Category: Pediatrics

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Complications following decompression of Chiari malformation Type I in children: dural graft or sealant?

J Neurosurg Pediatrics 8:177–183, 2011. DOI: 10.3171/2011.5.PEDS10362

Posterior fossa decompression with duraplasty for Chiari malformation Type I (CM-I) is a common pediatric neurosurgery procedure. Published series report a complication rate ranging from 3% to 40% for this procedure. Historically, many dural substitutes have been used, including bovine grafts, human cadaveric pericardium, synthetic dura, and autologous pericranium. The authors hypothesized that a recently observed increase in complications was dependent on the graft used.

Methods. Between January 2004 and January 2008, 114 consecutive patients ≤ 18 years old underwent primary CM-I decompression using duraplasty. Records were retrospectively reviewed for short- and intermediate-term complications and operative technique, focusing on the choice of duraplasty graft with or without application of a tissue sealant.

Results. The average age of the patients was 8.6 years. The dural graft used was variable: 15 were treated with cadaveric pericardium, 12 with Durepair, and 87 with EnDura. Tisseel was used in 75 patients, DuraSeal in 12, and no tissue sealant was used in 27 patients. The overall complication rate was 21.1%. The most common complications included aseptic meningitis, symptomatic pseudomeningocele, or a CSF leak requiring reoperation. The overall complication rates were as follows: cadaveric pericardium 26.7%, Durepair 41.7%, and EnDura 17.2%; reoperation rates were 13%, 25%, and 8.1%, respectively. Prior to adopting a different graft product, the overall complication rate was 18.1%; following the change the rate increased to 35%. Complication rates for tissue sealants were 14.8% for no sealant, 18.7% for Tisseel, and 50% for DuraSeal. Nine patients were treated with the combination of Durepair and DuraSeal and this subgroup had a 56% complication rate.

Conclusions. Complication rates after CM-I decompression may be dependent on the dural graft with or without the addition of tissue sealant. The complication rate at the authors’ institution approximately doubled following the adoption of a different graft product. Tissue sealants used in combination with a dural substitute to augment a duraplasty may increase the risk of aseptic meningitis and/or CSF leak. The mechanism of the apparent increased inflammation with this combination remains under investigation.

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Neuropathological and Neuroradiological Spectrum of Pediatric Malignant Gliomas: Correlation With Outcome

Neurosurgery 69:215–224, 2011 DOI: 10.1227/NEU.0b013e3182134340

The diagnostic accuracy and reproducibility for glioma histological diagnosis are suboptimal.

OBJECTIVE: To characterize radiological and histological features in pediatric malignant gliomas and to determine whether they had an impact on survival.

METHODS: We retrospectively reviewed a series of 96 pediatric malignant gliomas. All histological samples were blindly and independently reviewed and classified according to World Health Organization 2007 and Sainte-Anne classifications. Radiological features were reviewed independently. Statistical analyses were performed to investigate the relationship between clinical, radiological, and histological features and survival.

RESULTS: Cohort median age was 7.8 years; median follow-up was 4.8 years. Tumors involved cerebral hemispheres or basal ganglia in 82% of cases and brainstem in the remaining 18%. After histopathological review, low-grade gliomas and nonglial tumors were excluded (n = 27). The World Health Organization classification was not able to demonstrate differences between groups and patients survival. The Sainte-Anne classification identified a 3-year survival rate difference between the histological subgroups (oligodendroglioma A, oligodendroglioma B, malignant glioneuronal tumors, and glioblastomas; P = .02). The malignant glioneuronal tumor was the only glioma subtype with specific radiological features. Tumor location was significantly associated with 3-year survival rate (P = .005). Meningeal attachment was the only radiological criteria associated with longer survival (P = .02).

CONCLUSION: The Sainte-Anne classification was better able to distinguish pediatric malignant gliomas in terms of survival compared with the World Health Organization classification. In this series, neither of these 2 histological classifications provided a prognostic stratification of the patients.

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Neurosurgical treatment of craniopharyngioma in adults and children: early and long-term results in a large case series

J Neurosurg 114:1350–1359, 2011.DOI: 10.3171/2010.11.JNS10670

Craniopharyngioma accounts for 2%–5% of all primary intracranial neoplasms. The optimal management of craniopharyngioma remains controversial. The authors evaluated the early results of surgery and the longterm risk of tumor recurrence in a large series of patients undergoing resection of craniopharyngiomas.

