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.     

Endoscopic third ventriculostomy through the interfornicial space

Endoscopic third ventriculostomy (ETV) is the most common cranial endoscopic surgery being done worldwide for a variety of conditions resulting in hydrocephalus. We report ETV in a case of congenital hydrocephalus with deficient third ventricular roof and wide interfornicial space. A 6-month-old male child presented with gradual progressive head enlargement and signs of raised intracranial pressure. Computed tomography of the head showed aqueductal stenosis with enlargement of third and lateral ventricles. The patient was operated under general anesthesia with endotracheal intubation. A right coronal burr hole and free-hand entry were made into the right lateral ventricle. The anterior part of the septum and the third ventricular roof were deficient with a narrow Monro's foramen and the third ventricular floor was visible through the space between both fornices. ETV was done in standard fashion. Postoperatively the patient showed uneventful recovery clinicoradiologically. We review the literature and discuss the findings and technique of ETV in this rare entity.     

The impact of endoscopic third ventriculostomy on the management of newly diagnosed hydrocephalus in infants

OBJECTIVES: To evaluate the role of endoscopic third ventriculostomy (ETV) as a primary treatment for hydrocephalus in children less than 1 year old and to determine its impact as a whole on the reduction of shunts necessary in a new population of hydrocephalic infants. METHODS: Data were collected prospectively on 47 infants with newly diagnosed hydrocephalus of all aetiologies who were referred between 1st April 1998 and 30th September 2000. Twenty-one patients (median age 6 weeks, range 34 weeks of gestation to 10 months) underwent ETV, while the remaining 26 patients had insertion of a ventriculoperitoneal shunt. Anatomical criteria and demonstration of third ventricle outflow obstruction on preoperative magnetic resonance imaging were used to select patients for ETV. RESULTS: There was no mortality or major morbidity following ETV. The median follow-up period was 18 (range 8-36) months. During the follow-up period, the ETV remained patent in 7 (33%) of the 21 patients. Of the 14 patients with failed ETV, 11 had insertion of a ventriculoperitoneal shunt, while 3 have undergone successful redo ETV. Therefore, in total 10 patients (48%) of the ETV group remain shunt independent. The best results were obtained in patients with congenital aqueduct stenosis with 71% (5 of 7 patients) success rate, while patients with posthaemorrhagic hydrocephalus did particularly badly with only 1 of 10 patients having a successful ETV. Overall, 10 of 47 (21%) infants with newly diagnosed hydrocephalus have avoided a shunt. CONCLUSIONS: Our results suggest that the selective use of ETV as the primary treatment in infants with hydrocephalus is safe and can lead to a reduction in the shunted population of all newly diagnosed hydrocephalic infants by up to 21%. Success of ETV is aetiology, not age dependent.     

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.     

Establishing successful cerebrospinal fluid flow for radioimmunotherapy

Successful delivery of intraventricular radioimmunotherapy is contingent on adequate CSF flow. The authors present a patient with medulloblastoma in whom obstructed CSF flow was causing hydrocephalus, which was initially corrected by implantation of a programmable shunting device. While managing the hydrocephalus, an endoscopic third ventriculostomy (ETV) needed to be performed in a collapsed ventricular system to ensure adequate radioimmunotherapy distribution. This 18-month-old patient with medulloblastoma involving leptomeningeal dissemination presented for intraventricular radioimmunotherapy. A CSF (111)In-DTPA scintigraphy study obtained through the existing programmable ventriculoperitoneal shunt demonstrated activity in the lateral and third ventricles, but no activity over the cerebral convexities or spinal canal, consistent with obstruction at the level of the cerebral aqueduct. By maximization of ventricular size in a controlled setting, the patient was able to undergo a trial of ETV through very small ventricles. A postoperative CINE MR imaging study confirmed patent ETV. The pressure settings on the shunt were kept at the highest opening pressure (200 mm H(2)O) to maximize flow through the stoma and improve the distribution of CSF throughout the subarachnoid space. The CSF flow scintigraphy study was again performed, this time with tracer activity demonstrated down the thecal sac at 3 hours, and symmetrically over the cerebral convexities at 24 hours. The patient began weekly intraventricular administration of (131)I-3F8 therapy. Successful rerouting of CSF flow for the purpose of therapeutic radioisotope administration is possible. Endoscopic third ventriculostomy can be considered in patients with programmable shunting devices; normal or slit ventricles do not preclude successful ETV.     

