神经内窥镜三脑室造瘘术治疗小儿脑积水

目的：介绍神经内窥镜三脑室造瘘术治疗小儿脑积水方法。方法：男8例，女2例，年龄5个月至11岁，平均33个月。CT、MRI及同位素^99Tcm-DTPA脑池显像显示为非脑脊液吸收障碍性脑积水。神经内窥镜从侧脑室经孟氏孔进入三脑室，在乳头小体前言三脑室底最薄处造一瘘口与脚间 池相通。结果：术后随访3－6个月，所有患儿症状均有改善。结论：神经内窥镜行三脑室造瘘术非脑脊液吸收障碍性脑积水是一种有效的微创手术。     

Congenital hemiparesis: the spectrum of lesions. A clinical and computerized tomographic study of 37 cases

Cerebral computerized tomography (CT) was obtained in 37 children with congenital hemiparesis to document the type of cerebral anomalies found in this group, correlate them with various features of the neurological handicap and clinical history and determine the prognostic and practical value of this examination in a newly diagnosed case. The CT-findings were: 1. normal CT: 4 children (11%); 2. unilateral ventricular enlargement: 9 children (24%); 3. others (cortical and subcortical lesions): 24 children (63%). In this latter group, special patterns were found: a) focal ischemic lesions: 7 cases; b) cystic porencephaly: 2 cases; c) ventricular deformities with hydrocephalus: 7 cases; d) varia: 8 cases. The children with normal CT or unilateral ventricular enlargement had mild or moderate hemiparesis without epilepsy or mental retardation. The third group comprised the more severe cases but the prognosis was very variable. The different types of CT-lesions, their possible origin and neuropathological correlates are described and compared with two previous studies. The variety of lesions found in this series and the relative prognostic value of the CT-Scan justify this examination in a newly diagnosed case.     

Assessment and therapeutic possibilities in posthemorrhagic hydrocephalus of the newborn infant

Internal hydrocephalus follows intraventricular hemorrhage in about 10%. Progression is directly related to the degree of hemorrhage. Several studies confirmed that cerebral damage may occur without an increase of intracranial pressure or head circumference. Assessment and therapeutic consequences depend entirely on sonographic and clinical criteria. In our series, 40 of 135 neonates with intraventricular hemorrhage developed internal hydrocephalus. Therapy was necessary in 35 children, 11 died. Serial lumbar punctures were the most frequent therapeutic approach in 70% of the children. 40% required a shunt, usually a ventriculo-peritoneal system. In this group the proportion of Grade III and IV hemorrhage was high. In 25% external drainage for one week was necessary. 10% were treated with acetazolamide and furosemide. By this therapy shunt placement could be avoided in 9 children (69.2%), in the group with Grade III hemorrhage. In addition it was possible to postpone shunting to the third month on the average. 25 children were followed-up. 40% were normal or had a mild developmental delay. 60% were seriously handicapped. Poor neurodevelopmental outcome was related directly to hemorrhage Grade III and IV, therefore to brain damage in the early phase. To prevent additional lesions it should be emphasized that in infantile hydrocephalus best long-term results have been obtained if the process was controlled early rather than later.     

Learning, memory and executive functions in children with hydrocephalus

AIMS: To explore learning, memory and executive abilities in children with hydrocephalus without learning disabilities, and to find out whether children with an isolated hydrocephalus differed from those with hydrocephalus in combination with myelomeningocele (MMC). METHODS: Thirty-six children with an intelligence quotient (IQ) of >or=70 from a population of all the 107 children with hydrocephalus born in western Sweden in 1989-1993 were examined and compared with age- and gender-matched controls. The neuropsychological assessment of the school-aged child (NIMES) test battery was used. RESULTS: The children with hydrocephalus differed significantly from controls in all functions apart from registration skills and recognition. Learning, memory and executive functions were all impaired. Twenty children with infantile hydrocephalus did not differ from those with hydrocephalus associated with MMC. Also, children with an IQ of >84 performed significantly worse than controls. CONCLUSIONS: Despite an IQ of >or=70, children with hydrocephalus had significantly impaired learning, memory and executive functions. When major brain lesions resulting in learning disability had been excluded, the hydrocephalus, rather than the underlying aetiology, was most important for the development of cognitive functions.     

