Perioperative treatment meningeoma complicated by intracerebral haemorrhage in patient with Klippel-Trenauney syndrome - case report

S.Šušnjar1, D.Rakanović1, M.Miškić2

1Clinic for anaesthesiology and intensive therapy, University clinical center of the Republic of Srpska - Banjaluka (Bosnia and Herzegovina), 
2Department of neurosurgery, University clinical center of the Republic of Srpska - Banjaluka (Bosnia and Herzegovina)

Background:Klippel-Trenauney syndrome (KTS) is a syndrome characterized by cutaneus hemangiomas (port-wine stains), abnormal growth of soft tissues and bones, and venous malformations with tendency to bleeding or thrombosis. The association of KST with intracranial tumors is extremely rare and is described as a sporadic case in the literature.

Case Report:Patient in our case was a female, 41 years old, with KTS and a meningeoma complicated by postoperative intracerebral haemorrhage. The patient was hospitalized for partial seizures and right hemiparasis (Todd's paralysis). After diagnostic treatment (CT and MR), the diagnosis of intracranial tumor was established. A surgery was performed in a balanced anesthetic technique (Sevofluran-Fentanyl-Atracurium). Immediate postoperative period went without complications – patient was conscious, haemodynamically stable, spontaneously breathing, without neurological deficits. During the second postoperative day, the neurological condition of the patient had worsened with loss of consciousness and the development of right-sided hemiplegia. The control CT scans showed intracerebral hemorrhage with generalized edema and threatening transententoral herniation. The patient was put on mechanical ventilation, conservatively treated (antiedematous and corticosteroid therapy) and introduced into the barbiturate coma (Tiopental). Four days later, Tiopental was disconnected. On the seventh day, the patient awoken - conscious, stable vital signs, weaned from mechanical ventilation with residual right-sided hemiparasis. Control CT scans showed a decrease in the size of the hematoma and brain edema. The ninth day patient was transferred to the neurosurgery department for further treatment.

Discussion:The association of KTS with intracranial lesions requiring surgical treatment presents the challenge of the entire surgical and anesthetic team. Venous and capillary malformations lead to significantly frequent bleeding intraoperatively and postoperatively, which requires the administration of transfusion and prolongs the postoperative recovery and duration of treatment.
 
References: 1. TevfikYilmaz, UlasCikla, Alice Kirst and Mustafa K Baskaya. Glioblastoma multiforme and Klippel-Trenaunay-Weber syndrome: a case report. Journal of Medical Case Reports (2015) 9:83.

Learning points: It is necessary to pay attention in postoperative period due to early recognition and effective treatment of complications.


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