Methods. Between 1990 and 2008, 112 consecutive patients (57 male and 55 female patients with a mean [± SEM] age of 33.3 ± 1.8 years) underwent resection of craniopharyngiomas at the authors’ hospital. Recurrence or growth of residual tumor tissue during follow-up was assessed using MR imaging.

Results. There were 3 perioperative deaths (2.7%). Severe adverse events were more frequent in patients who underwent operations via the transcranial route (37%) than the transsphenoidal approach (5.6%; p < 0.001). Magnetic resonance imaging showed radical resection of the tumor in 78 (71.6%) of the remaining 109 patients. Previous surgery and maximum tumor diameter were associated with persistence of disease after surgery. Craniopharyngioma recurred in 26 (24.5%) of 106 patients. Presence of residual tumor on the first postoperative MR imaging, male sex, and no postoperative radiation therapy were associated with a risk of tumor recurrence. Quality-of-life data were assessed in the 91 patients who attended the authors’ institution for follow-up visits. Among them, 8.8% patients were partially or completely dependent on others for daily living activities before surgery. This percentage increased to 14.3% at the last follow-up visit. The 5- and 10-year overall survival rates were 94.4% (95% CI 90.0%–98.8%) and 90.3% (95% CI 83.4%–97.3%), respectively.

Conclusions. Complete surgical removal of craniopharyngioma can be achieved with reasonable safety in more than 70% of patients. Recurrence of craniopharyngioma may occur even after apparent radical excision. Prompt management of residual or recurring disease by radiotherapy, repeat surgery, or a combination of both is usually successful in controlling further tumor growth.

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Microscopic endonasal transsphenoidal pituitary adenomectomy in the pediatric population

J Neurosurg Pediatrics 7:000–000, 2011.DOI: 10.3171/2011.2.PEDS10278

Pituitary adenomas are uncommon in childhood. Although medical treatment can be effective in treating prolactinomas and some growth hormone (GH)–secreting tumors, resection is indicated when visual function is affected or the side effects of medical therapy are intolerable. The authors of this report describe their 10-year experience in managing pituitary adenomas via the microscopic endonasal transsphenoidal approach in a pediatric population.

Methods. They performed a retrospective review of a surgical case series based at a single institution and consisting of 34 consecutive pediatric patients with endocrine-active (32 patients) and endocrine-inactive (2 patients) adenomas. These patients were surgically treated via an endonasal transsphenoidal approach between 1999 and 2008. Patient charts were reviewed, and clinical data were compiled and analyzed using the chi-square and Kaplan-Meier tests.

Results. The patient cohort consisted of 20 girls and 14 boys, with ages ranging from 9 to 18 years and a median age of 16 years. Thirty-two patients (94%) underwent surgery for endocrine-active tumors, including 10 (29%) with Cushing disease, 21 (62%) with prolactinomas, and 1 (3%) with GH-secreting tumors. Two patients with nonsecreting adenomas underwent surgery for apoplexy. The mean tumor volume was 5.4 cm3, and 13 patients (38%) had suprasellar extension and 7 (21%) had cavernous sinus invasion. Gross-total resection was achieved in 26 patients (76%), although it was significantly less likely to be achieved in the setting of cavernous sinus invasion (p < 0.001) but was unaffected by suprasellar extension. Residual tumor was treated with radiation therapy in 6 patients (18%). The average duration of hospital stay was 1.6 days. The median follow-up time was 18 months. After surgery, 19 patients (56%) had normal hormone function without adjuvant therapy, 8 (24%) had normal function with adjuvant therapy, and 5 (15%) had persistently elevated hormone levels. Patients with a macroprolactinoma were significantly more likely to require postoperative adjuvant therapy than were those with a microprolactinoma (p < 0.03).

Conclusions. Endonasal transsphenoidal resection is a safe, well-tolerated, and potentially curative treatment option for pituitary adenomas in children. Despite the technical challenges associated with this approach in the pediatric population, these tumors can be effectively managed with minimal morbidity. Endocrine function is usually preserved, and the majority of patients will not require lifelong medical therapy.

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Outcomes in pediatric patients with Chiari malformation Type I followed up without surgery

J Neurosurg Pediatrics 7:000–000, 2011.DOI: 10.3171/2011.1.PEDS10341

The natural history of untreated Chiari malformation Type I (CM-I) is poorly defined. The object of this study was to investigate outcomes in pediatric patients with CM-I who were followed up without surgical intervention.