Endoscopic intraventricular surgery for treatment of hydrocephalus and loculated CSF space in children less than one year of age

Controversy exists regarding whether children under 1 year of age have a higher risk of treatment failure after neuroendoscopic procedures for the treatment of hydrocephalus than older children. We retrospectively reviewed the surgical management and clinical outcome of 15 infants who underwent neuroendoscopic surgery for the treatment of CSF space loculation and hydrocephalus. We performed 8 third ventriculostomies, 3 endoscopic arachnoid cyst fenestrations, 2 aqueductoplasties, 1 septostomy and in 1 patient, three endoscopic fenestrations for isolated ventricular compartments. Two of the third ventriculostomies, 1 of the aqueductoplasties and the 1 septostomy failed, and these patients underwent placement of a ventriculoperitoneal shunt. In all other patients, symptoms and signs related to hydrocephalus or CSF space loculation were relieved effectively after the endoscopic procedure. We conclude that neuroendoscopy presents an effective alternative for the treatment of hydrocephalus and CSF loculation in infants less than 1 year of age.     

Rapid deterioration of primary fourth ventricular outlet obstruction resulting in syndrome of inappropriate antidiuretic hormone secretion

Fourth ventricular outlet obstruction (FVOO) is a rare cause of obstructive hydrocephalus. Although FVOO accompanied by malformative syndrome and secondary causes of obstruction are common, there are few reports of primary FVOO (PFVOO). The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a rare presenting feature of hydrocephalus. A 1‐year, 8‐month‐old boy with a normal head circumference developed SIADH accompanied by rapid deterioration of symptoms of intracranial hypertension. PFVOO was diagnosed because magnetic resonance imaging revealed an enlarged ventricular system with a barely visible membranous obstacle at the foramen of Magendie. All symptoms were resolved by endoscopic third ventriculostomy. PFVOO should be considered as a rare form of congenital obstructive hydrocephalus, especially in patients with tetraventricular hydrocephalus. To the best of our knowledge, this is the first case of an infant with SIADH, resulting from acute deterioration of non‐tumoral raised pressure hydrocephalus.     

Management of intrinsic gliomas of the tectal plate in children. A ten-year review

The natural history, management, and long-term outcome for patients with benign, intrinsic tectal plate gliomas remain controversial in spite of their propensity to cause late-onset hydrocephalus. A 10-year retrospective review has identified 11 consecutive children with tectal plate lesions. Headache, vomiting, a decline in school performance, tremor, and complex partial seizures were common presenting symptoms. All patients presented with signs and symptoms of hydrocephalus. Magnetic resonance (MR) imaging delineated an intra-axial mass lesion of the midbrain primarily localized to the tectal plate which uniformly was hyperintense on T2-weighted imaging and had a more variable appearance on T1-weighted imaging and rare enhancement with gadolinium. No patient underwent surgical resection, chemotherapy, or radiotherapy. Three of 11 patients (27%) showed evidence of progression in size or a new focus of enhancement on MR imaging, which was clinically asymptomatic. In this series, no patient with a tectal plate lesion less than 1.5 cm in maximal diameter and without gadolinium enhancement showed any evidence of clinical or radiological progression. Although intrinsic tectal lesions in children are clinically indolent and the initial management consists of CSF diversion, these lesions may eventually progress and still warrant long-term follow-up with serial MR imaging.     

Endoscopic resection of an intraventricular dysembryoplastic neuroepithelial tumor of the septum pellucidum

Tumors located in the region of the foramen of Monro often present with signs and symptoms of obstructive hydrocephalus. Various types of lesions occur in this location. We describe a case of a dysembryoplastic neuroepithelial tumor of the septum pellucidum presenting with obstructive hydrocephalus and its successful endoscopic excision. The surgical considerations and pathologic findings are discussed.     

