ABSTRACT
BACKGROUND: Hydrocephalus caused by unilateral foramen of Monro (FM) obstruction has been referred to in literature by many different terminologies. Precise terminology describing hydrocephalus confined to just one lateral ventricle has a very important prognostic value and determines whether or not the patient can be shunt free after an endoscopic procedure. METHODS: Aiming to define the best term for unilateral FM obstruction, 19 terms were employed on PubMed database (http://www.ncbi.nlm.nih.gov/pubmed) as quoted phrases. RESULTS: A total of 194 articles were found. Four patterns of hydrocephalus were discriminated as a result of our research term query and were divided by types for didactic purpose. Type A - partial dilation of the lateral ventricle; Type B - pure unilateral obstruction of the FM; Type C - previously shunted patients with secondary obstruction of the FM; and Type D - asymmetric lateral ventricles with patent FM. CONCLUSION: In unilateral FM obstruction hydrocephalus, an in-depth review on terminology application is critical to avoid mistakes that may compromise comparisons among different series. This terminology review suggests that Type B hydrocephalus, i.e., the hydrocephalus confined to just one lateral ventricle with no other sites of cerebrospinal fluid circulation blockage, are best described by the terms unilateral hydrocephalus (UH) and monoventricular hydrocephalus, the first being by far the most popular. Type A hydrocephalus is best represented in the literature by the terms uniloculated hydrocephalus and loculated ventricle; Type C hydrocephalus by the terms isolated lateral ventricle and isolated UH; and Type D hydrocephalus by the term asymmetric hydrocephalus.
ABSTRACT
Proteus syndrome is a rare hamartomatous disorder affecting multiple tissues and manifesting itself in a variety of ways. The understanding of the complete spectrum of clinical features, the natural clinical course of the disease and the proper management of such a rare but highly variable syndrome depend heavily on experiences gathered by previously reported cases. We present an unusually severely affected and rapidly progressive case of proteus syndrome in a neonate who presented with craniofacial hemihypertrophy, subcutaneous masses, capillary hemangioma, varicose veins, epidermal nevi and macrodactyly. The cranial ultrasonogram revealed unilateral hydrocephalus with partial obstruction of the foramen of monro.
ABSTRACT
BACKGROUND: Congenital unilateral hydrocephalus is an uncommon entity occurring almost exclusively in children. Atresia, stenosis, membranous occlusion and even functional obstruction of the foramen of Monro have been described to be the main cause of this type of hydrocephalus. There are two options available in the surgical management of unilateral hydrocephalus: one is the placement of shunt CSF diversion from the dilated ventricle and the other is fenestration of the occluded foramen of Monro or septum pellucidum by endoscopy or by stereotactic method. Migration of the ventriculoperitoneal (VP) shunt in or out of ventricles is not so uncommon, but the relocation of the ventricular tip of a catheter from the ventricle into the quadrigeminal cisterns and superior vermis in association with ventriculostomy is extremely rare. Spontaneous ventriculostomy is a rare event and results from spontaneous rupture of a ventricle into the subarachnoid space. CASE DESCRIPTION: A 5½-month-old baby with a right-sided congenital unilateral hydrocephalus underwent a VP shunt andhad experienced an uneventful outcome. Four years later on an MR imaging examination, the tip of the ventricular catheter passing through the medial wall of the ventricle and the quadrigeminal cistern was found to be situated in the superior vermis. During the follow-up period, there were no neurological difficulties. The cognitive and motor skill development corresponded well with the child's age. It transpired that the hydrocephalic ventricle reduced its size dramatically to normal. CONCLUSION: We have described the extremely rare site of the relocation of the ventricular catheter after the treatment of the congenital unilateral hydrocephalus by VP shunting. Spontaneous ventriculostomy as a rare phenomenon may be the explanation of the relocation of the ventricular catheter.
ABSTRACT
OBJECTIVE: Unilateral hydrocephalus (UH) is characterized by enlargement of just one lateral ventricle. In this paper, the authors will demonstrate their experiences in the neuroendoscopic management of this uncommon type of hydrocephalus. METHOD: The authors retrospectively reviewed a serie of almost 800 neuroendoscopic procedures performed from September 1995 to July 2010 and selected seven adult patients with UH. Clinical and radiological charts were reviewed and analyzed. RESULTS: Six patients had intraventricular neurocysticercosis and one patient had congenital stenosis of the foramen of Monro. Headaches were the most common symptom. A septostomy restored cerebrospinal fluid circulation. During follow-up period (65.5 months, range 3-109) no patient has presented clinical recurrence as well as no severe complications have been observed. CONCLUSION: UH is a rare condition. A successful treatment can be accomplished through a neuroendoscopic approach avoiding the use of ventricular shunts.
OBJETIVO: Hidrocefalia unilateral (HU) é caracterizada pelo alargamento de apenas um dos ventrículos laterais. Neste estudo, os autores demonstraram sua experiência no manejo deste tipo incomum de hidrocefalia. MÉTODO: Foram revisados, de uma série de quase 800 cirurgias neuroendoscópicas realizadas entre Setembro de 1995 e Julho de 2010, sete pacientes adultos com diagnóstico de HU. Dados clínicos e radiológicos foram analisados. RESULTADOS: Seis pacientes tinham neurocisticercose intraventricular e um apresentava uma estenose congênita do forame de Monro. Cefaléia foi o sintoma clínico mais comum. Uma septostomia restabeleceu o fluxo liquórico. Durante o seguimento (65,5 meses, de 3-109), nenhum paciente apresentou recorrência clínica assim como nenhuma complicação grave foi observada. CONCLUSÃO: HU é uma condição rara. O tratamento satisfatório pode ser alcançado por meio de uma abordagem neuroendoscópica evitando, desta maneira, o uso de sistemas de derivação ventricular.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Cerebral Ventricles , Cerebral Ventriculitis/complications , Hydrocephalus/surgery , Neurocysticercosis/complications , Neuroendoscopy/methods , Constriction, Pathologic , Follow-Up Studies , Hydrocephalus/etiology , Magnetic Resonance Imaging , Retrospective StudiesABSTRACT
La hidrocefalia congénita unilateral es una malformación extremadamente infrecuente (1 por 1.411 embarazos) y rara vez detectada en el período prenatal. Se considera una entidad distinta a la hidrocefalia bilateral, en cuanto a morbilidad, mortalidad y pronóstico perinatal. En la hidrocefalia unilateral se reporta una sobrevida elevada y un resultado neurológico favorable cuando el grado de dilatación es leve, estable en el tiempo y no asociada a otras malformaciones. El diagnóstico prenatal se realiza mediante ecografía y resonancia magnética y es confirmado al nacimiento con ecografía, tomografía axial computada o resonancia magnética. No existen actualmente pautas estandarizadas para su manejo y seguimiento a largo plazo. Se presenta a continuación un caso clínico de diagnóstico antenatal de hidrocefalia congénita unilateral y la revisión de la literatura.
Congenital unilateral hydrocephalus is an extremely rare malformation (1 per 1,411 pregnancies), rarely detected in the prenatal period. It is considered a different entity than bilateral hydrocephalus, in terms of morbidity, mortality and perinatal outcome. Unilateral hydrocephalus has a high survival rate and favorable neurological outcome when dilatation is mild, stable over time and when not associated with other malformations. Prenatal diagnosis is performed by ultrasound and magnetic resonance imaging and confirmed at birth with ultrasound, CT sean or MRI. Not currently standard guidelines are available for management and long-term monitoring. We present a case report of antenatal diagnosis of congenital unilateral hydrocephalus and literature review.