Ventriculosubgaleal shunts for posthemorrhagic hydrocephalus in premature infants

OBJECTIVE: The early management of posthemorrhagic hydrocephalus in premature infants is challenging and controversial. These infants need a temporary cerebrospinal fluid (CSF) diversion procedure until they gain adequate weight, and the blood and protein levels in CSF are reasonably low before permanent shunt can be placed. Various options are available with their associated advantages and disadvantages. Ventriculosubgaleal shunts have been recommended as a more physiologic and less invasive means of achieving this goal. We have performed this procedure in 6 premature infants to evaluate their effectiveness and complications. METHODS: Six consecutive premature infants with posthemorrhagic hydrocephalus underwent placement of ventriculosubgaleal shunts over a 1-year period of time. We reviewed their clinical and imaging progress to assess the ability of the shunt to control hydrocephalus and the complication rates. RESULTS: In all 6 patients, the ventriculosubgaleal shunt controlled the progression of hydrocephalus as assessed by clinical and imaging parameters. A permanent shunt was avoided in 1 patient (16.6%). However, 4 patients developed shunt infections, 1 involving the ventriculosubgaleal shunt itself, and 3 immediately after conversion to ventriculoperitoneal shunt. The total infection rate of the series was 66.6%. All infections were caused by staphylococcus species. There was only a 1% shunt infection rate in our institution for all nonventriculosubgaleal shunts during the same period of time. CONCLUSION: Placement of ventriculosubgaleal shunts for interim CSF diversion in neonates with posthemorrhagic hydrocephalus is effective as a temporary method of CSF diversion. However, our experience has shown that it is associated with a unacceptably high CSF infection rate. A potential cause for infection is CSF stasis just beneath the extremely thin skin of the premature infants, promoting colonization by skin flora. CSF sampling before conversion to a permanent shunt and replacement of the proximal hardware, which has been in situ for a prolonged period, may decrease the infection rates. At present, the procedure is no longer performed at our institution.     

Ascites in ventriculoperitoneal shunt

Objective : To fetch out the factors responsible for ascites, following shunt CSF diversion in cases of intracarnial lesions. Four children developing ascites/abdominal psuedocyst following ventriculoperitoneal shunt were analyzed to see the factors responsible for such complication.Methods : Records of 4 cases developing ascites were studied retrospectively. These children developed ascites at 8 months, 6 months, 1 year and 1 year 2 months interval following their shunt installation.Resuts ;The primary etiology of hydrocephalus was demonstrated as thalamic glioblastoma, choroid plexus papillomas of third ventricle, post tubercular meningitis hydrocephalus and suprasellar craniopharyngioma.Conclusion: The proposed etiology of ascites in these cases was peritoneal metastasis from thalamic glioblastoma through ventriculoperitoneal shunt in first case, excessive production of CSF by choroid plexus papilloma in second, infection in the third case and craniopharyngioma causing excessive production of CSF in the fourth child. All the children were treated by reasonable laparotomy and fenestration of cyst along with the repositioning of shunt tip at another site.     

Thalamic glioblastoma with cerebrospinal fluid dissemination in the peritoneal cavity

Glioblastoma multiforme is one of the commonest primary malignant tumours of the brain with rare incidence of extracranial metastases. Systemic dissemination via the CSF or CSF diversionary shunt procedures is also rare. The reported 9-year-old child was a case of thalamic glioblastoma with hydrocephalus who underwent biventriculoperitoneal shunting before tumour decompression and radiotherapy. The child developed incapacitating ascites 8 months following surgical decompression and 9 months after the shunt diversion which was found to be caused by CSF dissemination of the glioblastoma via the ventriculoperitoneal shunt. The child ultimately succumbed to his disease.     