Methods. The authors retrospectively reviewed 124 cases involving patients with CM-I who presented between July 1999 and July 2008 and were followed up without surgery. The patients ranged in age from 0.9 to 19.8 years (mean 7 years). The duration of follow-up ranged from 1.0 to 8.6 years (mean 2.83 years). Imaging findings, symptoms, and findings on neurological examinations were noted at presentation and for the duration of follow-up.

Results. The mean extent of tonsillar herniation at presentation was 8.35 mm (range 5–22 mm). Seven patients had a syrinx at presentation. The syrinx size did not change in these patients on follow-up imaging studies. No new syrinxes developed in the remaining patients who underwent subsequent imaging. The total number of patients with presenting symptoms was 81. Of those 81 patients, 67 demonstrated symptoms that were not typical of CM-I. Of the 14 patients with symptoms attributed to CM-I, 9 had symptoms that were not severe or frequent enough to warrant surgery, and surgery was recommended in the remaining 5 patients. Chiari malformation Type I was also diagnosed in 43 asymptomatic patients who had imaging studies performed for various reasons. No new neurological deficits were noted in any patient for the duration of follow-up.

Conclusions. The majority of patients with CM-I who are followed up without surgery do not progress clinically or radiologically. Longer follow-up of this cohort will be required to determine if symptoms or new neurological findings develop over the course of many years.

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Surgical treatment of trigonocephaly: technique and long-term results in 48 cases

J Neurosurg Pediatrics 7:000–000, 2011.DOI: 10.3171/2010.12.PEDS10359

The authors present their experience in the surgical treatment of metopic synostosis by orbital bandeau remodeling and frontal bone rotation. The pitfalls and advantages of the surgical technique are discussed, along with the long-term clinical results in 48 consecutive cases.

Methods. Forty-eight consecutive patients in whom trigonocephaly was diagnosed between 1990 and 2009 were treated with frontal bone rotation and frontoorbital bandeau remodeling. Of these patients, 38 (79%) were boys and 10 (21%) were girls. The age at the time of surgical treatment ranged between 4 and 42 months (mean ± SD 11.4 ± 8.7 months). The average follow-up period was 5.5 ± 4.2 years (range 5 months–19 years). The preoperative and latest postoperative photographs of the patients were evaluated for the following features: 1) shape of the forehead; 2) hypotelorism; and 3) temporal depression. Scores of 0, 1, or 2 were assigned for each item: 0 was normal, 1 meant moderate deformity, and 2 denoted severe deformity.

Results. In the early postoperative period, no complications were documented. The average hospitalization period was 4 days. Follow-up radiographs or 3D CT scans were obtained at regular intervals. The mean preoperative scores for the evaluated items were 1.38 ± 0.49 for the shape of the forehead, 1.33 ± 0.48 for hypotelorism, and 1.7 ± 0.46 for the temporal depression. The mean postoperative scores were 0.06 ± 0.24 for the shape of the forehead, 0.21 ± 0.4 for hypotelorism, and 0.67 ± 0.48 for the temporal depression. Overall, the total preoperative score dropped from 4.4 to 0.93 postoperatively (p < 0.05). All the patients were contented with the cosmetic results.

Conclusions. Early detection and treatment of metopic suture synostosis has a significant, favorable influence on the outcomes. Good understanding of the structural abnormality and the pathophysiological mechanisms of the possible complications is very important for performing proper surgical reconstruction.

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Variability among pediatric neurosurgeons in the threshold for ventricular shunting in asymptomatic children with hydrocephalus

J Neurosurg Pediatrics 7:000–000, 2011.DOI: 10.3171/2010.11.PEDS10275

The thresholds for shunting CSF in children with asymptomatic hydrocephalus are unclear; there are neither guidelines nor sufficient research to determine what degree of hydrocephalus should be treated. The authors hypothesize that 1) pediatric neurosurgeons currently have high thresholds for recommending treatment for these children, but 2) there is significant variability among these treatment thresholds.

