Neurological signs and operative indication by agenesis of the perisylvian region: a study of 13 operated cases

Sinais neurológicos e indicação operatória em casos de agenesia da região perisilviana: estudo de 13 casos operados

Neurological signs and operative indication by agenesis of the perisylvian region: a study of 13 operated cases

Sinais neurológicos e indicação operatória em casos de agenesia da região perisilviana: estudo de 13 casos operados

E. Markakis^I; F. Theophilo^II; R. Heyer^III; L. Stoeppler^IV

^IProfessor of Neurosurgery. Neurosurgical Clinic, Hannover Medical School, West Germany (Head: Prof. Dr. med. Dr. h.c. Hermann Dietz)

^IIAssistent of the Neurosurgical Clinic. Neurosurgical Clinic, Hannover Medical School, West Germany (Head: Prof. Dr. med. Dr. h.c. Hermann Dietz)

^IIINeuropediatrician. Neurosurgical Clinic, Hannover Medical School, West Germany (Head: Prof. Dr. med. Dr. h.c. Hermann Dietz)

^IVNeuroradiologist. Neurosurgical Clinic, Hannover Medical School, West Germany (Head: Prof. Dr. med. Dr. h.c. Hermann Dietz)

SUMMARY

The article reports on 13 cases of agenesis of the perisylvian region in patients aged 2 to 53 years. The agenesis occurred in the temporal operculum in 7 cases, in the frontal operculum in 5 cases and the whole fronto-parieto-temporal region in one case. The agenesis was always associated with space requiring arachnoidal cysts. Symphyses and obstruction of the flow of the basal CSF spaces were also seen. In 85% of the patients were males. The patients tend to decompensate after light cerebrocranial trauma, and in many cases surgery reveals a subdural haematoma which fills the aplastic region, due to detachment of the exposed cerebral veins effecting basal drainage. In the remaining cases, surgery was performed because of general signs of cerebral compression, exarcebation of an existing disease associated with attacks, or because of the space occupying character of the concomitant arachnoidal cysts. In patients with agenesis of the perisylvian region without space occupying arachnoidal cysts, surgery is not performed for the time being, instead, regular neurological and CAT-Scan control is effected.

RESUMO

O artigo trata de G3 casos de agenesia da região perisilviana em pacientes de 2 a 53 anos. A aplasia foi encontrada com sua maior extensão no lobo temporal em 7 casos, na região fronto-temporal em 5 casos, e um caso abrangendo toda a região fronto-parieto-temporal, todos os casos acompanhados de cisto aracnóideo de caráter expansivo. Também foram encontrados aderências e distúrbios da circulação liquórica a nivel das cisternas basais. Em todos os casos, com exceção de dois, localizava-se a aplasia do lado esquerdo; o sexo masculino é predominante (11 casos) atingindo o sexo feminino em nossa série em apenas dois casos. Os pacientes apresentam uma tendência a descompensação após traumatismos cranio-encefálicos leves, e em muitos casos foram encontrados hematomas subdurals ocasionados pela ruptura de veias cerebrais que se encontravam soltas sobre o cisto aracnóideo. Nos casos restantes a operação foi realizada por causa da existência de pressão intracraniana elevada, exacerbação de crises convulsivas ou por causa do caráter expansivo do cisto aracnóideo concomitante. Em pacientes com agenesia da região perisilviana porém com cistos aracnóideos que não apresentam caráter expansivo, não é indicado o tratamento cirúrgico e sim o acompanhamento neurológico e repetição da tomografia computadorizada em espaços de 6 a 12 meses.

ZUSAMMENFASSUNG

Es wird über 13 Fälle mit Aplasie der perisylvischen Region berichtet bei Patienten im Alter von 2 bis 53 Jahren. Die Aplasie betraf das temporale Operculum in 7 Fälle, das frontale Operculum in 5 Fälle und die ganze fronto-parieto-temporale Region in einem Fall. Sie wurde in alien Fallen von raumfordernden Arachnoidalzysten begleitet. Ebenfalls lagen Verwachsungen und Abflussbehinderungen der basalen Liquorrãume vor. Zu 85% war die Aplasie linksseitig lokalisiert, ebenso häufig wurde das männliche Geschlecht betroffen. Die Patienten dekompensieren leicht nach einem Schadelhirntrauma, oft findet sich bei der Operation ein den aplastischen Bereich ausfüllendes subdurales Hämatom wegen Abrises der frei liegenden basal drainierenden Hirnvenen. Bei den restlichen Fällen wurde die Operation wegen allgemeiner Hirndrucksymptomatik, Verschlimmerung eines bestehenden Anfallsleidens oder wegen des raumfordenden Charakters der begleitenden Arachnoidalzysten durchgeführt. Bei Patienten mit perisylvischer Aplasie ohne raumfordernde Arachnoidalzysten wird eine Operation zunüchst nicht durchgeführt, sondern es findet die regelmässige neurologische und computer-tomographische Kontrolle statt.

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Neurochirurgische Klinik der medizinischen Hochschule Hannover - Karl Wiechert Allee 9 - 3000 Hannover 61 - Deutschland.

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Publication Dates

• Publication in this collection
  23 Aug 2012
• Date of issue
  Dec 1981