Alternative uses for the subgaleal shunt in pediatric neurosurgery

The subgaleal shunt has been used for the temporary bypass of the normal cerebrospinal fluid (CSF) pathways. We retrospectively reviewed all subgaleal shunts placed at the Children's Hospital, Birmingham, Ala., USA, from 1997 to the present and examined all uses (e.g. indication, length of follow-up) of the subgaleal shunt outside its use for temporary CSF diversion in premature infants with intraventricular hemorrhage and subsequent hydrocephalus. The average length of survival of the primary subgaleal shunt in this population was 32.2 days. We have had good success with subgaleal shunts in children with malignant brain tumors, intraventricular abscesses, chronic truncal wounds, chronic subdural hygromas and meningitis. However, the greatest utility has been in those scenarios in which the peritoneal cavities were not currently, but with time would be, candidates for distal shunt implantation. Examples of these instances are patients with hydrocephalus and necrotizing enterocolitis or hydrocephalus and preoperative abdominal wall pathology such as omphalocele.     

Management of hydrocephalus in children with plasminogen deficiency.

Congenital plasminogen deficiency is an infrequent disorder, which usually becomes symptomatic as ligneous conjunctivitis. However, pseudomembranous lesions in the mucosa of the pharynx, tracheobronchial tree, and the peritoneum may likewise occur. An accompanying hydrocephalus is extremely rare; only 16 cases have been reported to date. The reports indicate that hydrocephalus, even if treated by ventriculoperitoneal (VP) cerebrospinal fluid (CSF) shunting, worsens the prognosis substantially. Thus, VP CSF shunting does not seem to be the optimal therapy for hydrocephalic children with plasminogen deficiency. We add two cases to the literature, and, on the base of our experience, we propose a management strategy for the hydrocephalus. We report the case history of two children with plasminogen deficiency and associated hydrocephalus. Both children initially were treated with VP shunts and had a very similar clinical course with multiple shunt malfunctions due to nonabsorption by the peritoneum. In the first child, the attempt to treat the hydrocephalus with a ventriculoatrial (VA) shunt failed due to catheter thrombosis. Finally, a ventriculocholecystic shunt was placed in both children, which worked well. In patients with plasminogem deficiency and associated hydrocephalus, special care must be taken in the management of hydrocephalus. The absorptive capacity of the peritoneum is reduced by pseudomembrane formation, which results in VP shunt malfunction. The plasminogen deficiency results in early thrombus formation if atrial catheters are used. Therefore, the authors believe that ventriculocholecystic shunting should be considered early on in the course of the disease.     

Intraventricular neurocysticercus cyst(s) in Indian children

Eleven cases of intraventricular neurocysticercosis were managed over a 10-year period. All patients presented with features of hydrocephalus. In 9 cases the cysts were blocking the cerebrospinal fluid (CSF) pathway resulting in hydrocephalus. The 4 cases of lateral ventricular cyst had communicating hydrocephalus, while 2 of these had asymmetrical ventriculomegaly. Two of 4 cases had more than one cyst. One of 4 children with lateral ventricular cyst had narrowing of the foramen of Monro causing asymmetrical hydrocephalus. Following cyst removal, CSF diversion was not required in 6 cases, while 5 required CSF diversion despite removal of their cysts in 4 of 5. One patient had a cyst at the aqueductal inlet and he was managed by shunt and albendazole therapy. Thus, we managed 6 of our cases with excision of the cysts obstructing the CSF pathway, and in 4 cases excision of cysts with ventriculoperitoneal shunt was adequate.     

Accumulation of transforming growth factor-beta2 and nitrated chondroitin sulfate proteoglycans in cerebrospinal fluid correlates with poor neurologic outcome in preterm hydrocephalus

