Cauterização endoscópica do plexo coroide no tratamento da Hidranencefalia e Hidrocefalia Extrema

Abstract

Introduction Hydranencephaly is characterized by the absence of cerebral hemispheres keeping intact the meninges and skull. Extreme hydrocephalus represents the highest degree of ventricular dilation preserving a thin cortical mantle mainly at the frontal region. The standard treatment for these entities consists on implanting of ventricular derivations to the peritoneum though complications happen frequently. The cauterization of the plexus choroid (CPC) comes as an alternative, but the long term efficacy and the associated factors to success and failure of the treatment at controlling cephalic perimeter (CP) are not yet well defined in literature. Method Forty two children who underwent CPC due to extreme hydrocephalus and hydranencephaly at Hospital das Clinicas or Hospital da Baleia were retrospectively evaluated from 2006 to 2014. Children with follow up inferior to three months were excluded. The first part of the work evaluated the possible influence of variables on sucess rate (presence of intrauterine infection, kind of disturbance in the liquor accumulation, childs age and CP at the moment of surgery and birth, performance or not of the third ventriculostomy in cases of extreme hydrocephalus). In the second part of the work the pre operation computerized tomography images were compared to the intraoperative anatomic aspects. Two anatomic patterns were created named classic and destructive. The anatomic variables were compared to the treatment sucess rate. Results Thirty four children were followed closely for more than three months and were considered for effectiveness analysis. Treatment was sucessfull in 24 children (70,6%) and failure occurred in 10 (29,4%). Failure was detected soon after the endoscopic procedure (average 116 days). There was no difference at effectiveness when compared the age at the moment of surgery (p=0,473), kind of malformation (p=1), CP at birth (0,699), CP at the moment of surgery (p=0,648). The performance of the third ventriculostomy did not significantly increase the effectiveness of the procedure (p=0,564). Eighteen patients with over three months follow up had videos and head imaging which allowed making anatomic analysis. There was no significant difference at effectiveness of the procedure in relation to a morphologic classification (classic x destructive, p=0,6310) neither in relation to plexus choroid volume (p=0,321). Surgical death rate was 7,14%. Conclusion The endoscopic cauterization of the plexus choroid was a valid procedure at handling hydranencephaly and extreme hidrocephaly and showed effectiveness in 70,6% of cases with an average follow up of 32 months. The failures, when existed, were early. None of the analyzed variables interfered in the success of the treatment.