Spectrum of the syndrome of the isolated fourth ventricle in posthemorrhagic hydrocephalus of the premature infant

Ten patients who were premature born and had intracranial/intraventricular hemorrhage and developed hydrocephalus in early life were treated with ventriculoperitoneal shunts because of progression of the hydrocephalus. The hydrocephalus remained well controlled but in a follow-up period of 2 months to 7 years after shunt placement the patients developed an isolated fourth ventricle that required treatment. The presenting symptoms varied: increasing head size, fontanelle fullness, irritability difficulty with swallowing, vomiting, hypoactivity, headaches and lethargy. One patient presented with full cardiorespiratory arrest and expired. The remainder on neuroimaging studies revealed an extremely large fourth ventricle creating a mass effect and very small (slit-like) lateral ventricles, indicating a functioning lateral ventricle-peritoneal shunt. These patients were treated with the placement of a fourth ventricle shunt catheter and connection to the existing shunt, with resolution of symptoms. Premature infants with hydrocephalus need to be followed and assessed for findings of the syndrome of the isolated fourth ventricle in an attempt to prevent sudden neurological deterioration.     

Sonographic characteristics of the Arnold-Chiari syndrome and hydrocephalus in children with meningomyelocele

22 children with myelomeningocele had serial examination by gray scale ultrasonography through the open fontanelle as an acoustic window. 18 children (82%) developed a progressive hydrocephalus and were eventually shunted. In 18 children the hydrocephalus was caused by an Arnold-Chiari-II-malformation with the sonografic signs of a caudal displacement of the dysplastic cerebellum, brainstem, 4th ventricle and cisterna magna. The forth ventricle was elongated and flattened in all babies with Arnold-Chiari-syndrome. The cisterna magna was not visible in any child. The Arnold-Chiari-II-malformation caused an occlusive hydrocephalus which was characterized by the dilatation of the occipital horns (91%) and frontal horns (86%) of the side ventricles, whereas the temporal horns were normal in size (86%). The dysplastic 3. ventricle was displaced caudally and ventrally and showed a prominent suprapineal recess in 41%. Associated malformations of the brain were a large massa intermedia (55%), a prominent plexus choriodeus (50%) and agenesia (36%) or fenestration (9%) of the septum pellucidum. Gray scale ultrasonography is the best method not only for early diagnosis of Arnold-Chiari-malformation and resulting hydrocephalus in infants but also for further controls after shunt implantation, especially for early detection of shunt complications and shunt insufficiency.     

Asymmetric growth of the lateral cerebral ventricle in infants with posthemorrhagic ventricular dilation

Lateral cerebral ventricular volume in 36 preterm infants with or without an intraventricular hemorrhage, and with or without posthemorrhagic hydrocephalus, was measured longitudinally and compared with the ventricular index measurements of the same ventricles. A poor correlation was found (r2 = 0.67). To determine a reason for this poor relationship, we analyzed the volumes of the regions of the ventricles by a segmental volume analysis. The occipital region of the lateral cerebral ventricle enlarged at a much faster rate (1.904 +/- 0.477 ml/day) than either the anterior region (0.546 +/- 0.253 ml/day; p less than 0.01) or the middle region (-0.209 +/- 0.334 ml/day; p less than 0.01) in infants with posthemorrhagic hydrocephalus. The rate of growth of the middle region of the lateral cerebral ventricles was the same for all infants. Linear indexes, such as the ventricular index and the lateral ventricular ratio, do not allow for accurate serial estimates of ventricular size in posthemorrhagic hydrocephalus because of asymmetric growth of the lateral cerebral ventricle. We conclude that sequential volume measurements are more useful than ventricular index measurements to follow ventricular size sequentially in infants with posthemorrhagic hydrocephalus.     

Ommaya储液囊在儿童急性梗阻性脑积水中的应用

目的 探讨Ommaya储液囊在儿童急性梗阻性脑积水中的应用.方法 回顾性分析我科2006至2009年22例晚发维生素K依赖因子缺乏性脑室出血和结脑致急性梗阻性脑积水患儿,行Ommaya储液囊植入手术,观察脑室大小、脑脊液细胞数、蛋白含量变化.结果 结核性脑膜炎7例经储液囊植入行泵内多次注射抗生素,治疗2～4个月、随访脑脊液恢复正常,6例脑积水消失;晚发维生素K依赖因子缺乏性脑室出血伴脑积水15例植入脑室内行泵内穿刺冲洗引流,1～2周头颅CT复查脑室积血消失,脑积水减轻,2例行侧脑室腹腔分流术,无一例发生颅内感染.结论 Ommaya储液囊植入术治疗儿童急性梗阻性脑积水效果良好,手术简便、创伤小、并发症少,值得临床广泛应用.