Methods. The authors surveyed attendees of the Joint Pediatric Neurosurgery Section meeting in Spokane, Washington, in December 2008, regarding their treatment thresholds for 22 clinical scenarios. Each participant was provided an illustration of 5 imaging studies (3 slices each) showing progressively larger ventricles. For each scenario, respondents were asked to indicate the minimum ventricular size they would treat, if any. Responses were quantified from 1 to 6 from smaller to larger, with 6 being no treatment, and a mean theoretical treated ventricular size (MTTVS) was calculated for each scenario.

Results. Respondents were relatively conservative in recommending treatment, with MTTVSs of 3.7–6.0; in 13 scenarios, the MTTVS was greater than 5.0 (larger than the largest presented ventricular size). For scenarios in which a mean frontooccipital ratio could be calculated, the value ranged from 0.55 to 0.67 (moderate to severe hydrocephalus). Although there were clear majority responses for each scenario, there was also significant variability. There were no patterns of association with the respondent’s age, training, board certification, or type or location of practice.

Conclusions. This study demonstrates that pediatric neurosurgeons’ thresholds for treating asymptomatic children with hydrocephalus are generally high, but there is also significant variability.

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Shunting for the Treatment of Arachnoid Cysts in Children

Neurosurgery 67:1632–1636, 2010 DOI: 10.1227/NEU.0b013e3181f94476

The optimal treatment of symptomatic arachnoid cysts remains controversial.

OBJECTIVE: We present a single-institution experience on surgical treatment of arachnoid cysts.

METHODS: Between January 1983 and January 2008, 92 patients with symptomatic arachnoid cysts underwent surgery at our institute. All patients were evaluated with CT and/or MRI and were operated upon and had regular follow-up examinations.

RESULTS: There were 60 males and 32 females. The mean age was 3.6 years (range, 2 days to 14 years). Forty-eight cysts (52%) were within the sylvian fissure, 16 (17%) were in the cerebral convexity, 15 (16%) were infratentorial, 10 (11%) were in the interhemispheric fissure, and 3 (4%) were parasellar. Cystoperitoneal shunt was placed in 67 (73%) patients; ventricular drainage alone was performed in 14 (15%) patients; and combined drainage of the ventricular system and cyst, using a 3-way connector, was performed in 8 (9%) patients. Craniotomy and fenestration of the cyst was performed in 2 (2%) patients, whereas total excision of the cyst was performed in 1 patient. Shunt revision for various reasons was performed in 34 patients (39%), whereas 13 (14%) patients required more than one revision. Complete alleviation of symptoms was achieved in all patients after treatment, regardless of cyst reduction. After a mean follow-up of 8.5 years no further enlargement of the arachnoid cysts was noted.

CONCLUSION: Shunt placement is a safe and effective surgical treatment of symptomatic arachnoid cysts in children. Cyst reduction is greater in children younger than 2 years of age.

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Posterior fossa ependymomas: new radiological classification with surgical correlation

Childs Nerv Syst (2010) 26:1765–1772. DOI 10.1007/s00381-010-1251-6

The key determinant of long-term outcome in infratentorial ependymomas remains the extent of surgical resection. We describe a new radiological classification system which is validated against surgical findings and correlated with risk of post-operative residual tumour.

Methods Twenty-five consecutive patients (12 females, mean age 4.9 years, range 0.5–17 years) with infratentorial ependymomas were studied. Lesions were classified on preoperative MRI according to the pattern of extension, brainstem displacement and involvement of the obex, as lateral-type or midfloor-type tumours. Twenty-one operative records were reviewed with respect to the microanatomical tumour origin by a paediatric neurosurgeon, blinded to MRI findings. Follow-up imaging studies were evaluated for residual tumour.

Results There were 15 cases of midfloor-type tumour (anterior displacement of brainstem, infiltration of obex) and 10 cases of lateral-type tumour (lateral displacement of brainstem, obex free of tumour). Extension into prepontine or cerebellopontine cisterns was more common in lateraltype tumours. Agreement between the radiological classification and tumour origin, as defined by operative records, was seen in 18 out of 20 cases. Risk of residual tumour in lateral-type tumours was more than twice that of midfloortype tumours (80% vs. 33%, p=0.04). Risk of tumour residual was also significantly higher when vessel encasement or prepontine extension was observed.

Conclusions Infratentorial ependymomas can be preoperatively classified as lateral-type or midfloor-type tumours. This correlates well with operative findings. Lateral-type tumours have significantly increased risk of residual tumour compared to midfloor- type tumours and this may influence intensity of imaging surveillance.