BACKGROUND: Progressive post-hemorrhagic hydrocephalus in preterm infants strongly predicts abnormal neurologic development, and often accompanies cystic periventricular leukomalacia (cPVL). Transforming growth factor-beta1 (TGF-beta1), associated with hydrocephalus, can upregulate the chondroitin sulfate proteoglycan (CSPG) synthesis. To date, CSPG and their nitrated metabolites (NT-CSPG) have not been evaluated in hydrocephalus. OBJECTIVES: We hypothesized that TGF-beta1, TGF-beta2, CSPG, and NT-CSPG would accumulate in cerebrospinal fluid (CSF) in preterm hydrocephalus, and their concentrations would correlate with poor long-term outcomes. METHODS: TGF-beta1, TGF-beta2, CSPG, and NT-CSPG concentrations in CSF were measured prospectively by ELISA in 29 preterm newborns with (n=22) or without (n=34) progressive post-hemorrhagic hydrocephalus, and correlated with progressive neonatal hydrocephalus and neurologic outcome. Only concentrations from each patient's initial CSF sample were used for statistical analysis. RESULTS: Compared to neonates without hydrocephalus, CSF [TGF-beta1], [TGF-beta2], [CSPG] and [NT-CSPG] were significantly greater by >3-, >35-, >8-, and >3-fold, respectively. Unlike CSF [TGF-beta2] and [CSPG], [TGF-beta1] correlated with CSF [total protein]. Only CSF [NT-CSPG] correlated with cPVL. Unlike [TGF-beta2] or [CSPG], [NT-CSPG] correlation with preterm progressive post-hemorrhagic hydrocephalus (PPHH) was explained entirely by the presence of cPVL among these patients. [TGF-beta2] was >20-fold greater in preterm survivors who required a ventriculoperitoneal shunt for PPHH (n=9), as compared to survivors who did not require a shunt (n=2), or those without hydrocephalus (n=12). [TGF-beta2] and [NT-CSPG] correlated inversely with Bayley Index Scores (15.0 months median adjusted age). CONCLUSIONS: This is the first report that [TGF-beta2], [CSPG], and [NT-CSPG], measured well before term, accumulate abnormally in preterm progressive post-hemorrhagic hydrocephalus CSF, and correlate with adverse neurologic outcome.     

Serotonin and 5-hydroxy indole acetic acid in infantile hydrocephalus

The concentration of metabolites of neurohormones in cerebrospinal fluid (CSF) is an index of turnover of substances in brain parenchyma. The raised intracranial pressure in hydrocephalic children may cause alteration in the metabolism of neurohormones. Serotonin and its metabolite 5-HIAA have been studied extensively in CSF of patients with neuropsychiatric diseases. Hence we studied the neurohormones serotonin and its end product 5-hydroxy indole acetic acid (5-HIAA) in CSF of hydrocephalus infants before and after ventriculoperitoneal (VP) shunt. Ventricular CSF samples form 50 hydrocephalic infants were obtained serially at the time of shunt insertion, and then on day 8 and day 30 postoperatively by direct puncture from shunt chamber using 26G needles. Control CSF samples were taken from otherwise healthy children operated under spinal anesthesia. The samples were analyzed for serotonin and 5-HIAA by spectrofluorophotometric method. At the time of shunt insertion, serotonin was significantly decreased (P < 0.05) while 5-HIAA was significantly increased (P < 0.001) in hydrocephalic infants. On day 8 and day 30 values of serotonin and 5-HIAA approached the baseline values. In patients who developed VP shunt blockade there was again a rise in levels of 5-HIAA. However, no correlation could be established between the levels of serotonin, 5-HIAA and the duration of hydrocephalus and the type of hydrocephalus. Our study shows increased 5-HIAA concentration in CSF indicating increased turnover of serotonin to its metabolite due to pressure changes in hydrocephalus. Long-term follow-up is required to assess if they could be of prognostic significance as regards to long term attainment of brain functions in hydrocephalic children.     

A case of cerebrospinal fluid eosinophilia associated with shunt malfunction

A 3-month-old female patient presented with a meningomyelocele at the lumber region associated with congenital hydrocephalus. She underwent ventriculoperitoneal (V-P) shunt surgery using the Sophy system. The shunt system was replaced due to a malformation. Following replacement, the patient presented with cerebrospinal fluid (CSF) eosinophilia at the age of 8 months. The eosinophilic granulocytosis of the CSF improved dramatically following systemic prednisolone administration. CSF eosinophilia without accompanying inflammation or pyrexia in the present case may have resulted from an allergic response to a foreign material such as the silicone tube pressure valve of the Sophy system or the sutures rather than bacterial or fungal infection. Based on our results, we believe that some patients may experience CSF eosinophilia following postoperative V-P shunt due to an allergic reaction to the shunt equipment. Prompt steroid treatment can produce spontaneous regression in such cases.     