Abstract：

Objective To review the Ommaya pump application in acute obstructive hydrocephalus in children. Methods We carried out a retrospective analysis of 22 children with obstructive hydrocephalus admitted into our department between 2006 2009. The causes of hydrocephalus included intraventricular hemorrhage secondary to vitamin K deficiency and tuberculous meningitis. Ommaya pump were implanted and the ventricle size and cerebrospinal fluid cell count, protein content were studied. Results Seven children had intracranial infection and treated with antibiotic injection into pump for 2-4 months. Follow-up revealed no recurrence. Fifteen children with ventricle hemorrhage and hydrocephalus underwent drainage from the pump. CT scan after 1-2 weeks demonstrated disappearance of ventricle hemorrhage. There was, no intracranial infection. Conclusions Ommaya pump for the treatment of acute obstructive hydrocephalus produces good result. The surgery is simple. It incurs less trauma and has less complications.     

Ommaya储液囊在儿童急性梗阻性脑积水中的应用

目的 探讨Ommaya储液囊在儿童急性梗阻性脑积水中的应用.方法 回顾性分析我科2006至2009年22例晚发维生素K依赖因子缺乏性脑室出血和结脑致急性梗阻性脑积水患儿,行Ommaya储液囊植入手术,观察脑室大小、脑脊液细胞数、蛋白含量变化.结果 结核性脑膜炎7例经储液囊植入行泵内多次注射抗生素,治疗2～4个月、随访脑脊液恢复正常,6例脑积水消失;晚发维生素K依赖因子缺乏性脑室出血伴脑积水15例植入脑室内行泵内穿刺冲洗引流,1～2周头颅CT复查脑室积血消失,脑积水减轻,2例行侧脑室腹腔分流术,无一例发生颅内感染.结论 Ommaya储液囊植入术治疗儿童急性梗阻性脑积水效果良好,手术简便、创伤小、并发症少,值得临床广泛应用.     

Failure to treat obstructive hydrocephalus with endoscopic third ventriculostomy in a patient with neurodegenerative Langerhans cell histiocytosis

The second most frequent central nervous system involvement pattern in Langerhans cell histiocytosis (LCH) is a rare condition documented in a number of reports called "neurodegenerative LCH" (ND-LCH). Magnetic resonance images confirming the presence of the disease usually demonstrate striking symmetric bilateral hyperintensities predominantly in the cerebellum, basal ganglia, pons, and/or cerebral white matter. The authors here describe for the first time in the literature a patient with ND-LCH and concomitant hydrocephalus initially treated using endoscopic third ventriculostomy (ETV). This 9-year-old boy, who had undergone chemotherapy for skin and lung LCH without central nervous system involvement at the age of 10 months, presented with acute ataxia, headaches, and paraparesis and a 1-year history of gradually increasing clumsiness. Magnetic resonance images showed obstructive hydrocephalus at the level of the aqueduct of Sylvius and signs of ND-LCH. After registering high intracranial pressure (ICP) spikes with an intraparenchymal pressure monitor, an ETV was performed. A second ETV was required months later because of ostomy occlusion, and finally a ventriculoperitoneal shunt was placed because of ostomy reocclusion. Endoscopic third ventriculostomy was initially considered the treatment of choice to divert cerebrospinal fluid without leaving a ventriculoperitoneal shunt and to obtain biopsy specimens from the periinfundibular recess area. The third ventriculostomy occluded twice, and an endoscopic aqueduct fenestration was unsuccessful. The authors hypothesized that an inflammatory process related to late ND disease was responsible for the occlusions. Biopsy specimens from the infundibular recess and fornix column did not show histopathogical abnormalities. Increased ICP symptoms resolved with cerebrospinal fluid diversion. This case is the first instance of ND-LCH with hydrocephalus reported in the literature to date. Shunt placement rather than ETV seems to be the favorable choice in relieving elevated ICP.     