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Spinal cord traction, vascular compromise, hypoxia, and metabolic derangements in the pathophysiology of tethered cord syndrome

Neurosurg Focus 29 (1):E9, 2010. (10.3171/2010.3.FOCUS1085)

The definition of tethered cord syndrome (TCS) relies mainly on radiological criteria and clinical picture. The presence of a thickened filum terminale and a low-lying conus medullaris in symptomatic patients is indicative of TCS. The radiological definition of TCS does not take into account cases that involve a normal-lying conus medullaris exhibiting symptoms of the disease.

Methods. The authors performed a MEDLINE search using the terms “tethered cord” and “pathophysiology.” The search returned a total of 134 studies. The studies were further filtered to identify mostly basic research studies in animal models or studies related to the biomechanics of the filum terminale and spinal cord.

Results. Spinal cord traction and the loss of filum terminale elasticity are the triggers that start a cascade of events occurring at the metabolic and vascular levels leading to symptoms of the disease. Traction on the caudal cord results in decreased blood flow causing metabolic derangements that culminate in motor, sensory, and urinary neurological deficits. The untethering operation restores blood flow and reverses the clinical picture in most symptomatic cases.

Conclusions. Although classically defined as a disease of a low-lying conus medullaris, the pathophysiology of TCS is much more complex and is dependent on a structural abnormality, with concomitant altered metabolic and vascular sequelae. Given the complex mechanisms underlying TCS, it is not surprising that the radiological criteria do not adequately address all presentations of the disease.

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Decompression of Chiari malformation with and without duraplasty: morbidity versus recurrence

J Neurosurg Pediatrics 5:474-478, 2010. DOI: 10.3171/2010.1.PEDS09218

The optimal surgical management of Chiari malformation (CM) is evolving. Evidence continues to accrue that supports decompression without duraplasty as an effective treatment to achieve symptomatic relief and anatomical decompression. The risks and benefits of this less invasive operation need to be weighed against decompression with duraplasty.

Methods. The authors performed a retrospective review of all CM decompressions from 2003 to 2007. All operations were performed by a single surgeon at a single institution. Data were analyzed for outcome, postoperative morbidity, and recurrence.

Results. Of 121 unique patients, 56 underwent posterior fossa decompressions without duraplasty (PFD) and 64 patients underwent posterior fossa decompressions with duraplasty (PFDD). Of the 56 PFD patients, 7 (12.5%) needed a subsequent PFDD for symptomatic recurrence. Of the 64 patients who underwent a PFDD, 2 (3.1%) needed a repeated PFDD for symptomatic recurrence. Patients treated with PFDD had an average operative time of 201 minutes in contrast to 127 minutes for those who underwent PFD (p = 0.0001). Patients treated with PFDD had average hospital stays of 4.0 days, whereas that for patients treated with PFD was 2.7 days (p = 0.0001). While in the hospital, patients treated with PFDD used low-grade narcotics, intravenous narcotics, muscle relaxants, and antiemetic medications at statistically significant differing rates.

Conclusions. While PFD was associated with a higher rate of recurrent symptoms requiring repeated decompression, this may be justified by the significantly lower morbidity rate. Clearer delineation of the trade-off between morbidity and recurrence may be used to help patients and their families make decisions regarding care.


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Telemedicine Through the Use of Digital Cell Phone Technology in Pediatric Neurosurgery: A Case Series

Neurosurgery. 66(5):999-1004, May 2010. DOI: 10.1227/01.NEU.0000368443.43565.2A

Advances in medicine have largely followed advances in technology. Medical strides have been made when physicians and researchers have adapted growing science to target specific problems. A new medical field, telemedicine, has emerged that links physicians with colleagues and patients. Cell phone technology is affordable for almost everyone, and basic models include digital photography.

OBJECTIVE: We present a case series exhibiting the utility of digital pictures taken with patients’ cell phones.

CLINICAL PRESENTATION: Our patients had wound infections requiring daily intravenous antibiotics and dressing changes. Previously, these patients would have required prolonged hospitalizations. Currently, patients with these infections are discharged from the hospital, but close outpatient observation is required to monitor the wound. Our patients lived up to 8 hours away from the hospital. Daily appointments for wound checks in the clinic were impractical. Wounds were monitored via cell phone images without the inconvenience of travel and the expense of a local hotel, and unnecessary appointments in the clinic.