Endoscopic coagulation of choroid plexus hyperplasia

Hydrocephalus is a clinical disorder resulting from an imbalance between the production of CSF and its resorption, of which the latter is mostly a disadvantage. In rare cases of choroid plexus papilloma or carcinoma, hydrocephalus is due to an overproduction of CSF. Choroid plexus hyperplasia (CPH) is a distinct clinicopathological entity in which the enlarged choroid plexus produces large amounts of CSF. Historically, patients with CPH were treated by shunt procedures or by microsurgical removal of the choroid plexus, which is associated with a high complication rate. In this paper the authors show that endoscopic plexus coagulation can result in restoring the equilibrium of the intracranial fluid volumes, resulting in shunt independency. In this way, both the shunt-related complications and the bleeding risks of microsurgical plexectomy are avoided. In instances of hydrocephalus, thorough efforts should be made to demonstrate the underlying pathophysiology to choose the optimal treatment, of which shunt procedures should receive the least priority.     

Subdural empyema complicating cerebrospinal fluid shunt infection.

Subdural empyema has not been reported previously as a complication of cerebrospinal fluid (CSF) shunt surgery. An infant submitted to CSF shunt insertion for congenital hydrocephalus developed subdural empyema after a failed attempt to treat a superficial scalp wound infection with oral antibiotics. Enterobacter cloacae was isolated from the empyema. Temporizing management of the preceding superficial wound infection with oral antibiotics probably was the cause of this exotic pathogen. The treatment of infected scalp wounds contiguous with shunt hardware must be surgical.     

Use of acetazolamide to decrease cerebrospinal fluid production in chronically ventilated patients with ventriculopleural shunts

Acetazolamide (ACTZ), a carbonic anhydrase inhibitor, has been shown to decrease cerebrospinal fluid (CSF) production in both in vivo and in vitro animal models. We report two children with hydrocephalus who experienced multiple shunt failures, and who had externalised ventriculostomy drains (EVD) prior to ventriculopleural shunt placement. The effects of increasing doses of ACTZ on CSF production and subsequent tolerance to ventriculopleural shunts were evaluated. The patients had a 48% and a 39% decrease in their EVD CSF output when compared to baseline with maximum ACTZ dose of 75 mg/kg/day and 50 mg/kg/day, respectively (p < 0.05). This is the first report of change in CSF volume in children after extended treatment with ACTZ. ACTZ treatment in mechanically ventilated paediatric patients with hydrocephalus may improve tolerance of ventriculopleural shunts and minimise respiratory compromise. Potassium and bicarbonate supplements are required to correct metabolic disturbances.     

Use of acetazolamide to decrease cerebrospinal fluid production in chronically ventilated patients with ventriculopleural shunts.

Acetazolamide (ACTZ), a carbonic anhydrase inhibitor, has been shown to decrease cerebrospinal fluid (CSF) production in both in vivo and in vitro animal models. We report two children with hydrocephalus who experienced multiple shunt failures, and who had externalised ventriculostomy drains (EVD) prior to ventriculopleural shunt placement. The effects of increasing doses of ACTZ on CSF production and subsequent tolerance to ventriculopleural shunts were evaluated. The patients had a 48% and a 39% decrease in their EVD CSF output when compared to baseline with maximum ACTZ dose of 75 mg/kg/day and 50 mg/kg/day, respectively (p < 0.05). This is the first report of change in CSF volume in children after extended treatment with ACTZ. ACTZ treatment in mechanically ventilated paediatric patients with hydrocephalus may improve tolerance of ventriculopleural shunts and minimise respiratory compromise. Potassium and bicarbonate supplements are required to correct metabolic disturbances.     

Massive ascites formation due to unabsorbed cerebrospinal fluid following abdominal surgery in ventriculoperitoneal shunt. A case report

A 15-year-old girl with a congenital heart disease (pulmonary stenosis and aortic insufficiency), who had a ventriculoperitoneal shunt operation because of hydrocephalus internus at the age of two years, 12 years later underwent colectomy because of juvenile colonic polyposis. After this operation, enormous production of ascites began which, because of progressive development, finally required ventriculoatrial shunt operation. A total volume of 17 liters of fluid was removed in two ascites drainages before and during the latter operation. Postoperatively the ascites production stopped completely.     

