The authors report two-sibling case of Adams Oliver syndrome. One was managed with early surgical intervention after a period of failed conservative management and the other conservatively managed until reconstruction and surgical correction of alopecia. The goal of treatment is to achieve a complete closure of the defect without encountering any fatal complications such as haemorrhage and infection or causing any long-term neurological morbidity. Various papers have been published related to ACC, yet there is no consensus on the therapeutic approach. The management decision hinges upon balancing the risks of complications including spontaneous sagittal sinus haemorrhage and the risk of surgical intervention. Both treatment modalities proved successful in these cases.
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