INTERVENTION: Wound evaluations were conducted with the patients’ cell phone cameras. These images were transmitted to the surgeon by text messaging and emails.

CONCLUSION: This application of cell phone technology has been documented in other literature and could become a legitimate method for close outpatient observation by neurosurgeons if medicolegal issues are addressed.

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Multimodality treatment of cerebral AVMs in children: a single-centre 20 years experience

Childs Nerv Syst (2010) 26:681–687.DOI 10.1007/s00381-009-1039-8

The purpose of this study was to review our experience with a multimodality treatment approach in the management of cerebral arteriovenous malformation (AVM) in children.

Methods We retrospectively analysed a consecutive series of 56 children who harboured a cerebral AVM and were treated at our institution between 1988 and 2008. During the whole treatment period, a combined treatment strategy, including microsurgery, endovascular treatment and gamma knife radiosurgery, was used.

Results Of the 56 patients (median age, 14.0; range, 3 months–18 years) reported, 36 (64.3%) were admitted after AVM rupture; of these, only one AVM (1.8%) was considered untreatable. In 30.9% (17/55) of the treated patients, a single treatment measure was sufficient to attain angiographic cure of the AVM. Among these, six patients (10.9%) had microsurgical AVM resection, four patients (7.3%) underwent endovascular treatment, and another seven patients (12.7%) underwent radiosurgical management of the AVM. The majority of the population (38/55; 69.1%) underwent combined treatment: 21 patients (38.2%) received embolisation followed by radiosurgery of the remnant nidus, ten patients (18.2%) underwent embolisation with subsequent surgical resection of the residual AVM, three patients (5.5%) had radiosurgery after incomplete surgical AVM nidus resection and another four patients (7.3%) required a combination of all three treatment modalities to achieve permanent angiographic cure of the AVM. We observed good clinical outcomes (Glasgow Outcome Scale 5 and 4) in 94.6% of the children. Complete angiographic obliteration was achieved in 93.3% of the patients treated.

Conclusion A multimodality treatment approach in children harbouring cerebral AVMs leads to excellent angiographic and clinical outcomes.

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Magnetic resonance imaging–graded hypothalamic compression in surgically treated adult craniopharyngiomas determining postoperative obesity

Neurosurg Focus 28 (4):E3, 2010. DOI: 10.3171/2010.1.FOCUS09303

Obesity as a consequence of management of pediatric craniopharyngioma is a well-described phenomenon related to the degree of hypothalamic involvement. However, weight change and obesity have not been analyzed in adult patients. Therefore, the purpose of this study was 1) to evaluate the pattern of postoperative weight gain related to preoperative body mass index (BMI), 2) determine if postoperative weight gain is an issue in adult patients, and 3) develop an objective MR imaging grading system to predict risk of postoperative weight gain and obesity in adults treated for craniopharyngioma.

Methods. The authors retrospectively screened 296 patients with known craniopharyngioma for the following inclusion criteria: pathologically confirmed craniopharyngioma, index surgery at the authors’ institution, and operative weight and height recorded with at least 3 months of follow-up including body weight measurement. Patients aged 18 years or younger were excluded, yielding 28 cases for analysis. Cases of craniopharyngiomas were compared with age- and sex-matched controls (pituitary adenoma patients) to evaluate the pattern and significance of perioperative weight changes.

Results. Mean age was 46 ± 17 years at surgery, and 64% of the patients were male. Complete resection was achieved in 71% of cases. There was no correlation of preoperative BMI and postoperative weight gain testing in a linear model. Sixty-one percent and 46% of patients had postoperative weight gains greater than 4 and 9%, respectively. Comparing craniopharyngioma patients (cases) to age- and sex-matched controls, the preoperative BMIs were similar (p = 0.93) between cases (mean 28.9 [95% CI 30.9–26.9]) and controls (mean 29.3 [95% CI 31.9–26.7]). However, there was a trend to a greater mean postoperative weight change (percentage) in cases (10.1%) than in controls (5.6%) (p = 0.24). Hypothalamic T2 signal change and irregular contrast enhancement correlated and predicted higher-grade hypothalamic involvement. Furthermore, they can be used to objectively grade hypothalamic involvement as the authors propose. Progressive hypothalamic involvement correlated with larger postoperative weight gains (p = 0.022); however, hypothalamic involvement did not correlate with preoperative BMI (p = 0.5).