Overdrainage in the treatment of hydrocephalus]

In infants or children, hydrocephalus is usually the consequence of an obstacle in the cerebro-spinal fluid (CSF) pathways and is most frequently treated by the insertion of a ventriculo-peritoneal shunt (rarely ventriculo-atrial). The CSF flow through such a shunt is equal to the ratio of the difference (DP) between the CSF input and output pressures over the valve resistance. When the child is in the upright position, a DP increase occurs due to the height of the hydrostatic column between the inlet and the shunt outlet. Thus if the shunt drains correctly in the prone position, it overdrains in the upright position. As the CSF flow through the shunt in standing patients is higher than the CSF secretion, the excess fluid will be taken out of the ventricles, resulting in pericerebral collections, slit ventricles or post-shunt craniostenoses. Many different techniques have been proposed to reduce overdrainage. The Orbis-Sigma shunt was designed in the "Service des Enfants-Malades" precisely for this purpose. In the upright position this shunt becomes a flow regulator and thus reduces overdrainage. Its use has decreased by 2-fold the number of mechanical complications related to overdrainage.     

Hydrocephalus due to diffuse villous hyperplasia of the choroid plexus. Case report and review of the literature

Diffuse villous hyperplasia of the choroid plexus (DVHCP) that causes hydrocephalus by overproduction of cerebrospinal fluid is a very rare lesion and difficult to diagnose initially. We present a male infant who was diagnosed with communicating hydrocephalus at 16 months of age and treated initially by ventriculoperitoneal shunt. In the postoperative course, he developed massive ascites. Magnetic resonance imaging obtained after the operation revealed DVHCP. Resection of the choroid plexus of the left lateral ventricle was performed, and postoperatively, his ascites was resolved. We review the literature and discuss the diagnosis and treatment of this rare entity.     

细菌性脑膜炎并脑积水的临床特点及危险因素

目的探讨细菌性脑膜炎并脑积水的临床特点,并分析其相关的危险因素,以降低其发生率,改善细菌性脑膜炎的预后。方法对2004年1月-2010年6月本院收治的111例细菌性脑膜炎患儿的临床资料进行回顾性分析。根据影像学结果,将患儿分为细菌性脑膜炎并脑积水组(n=16)及细菌性脑膜炎无脑积水组(n=95),应用SPSS 17.0软件对2组患儿的基本情况、临床表现、实验室结果及抗生素治疗情况等进行比较,对相关因素进行Logistic回归分析,筛选出脑积水发生的高危因素。结果细菌性脑膜炎并脑积水的发生率为14.4%(16/111例),以梗阻性脑积水为主(14/16例,87.5%),75%(12/16例)患儿脑积水出现在起病4周内,确诊时CT/MRI的检查次数为1～3次,2例患儿在起病后2个月行脑室-腹腔分流术,2例脑积水患儿死亡。2组临床资料比较显示年龄、发热>10 d、惊厥、意识障碍、经验性治疗失败、颅内低密度灶、低Hb水平、高脑脊液蛋白水平、低脑脊液葡萄糖水平均与脑积水发生有关(Pa<0.05),Logistic回归分析显示意识障碍、经验性治疗失败、低Hb水平是细菌性脑膜炎并脑积水的独立危险因素。结论脑积水是细菌性脑膜炎的一个严重并发症之一,临床表现及实验室检查结果可作为细菌性脑膜炎并脑积水的预测指标。     

Measurement of cerebrospinal fluid output through external ventricular drainage in one hundred infants and children: correlation with cerebrospinal fluid production