Long-term outcome for endoscopic third ventriculostomy alone or in combination with choroid plexus cauterization for congenital aqueductal stenosis in African infants

Object The authors have previously reported on the overall improved efficacy of endoscopic third ventriculostomy (ETV) combined with choroid plexus cauterization (CPC) for infants younger than 1 year of age. In the present study they specifically examined the long-term efficacy of ETV with or without CPC in 35 infants with congenital aqueduct stenosis treated at CURE Children's Hospital of Uganda during the years 2001-2006. Methods Infants with congenital aqueductal stenosis were treated during 2 distinct treatment epochs: all underwent ETV alone, and subsequently all underwent ETV-CPC. Prospectively collected data in the clinical database were reviewed for all infants with an age < 1 year who had been treated for hydrocephalus due to congenital aqueductal stenosis. Study exclusion criteria included: 1) a history or findings on imaging or at the time of ventriculoscopy that suggested a possible infectious cause of the hydrocephalus, including scarred choroid plexus; 2) an open aqueduct or an aqueduct obstructed by a membrane or cyst rather than by stenosis; 3) severe malformations of the cerebral hemispheres including hydranencephaly, significant segments of undeveloped brain, or schizencephaly; 4) myelomeningocele, encephalocele, Dandy-Walker complex, or tumor; or 5) previous shunt insertion. The time to treatment failure was analyzed using the Kaplan-Meier method to construct survival curves. Log-rank (Mantel-Cox) and Gehan-Breslow-Wilcoxon tests were used to determine whether differences between the 2 treatment groups were significant. Results Thirty-five patients met the study criteria. Endoscopic third ventriculostomy alone was performed in 12 patients (mean age 4.7 months), and combined ETV-CPC was performed in 23 patients (mean age 3.5 months). For patients without treatment failure, the mean and median follow-ups were, respectively, 51.6 and 48.0 months in the ETV group and 31.2 and 26.4 months in the ETV-CPC group. Treatment was successful in 48.6% of the patients who underwent ETV alone, as accurately predicted by the Endoscopic Third Ventriculostomy Success Score (ETVSS), and in 81.9% of the patients who underwent ETV-CPC (p = 0.0119, log-rank test; p = 0.0041, Gehan-Breslow-Wilcoxon test; HR 6.42 [95% CI 1.51-27.36]). Conclusions Combined ETV-CPC is significantly superior to ETV alone for infants younger than 1 year of age with congenital aqueductal stenosis. The fact that the outcome for ETV alone was accurately predicted by the ETVSS suggests that these results are applicable in developed countries.     

The prognosis of children with hydrocephalus and congenital heart disease

The association of congenital hydrocephalus and heart disease in children is infrequent, but may present considerable dilemmas in management. This report describes the treatment and prognosis of 11 children with both clinical problems. There were 5 males and 6 females. Hydrocephalus occurred following aqueductal stenosis in 5 children and the Dandy-Walker malformation in 3. Three children were diagnosed with idiopathic hydrocephalus. Ten children underwent cerebrospinal fluid diversion procedures for control of hydrocephalus. Five children received pharmacological therapy for cardiac disease; 4 children required surgical correction. Two children died from medical conditions; 2 families declined treatment. Follow-up from 2 to 7 years in the remaining 7 children demonstrated moderate or severe neurodevelopmental disability in 5. One child at 2 years of age showed borderline developmental disability while 1 child is developing normally at 10 years of age. Overall the occurrence of symptomatic hydrocephalus and heart disease in the perinatal period resulted in mortality or neurodevelopmental disability in 9/11 children.     

Prenatal diagnosis of unilateral hydrocephalus

We report a case of unilateral hydrocephalus diagnosed at 20 weeks' gestation, at which time marked facial and cranial asymmetry was present already. Brain mantle reconstitution was incomplete following vetriculo-peritoneal shunting, and the child has significant neurodevelopmental disability.     

Suprasellar arachnoid cyst with delayed puberty

A 15-year-old female presented with primary amenorrhea and delayed onset of secondary sexual characteristics. Earlier she was operated for endoscopic third ventriculostomy (ETV) for a tense suprasellar arachnoid cyst with obstructive hydrocephalus. MRI revealed recurrence of hydrocephalus. Hormonal levels were suggestive of hypogonadism and deficiency of growth hormone. She was operated for fenestration of cyst. In this case, suprasellar arachnoid cyst presented with delayed puberty, which is unusual.     