Conclusions. Postoperative weight gain in adult patients undergoing surgery for craniopharyngioma is a significant problem and correlates with hypothalamic involvement, as it does in pediatric patients. Finally, objective MR imaging criteria can be used to predict risk of postoperative weight gain and aid in grading of hypothalamic involvement.

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Craniopharyngiomas: intratumoral chemotherapy with interferon-α: a multicenter preliminary study with 60 cases

Neurosurg Focus 28 (4):E12, 2010. (DOI: 10.3171/2010.1.FOCUS09310)

The authors assessed the efficacy of intratumoral interferon-α (IFNα)–based chemotherapy in pediatric patients with cystic craniopharyngiomas.

Methods. In a prospective multicenter study of 60 pediatric patients, the authors assessed the efficacy of intratumoral INFα2A-based chemotherapy. The study was conducted between 2000 and 2009 at 3 locations: the Medical School of the Federal University of São Paulo, Catholic University of Rome, and the Neurosurgery Institute of Santiago, Chile. The assessment included clinical and radiological control examinations, side effects observed, and total dose used.

Results. Sixty cases of cystic craniopharyngioma were analyzed. The cohort consisted of 35 male and 25 female children (mean age 11 years). Clinical and radiological improvement was achieved in 76% of the cases. New endocrinological deficits were observed in 13% of the cases. In approximately 30% of the patients, the evolution included some light side effects, the most common being headache (33%) and eyelid edema (28%). The number of cycles varied from 1 to 9 (mean 5 cycles), and the total dose applied per cycle was 36,000,000 IU.

Conclusions. This has been the largest documented series of intratumoral chemotherapy using INFα for the control of cystic craniopharyngiomas. The treatment has proved efficacious; there was no mortality, and morbidity rates were low.


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Endoscopic third ventriculostomy in patients with a diminished prepontine interval

J Neurosurg Pediatrics 5:02050–200540, 2010. DOI: 10.3171/2009.10.PEDS09187

Fenestration of the floor of the third ventricle is vital to the success of endoscopic third ventriculostomy (ETV) in treating patients with noncommunicating hydrocephalus. A generous prepontine interval (PPI) is generally accepted as one anatomical feature that may affect the safety and functionality of ETV. Whether a diminished PPI influences the safety or success of ETV, however, has not been adequately assessed.

Methods: A review was conducted on the last 100 ETV procedures performed by the first author (M.M.S.). From archived preoperative MR imaging studies, the PPI was measured between the dorsum sellae and the basilar artery. For any patient with an interval of ≤1 mm, the technical and functional success of the procedure was recorded. Technical success was defined when a surgically created fenestration was accomplished without patient morbidity. Functional success was defined as the patient not needing any additional CSF diversionary procedure within 3 months after ETV.

Results: In the entire cohort, the PPI ranged from 0 to 9.5 mm (mean 3.2 mm). There were 15 procedures performed in patients with a PPI of ≤1 mm. In all 15 procedures, a fenestration of the tuber cinereum was accomplished without vascular injury or patient morbidity. The ETV was successful in 11 patients (73.3%). All 4 failures occurred in children who had surgery during infancy (mean age 11 months).

Conclusions: Patients with an obliterated or reduced PPI can safely undergo ETV. The functional success rate appears equivalent to historical controls. Most failures in this series may be attributed to other patient characteristics, namely young age at the time of ETV.

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Endoscopic third ventriculostomy in the treatment of childhood hydrocephalus in Uganda: report of a scoring system that predicts success

Journal of Neurosurgery: Pediatrics. (DOI: 10.3171/2009.9.PEDS09196)

In Uganda, childhood hydrocephalus is common and difficult to treat. In some children, endoscopic third ventriculostomy (ETV) can be successful and avoid dependence on a shunt. This can be especially beneficial in Uganda, because of the high risk of infection and long-term failure associated with shunting. Therefore, the authors developed and validated a model to predict the chances of ETV success, taking into account the unique characteristics of a large sub-Saharan African population.

Methods. All children presenting with hydrocephalus at CURE Children’s Hospital of Uganda (CCHU) between 2001 and 2007 were offered ETV as first-line treatment and were prospectively followed up. A multivariable logistic regression model was built using ETV success at 6 months as the outcome. The model was derived on 70% of the sample (training set) and validated on the remaining 30% (validation set).