Synchronous occurrence of a hemorrhagic hypothalamic hamartoma and a suprasellar teratoma

Hypothalamic hamartomas have been reported to coexist with lesions like Rathke's cleft cyst and arachnoid cysts in the suprasellar or temporo-sylvian regions. This is the first report in indexed literature describing its association with a suprasellar teratoma. A 7-year-old girl presented with long-standing precocious puberty and generalized tonic-clonic seizures and recent-onset raised intracranial pressure. MRI done prior to the onset of symptomatic raised intracranial pressure revealed 2 distinct lesions in the suprasellar region. One was a midline, pedunculated lesion arising from the hypothalamus, with evidence of an old bleed within it. A separate lesion, with a wide base near the tuberculum sellae and a posteriorly directed conical tip, was noted in an adjacent sagittal cut. CT scan done at the time of admission demonstrated a re-bleed in the suprasellar region with blood in the lateral and third ventricles and gross hydrocephalus. The child was taken up for a ventriculoperitoneal shunt followed by complete excision of the lesions. Histopathologic examination confirmed the pedunculated lesion to be a hypothalamic hamartoma with evidence of hemorrhage, and the other to be a mature teratoma. Postoperative MRI confirmed complete excision of both the lesions. The child reported regression of precocious puberty and remained seizure-free until the last follow-up 6 months after surgery. A hypothesis based on a dysontogenetic mechanism is discussed to explain the unusual occurrence of the dual, seemingly unrelated pathologies. Hemorrhage into the hamartoma was an added oddity in this case.     

The bifrontal olfactory nerve-sparing approach to lesions of the suprasellar region in children.

Suprasellar masses in children include lesions such as craniopharyngiomas and germ cell neoplasms. The difficult location of these lesions and their proximity to important neural and vascular structures pose challenges to resection. We operated on 14 patients using a bifrontal craniotomy with removal of both orbital rims to provide access to suprasellar masses, even those with significant extension into the third ventricle. A complete resection was possible in 8/14 patients and 8/10 craniopharyngiomas. In 13 patients, the optic nerves were identified and preserved, and vision was stable or improved postoperatively. Postoperatively, 1 patient with hydrocephalus developed a CSF leak which was successfully treated with a ventriculoperitoneal shunt. No patient had a cosmetic defect related to orbital rim removal. The bifrontal approach used here enhanced the exposure of the suprasellar region and minimized manipulation of the optic apparatus and the carotid arteries. It may be used alone, or in conjunction with other approaches, to resect lesions in the suprasellar region.     

神经内镜在小儿颅内囊液性病变治疗中的应用

目的 探讨神经内镜技术在小儿颅内囊液性疾病中的治疗应用,以及结合开颅术及分流术协同完成小儿颅内复杂畸形手术治疗的效果.方法 2004年2月至2009年2月,105例患儿接受内镜手术,年龄3个月至12岁.包括阻塞性脑积水53例,其中51例成功完成内镜下三脑室底造口术.脑内囊肿52例中有28例颞叶囊肿行镜下囊壁部分剥离术和脑池开放术,5例透明隔囊肿和5例侧脑室囊肿分别行囊肿-脑室造口术、3例鞍上及三脑室囊肿行囊肿剥离+三脑室底造口,幕下囊肿6例:其中内镜剥离1例,5例巨大囊肿合并脑积水者采取内镜结合分流或开颅显微镜下剥离术.幕上中线部位囊肿5例,囊肿部分剥离+开窗4例,1例多房打通结合囊肿腹腔分流术.结果 51例脑积水中,47例术后临床症状改善.52例颅内囊肿,术后随访2个月至3年,总体缩小率占98%,颞叶囊肿缩小率100%,11例术后有硬膜下积液,7例随访好转,4例术后4个月至1年出现慢性硬膜下血肿,行外引流术后治愈,1例合并中枢感染者放弃治疗.结论 内镜手术在小儿颅内囊液性疾病的治疗中效果良好,可优先选择.     

