele. It is suggested that some patients with the Goldenhar-Gorlin syndrome may have prominent central nervous system involvement in spite of only relatively slight facial involvement.
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We present a case of trans-sphenoidal encephalomeningocele in association with a posterior cranial fossa malformation which fulfils the criteria for the Dandy-Walker complex [1]. Congenital cardiovascular defects were also present. An abnormality of neural crest development may be responsible for the combined occurrence of these anomalies.
Chiari malformations are brain stem anomalies with or without spinal canal involvement. The combination of brain stem and cerebellar anomaly with a cervical cele is named Chiari III malformation. Patients with this extremely rare defect frequently present with severe neurological defects and can have a poor prognosis. Following a report on a newborn child with a Chiari III malformation that took a fatal but typical course the literature is reviewed.
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Microvascular reconstruction of a cranial defect in a 10-week-old infant, which is the youngest case in the literature, is reported. A latissimus dorsi free muscle flap was transferred to cover the defect and a split thickness skin graft was placed over the muscle flap. Despite the successful flap transfer in this case, microvascular reconstruction in an infant has its own risks and infants should be discussed as a separate entity apart from the other pediatric patients.