婴幼儿阻塞性脑积水两种手术方式的疗效比较

目的 回顾性分析2005年以来上海交通大学医学院附属新华医院收治的2岁以下阻塞性脑积水患儿的临床资料,比较神经内镜下第三脑室底造瘘术（ETV）与传统的脑室腹腔分流术（VP shunt）治疗婴儿阻塞性脑积水的疗效.方法 2005年以来作者收治36例阻塞性脑积水患儿,均为单纯导水管狭窄,根据手术方式分为ETV治疗组和VP shunt治疗组,比较两组围手术期情况、影像学检查结果以及临床症状,并进行统计学分析.结果 36例中,ETV治疗组20例;VP shunt治疗组16例.均手术顺利,无术中并发症.ETV组有2例术后当天出现癫痫,考虑为间脑发作,经药物治疗后未再复发;1例出现脑室内出血,1例出现硬膜下出血,均予药物治疗后吸收;1例术后第10天出现伤口渗液、脑脊液漏,改行VP shunt治疗后痊愈;ETV组成功率95%,再手术率5%,近期并发症发生率25%,无远期并发症.VP shunt组16例中,1例术后第10天出现头端伤口感染,予抗感染治疗后好转,2个月后出现颅内感染,行拔管术,抗感染治疗好转后重新置管;1例术后5个月后复发脑积水,行ETV后好转;1例转流功能障碍,行再次VP shunt后好转;1例分流管头端脱出,行再次VP shunt后好转,手术总成功率75%,再手术率25%,近期并发症发生率6.25%,远期并发症发生率18.75%.结论 2岁以下婴幼儿阻塞性脑积水ETV与分流治疗对比,ETV近期并发症的发生率高于VP shunt,长期随访未出现新的神经功能障碍,远期并发症明显少于VP shunt,具有推广价值.     

Ventricular dilation following periventricular-intraventricular hemorrhage: outcome at age 1 year

Ventricular dilation following periventricular-intraventricular hemorrhage can be managed without ventriculoperitoneal shunting in most cases. Twenty-six patients who had periventricular-intraventricular hemorrhage with subsequent ventricular dilation were examined at 1 year of age for neurodevelopmental outcome and hydrocephalus. As previously reported, ventricular dilation may be divided into two groups: ventriculomegaly and posthemorrhagic hydrocephalus. Fourteen patients with ventriculomegaly were followed up with serial ultrasound observations only, and 12 patients with posthemorrhagic hydrocephalus had temporary drainage of ventricular fluid. Only three patients with posthemorrhagic hydrocephalus required ventriculoperitoneal shunting in the neonatal period. Neurodevelopmental abnormalities were found in eight infants who had posthemorrhagic hydrocephalus and two who had ventriculomegaly. Six of these infants had intraparenchymal injury demonstrated by ultrasound, five as a result of the original hemorrhage and 1 by infection. A single infant with posthemorrhagic hydrocephalus, discharged from the hospital with stable ventricular size, developed hydrocephalus and neurodevelopmental delay after the neonatal period. This reversed with ventriculoperitoneal shunting at 1 year of age. It is suggested that even in patients developing ventricular dilation following periventricular-intraventricular hemorrhage, it is the primary intraparenchymal injury that is responsible for subsequent morbidity. Thus, provided serial reevaluations are possible, an expectant management of ventricular dilation is justified.     

Failed endoscopic third ventriculostomy in children: management options

Endoscopic third ventriculostomy (ETV) for obstructive hydrocephalus has a failure rate of 20-50% in various series. The present study analyzes ETV failures in 72 patients over a 2-year period and attempts to outline a management plan. Of the 72 patients who underwent ETV, it failed in 13. Seven of these failures occurred within 1 month, and in 5 others, ETV failed after 1-2 months. Another patient had a delayed failure 2 years after the initial surgery. Upon clinical failure, MRI scans were performed in all patients using either T2 fast spin echo or two-dimensional phase contrast MRI techniques. Of these, no flow could be demonstrated in 12 patients, whereas in 1 patient, good flow was observed. Endoscopic exploration was undertaken in the 12 patients in whom flow could not be demonstrated. Of the 12 who underwent endoscopic exploration, a patent stoma was observed in 7, necessitating insertion of a ventriculoperitoneal shunt (VPS). In the other 5, the stoma had closed by gliosis and a repeat ETV was performed. In 3 of these patients, in addition to the ETV, a VPS was also inserted in accordance with the family's wishes. VPS insertion was carried out in the patient with suggestion of good flow through the stoma. In failed ETV, MRI with flow studies is essential to identify the possible cause of failure. Endoscopic exploration is indicated for patients with no evidence of flow. A repeat ETV is indicated in patients with a closed stoma. Patients with a patent stoma could require insertion of a cerebrospinal fluid shunt.