OBJECTIVE: Cerebrospinal fluid (CSF) production rates influence shunt design and the care of children with hydrocephalus. Measurement of hourly CSF output through external ventricular drainage (EVD) reflects the CSF production. In the present study, hourly CSF outputs in children with hydrocephalus were measured while they were treated with EVD and correlated with the age, sex and body weight of the patients as well as other clinical parameters. METHODS: One hundred children with hydrocephalus due to various causes had EVD treatment. Twenty-six had EVD on two or three separate occasions; thus, the CSF output measurements were observed and analyzed on the basis of 130 EVDs. The most common reason for EVD treatment was shunt infection (n = 75). The duration of EVDs ranged from 25 to 774 h (mean 269 h). The height of the drip chamber from the mid-head position ranged from 0 to 23 cm (mean 9.8 cm). The hourly CSF output was analyzed according to the patient's age, sex and body weight as well as the presence of CSF infection. RESULTS: The hourly CSF output rapidly increases during the first year of life. By the second year, it reaches 64% of the hourly CSF output of 15-year-old children. The mean hourly output ranged from 0.1 to 26.5 ml/h (mean 8.1 ml/h), with the standard deviation ranging from 0.4 to 10.8 ml/h (mean 5.2 ml/h). A regression analysis indicated that the age and body weight appeared to correlate with the hourly CSF output. Using the natural logarithm of age, body weight and sex, these predictors accounted for 50.9% of the variability in hourly CSF output. The regression equation is as follows: hourly CSF output = 2.78 - 2.23(male = 0, female = 1) + 0.97 log(age in years) + 2.26 log(body weight in kg). R sd = 3.36, R(2) = 0.509. The type of infecting organism and the height of EVD did not influence the overall CSF output. CONCLUSION: The hourly CSF output fluctuates, but the CSF output increases logarithmically with age and body weight. The gender also influences the CSF output, with males having a greater output than females. The data produced by the present study will help us to understand CSF production rates in developing children. They will also help us in the care of children receiving EVD treatment, as well as in selecting and designing shunt systems.     

Late presentation of massive pleural effusion from intrathoracic migration of a ventriculoperitoneal shunt catheter: case report and review of the literature

We report an unusual case of ventriculoperitoneal (VP) shunt intrathoracic migration, associated with massive symptomatic hydrothorax. The VP shunt was inserted 10 years before presentation, after hemorrhagic hydrocephalus caused by prenatal intraventricular hemorrhage. The pleural fluid was drained via tube thoracostomy and the shunt was externalized, with full resolution of symptoms and signs. The patient was subsequently managed with shunt revision with drainage into the abdominal cavity. We review the 10 pediatric cases of cerebrospinal fluid hydrothorax reported in the literature and discuss the mechanism of shunt tip migration. Pleural effusion secondary to VP shunt insertion is a rare and potentially life-threatening occurrence, and it should be suspected in any patient with a VP shunt and respiratory distress.     

Elevated nerve growth factor and neurotrophin-3 levels in cerebrospinal fluid of children with hydrocephalus

Background

Elevated intracranial pressure (ICP) resulting from impaired drainage of cerebrospinal fluid (CSF) causes hydrocephalus with damage to the central nervous system. Clinical symptoms of elevated intracranial pressure (ICP) in infants may be difficult to diagnose, leading to delayed treatment by shunt placement. Until now, no biochemical marker of elevated ICP has been available for clinical diagnosis and monitoring. In experimental animal models, nerve growth factor (NGF) and neurotrophin-3 (NT-3) have been shown to be produced by glial cells as an adaptive response to hypoxia. We investigated whether concentrations of NGF and NT-3 are increased in the CSF of children with hydrocephalus.

Methods

NGF was determined in CSF samples collected from 42 hydrocephalic children on 65 occasions (taps or shunt placement surgery). CSF samples obtained by lumbar puncture from 22 children with suspected, but unconfirmed bacterial infection served as controls. Analysis was performed using ELISA techniques.

Results

NGF concentrations in hydrocephalic children were over 50-fold increased compared to controls (median 225 vs 4 pg/mL, p < 0.0001). NT-3 was detectable (> 1 pg/mL) in 14/31 hydrocephalus samples at 2–51 pg/mL but in none of 11 control samples (p = 0.007).

Conclusion

NGF and NT-3 concentrations are increased in children with hydrocephalus. This may represent an adaptive response of the brain to elevated ICP.