Ventriculoscope tract recurrence after endoscopic biopsy of pineal germinoma

Recurrence along an endoscope tract has been described after endoscopic biopsy and resection of malignant tumors arising in multiple organ systems. We describe a case of tract recurrence following the ventriculoscopic biopsy of a central nervous system tumor. A retrospective review of the patient's clinical course, radiology and pathology was undertaken to formulate a case report. A 14-year-old boy was treated with endoscopic third ventriculostomy and endoscopic biopsy of a pineal germinoma associated with hydrocephalus. Persistent hydrocephalus required repeat third ventriculostomy. The patient subsequently received focal external beam radiotherapy. Seventeen months after the first third ventriculostomy, a routine MRI revealed a 2-cm enhancing mass in the ventriculoscope tract. This was removed via a right frontal craniotomy. Again the pathology was germinoma. Serum and CSF markers were negative at initial presentation and at the time of recurrence. The potential for tract recurrence and CSF dissemination should be considered following the endoscopic biopsy of pineal germ cell tumors as this could affect the extent of radiotherapy and/or chemotherapy.     

Controversy about Management of Hydrocephalus – Shunt <Emphasis Type="Italic">vs.</Emphasis> Endoscopic Third Ventriculostomy

The best management of hydrocephalus is still controversial in the twenty-first century. Shunt treatment for hydrocephalus is the most common procedure performed in neurosurgical practice and is associated with the highest complications rate. But during the last 2 decades, the treatment of hydrocephalus has improved with better shunt devices available today, increased facilities for investigations and newer approaches like endoscopic third ventriculostomy. The recent advances in development of better endoscopes have provided the patient and treating doctor with an option for an alternative surgery for treatment of hydrocephalus.     

Information value of magnetic resonance imaging in shunted hydrocephalus.

OBJECTIVE--To study the role of magnetic resonance imaging (MRI) in evaluating children with shunted hydrocephalus. METHODS--Sixty one asymptomatic children with shunted hydrocephalus or cystic cerebrospinal fluid collections were studied by cranial MRI. The information obtained from the images was classified into three categories: provided (1) a new diagnosis, (2) additional information, or (3) no essential new information. The findings were compared with those of the last follow up computed tomograms. RESULTS--MRI provided a new diagnosis in seven cases (11.5%), and additional information was obtained in 34 (55.7%) cases. In 20 cases (32.8%) no essential new information was obtained. MRI visualised white matter lesions and corpus callosum pathology more often than computed tomograms. CONCLUSIONS--MRI provided new important information in cases of children with shunted hydrocephalus to such an extent that it can be recommended as the primary imaging method for every child with this disorder.     

Failure of open third ventriculostomy for shunt infections in infants.

Open third ventriculostomy (OTV) was performed on 4 infants with noncommunicating hydrocephalus and intractable shunt infections. All patients were resistant or relapsed after treatment with intravenous and intraventricular antibiotics along with change of the shunt apparatus. We performed phase-contrast cine magnetic resonance imaging (MRI) for preoperative and postoperative evaluation of cerebrospinal fluid (CSF) flow at the aqueduct of Sylvius. All patients required a second OTV approximately 3 weeks after the first OTV due to closure of the patency. Our experience led us to view OTV as an unsuccessful procedure in infantile noncommunicating hydrocephalus due to an insufficiently developed subarachnoid space. The patients' data, operative findings and probable causes of failure are presented here.     

Changes of third ventricle diameter (TVD) mirror changes of the entire ventricular system after initial therapy and during follow-up in pediatric hydrocephalus

PurposeRegular measurement of ventricular size is important in children with hydrocephalus. After closure of the fontanelle this is currently addressed by repetitive cranial MRI or CT imaging, coming along with risks of anaesthesia or radiation. As the third ventricle is accessible via the temporal bone window using ultrasound, determination of its diameter might be an easy and radiation-free alternative to assess the ventricular system. An essential precondition is that changes of the third ventricle diameter (TVD) mirror changes of the whole ventricular system. This study compares changes of TVD with changes of ventricular indices before and after initial treatment of hydrocephalus and during the following evolution.MethodsMRT/CT images from 117 children with hydrocephalus were evaluated at time of diagnosis, after initial therapy and during follow-up with functional shunts. Measurements included axial TVD and three standard linear measures of the lateral ventricles (Evans Index - EI, fronto-occipital horn ratio - FOHR Index, and Cella Media Index - CMI). Furthermore, a correlation within subjects was calculated in 8 patients over the entire available follow-up.ResultsRelative changes of TVD were significantly correlated to relative changes of all ventricular indices (r = 0.48, r = 0.68 and r = 0.701 for EI, FOHR and CMI, respectively, p < 0.01). The correlation within subjects was outstanding for EI (r = 0.988), FOHR (r = 0.99) and CMI (r = 0.99).ConclusionTVD showed a significant correlation with all three linear indices at the time of diagnosis and during follow-up changes independently of age, aetiology and ventricular width. TVD and its changes are therefore a reliable surrogate of changes in ventricular size in pediatric hydrocephalus undergoing treatment.     

Endoscopic third ventriculostomy in hydrocephalus associated with achondroplasia

Hydrocephalus in patients with achondroplasia is thought to be due to increased dural sinus venous pressure resulting from narrowing of the jugular foramen. In this setting, where hydrocephalus is presumed to be "vascular" in origin and therefore communicating, endoscopic third ventriculostomy (ETV) would seem contraindicated. The authors describe 3 patients in whom ETV was successfully performed, resulting in MR imaging-documented decreases in ventricle size. The patients were 11 months, 33 months, and 13 years at the time of surgery. All patients had serial preoperative MR images demonstrating progressive hydrocephalus in a "triventricular" pattern with a small fourth ventricle but an open aqueduct. All patients had undergone suboccipital decompression for foramen magnum stenosis prior to the treatment of hydrocephalus. Preoperative retrograde venography revealed variable pressure gradients across the jugular foramen. It is postulated that the increase in intracranial venous pressure resulting from jugular foramen stenosis may lead to disproportionate venous engorgement of the cerebellum and some degree of obstructive hydrocephalus amenable to ETV. The authors discuss the role of suboccipital decompression in the progression of hydrocephalus in patients with achondroplasia.     

Aqueductal stenosis presenting as isolated tremor: case report and review of the literature

Essential tremor is rare in children, particularly in the absence of a significant family history. We report the case of a child with compensated hydrocephalus secondary to aqueductal stenosis whose sole presenting symptom was tremor. An otherwise healthy 6-year-old male developed a fine hand tremor, which over the course of 4 years both increased in intensity and spread to involve the lower limbs and head. After an MRI had confirmed hydrocephalus due to aqueductal stenosis, the patient underwent an endoscopic third ventriculostomy. His tremor improved markedly, but did not completely resolve. Occult hydrocephalus should be considered in the differential diagnosis of new-onset tremor. Progression of the tremor should halt with treatment of the hydrocephalus, and clinical improvement may be seen.     

Endoscopic third ventriculostomy for hydrocephalus secondary to central nervous system infection or intraventricular hemorrhage in children

OBJECTIVE: We review our experience of endoscopic third ventriculocisternostomy (ETV) in children with hydrocephalus from central nervous system (CNS) infection or intraventricular hemorrhage to better elucidate success rates and predictors of success in these children. METHODS: We performed a retrospective review of 12 children less than 21 years of age with ETV treated from 1999 to 2002 with a minimum follow-up of 12 months. Children selected for surgery had been diagnosed with hydrocephalus from perinatal germinal matrix hemorrhage or CNS infection and had neuroimaging consistent with obstruction of the aqueduct, fourth ventricular outlets or both at the time of ETV. Charts and imaging studies were reviewed to determine clinical outcomes and predictors of successful ETV. An ETV was deemed successful if the child did not require placement or replacement of a ventriculoperitoneal shunt. RESULTS: Thirteen ETV were performed in 12 patients (7 boys and 5 girls; mean age 9.2 years, range 3.8-21 years), with an overall success rate of 60% for those with CNS infections and 71% for those with perinatal intraventricular hemorrhage at initial presentation. There were no significant complications from the procedure. CONCLUSIONS: ETV is a safe procedure, which in carefully selected children with etiologies of hydrocephalus thought to be 'communicating' in nature carries a satisfyingly high success rate. Further application and study of this modality in larger groups of patients with these causes of hydrocephalus is warranted.