Results. Endoscopic third ventriculostomy was attempted in 1406 patients. Of these, 427 were lost to followup prior to 6 months. In the remaining 979 patients, the ETV was aborted in 281 due to poor anatomy/visibility and in 310 the ETV failed during the first 6 months. Therefore, a total of 388 of 979 (39.6% and [55.6% of completed ETVs]) procedures were successful at 6 months. The mean age at ETV was 12.6 months, and 57.8% of cases were postinfectious in origin. The authors’ logistic regression model contained the following significant variables: patient age at ETV, cause of hydrocephalus, and whether choroid plexus cauterization was performed. In the training set (676 patients) and validation set (303 patients), the model was able to accurately predict the probability of successful ETV (Hosmer-Lemeshow p value > 0.60 and C statistic > 0.70). The authors developed the simplified CCHU ETV Success Score that can be used in the field to predict the probability of ETV success.

Conclusions. The authors’ model will allow clinicians to accurately identify children with a good chance of successful outcome with ETV, taking into account the unique characteristics and circumstances of the Ugandan population.

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Endoscopic treatment of middle fossa arachnoid cysts: a series of 40 patients treated endoscopically in two centres

Childs Nerv Syst (2010) 26:163–172.DOI 10.1007/s00381-009-0952-1

Purpose Middle fossa arachnoid cysts (MFAC) are a relatively common, benign pathology that pose a therapeutic challenge for both symptomatic and asymptomatic patients. The optimal surgical strategy and indication to treat are still debated by neurosurgeons. We reviewed our experience and results in a group of patients treated with endoscopic fenestration with the aim to assess indications to treat and clinical and neuroradiological results. Methods The data on 40 patients operated with endoscopic fenestration for MFAC in two centres, “Anna Meyer” Children’sHospital, Florence, Italy, and Royal Liverpool Children’s Hospital “Alder Hey”, Liverpool, UK, between 2001 and 2007 were retrospectively reviewed with prospective followup. We analysed clinical and neuroradiological presentation, indications to treat, surgical technique, complications, and clinical and neuroradiological follow-up. Results There were 30 males and ten females: mean age, 7.8 years; mean follow-up, 21 months. The neuronavigation system was used in 12 patients in the English cohort. Thirty-seven patients (92.5%) had a satisfactory clinical outcome. The cyst was reduced in size or completely disappeared in 29 patients (72.5%). There was no death or significant morbidity associated with the procedure. Four patients required further surgical treatment. Four patients experienced a post-traumatic intracystic bleeding after surgery. Conclusion Compared to microsurgical fenestration and cyst shunting, our experience with endoscopic fenestration was as effective and safe but less invasive. Each case must be assessed with its individual characteristics to define the optimal surgical strategy. Successful treatment may not reduce the risk of post-traumatic head injury haemorrhage.


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Quality of life in obstructive hydrocephalus: endoscopic third ventriculostomy compared to cerebrospinal fluid shunt

Childs Nerv Syst (2010) 26:75–79.DOI 10.1007/s00381-009-0983-7

In the current literature, there are essentially no comparisons of quality of life (QOL) outcome after endoscopic third ventriculostomy (ETV) and shunt in childhood hydrocephalus. Our objective was to compare QOL in children with obstructive hydrocephalus, treated with either ETVor shunt. Methods A cross-sectional survey was conducted at SickKids, Toronto of children between ages five and 18 years, with obstructive hydrocephalus due to aqueductal obstruction and no other brain abnormalities. Measures of QOL were the Hydrocephalus Outcome Questionnaire and the Health Utilities Index Mark 3. A subset of patients was given the Wechsler Intelligence Scales for Children (WISC-IV). Results A total of 47 of 59 (80%) eligible patients participated (24 had ETV as primary treatment, 23 had shunt as primary treatment), with a mean age of 12.1 years (standard deviation 3.9) at assessment. The ETV group was older at initial surgery (p<0.001) and had larger ventricle size at last follow-up (p= 0.047). In all QOL measures, there were no significant differences between the ETV group and shunt group (all p≥ 0.09). Treatment failure, hydrocephalus complications, and the presence of a functioning ETV at assessment were not associated with QOL differences. Among the 11 children (six ETV, five shunt) who were given the WISC-IV, there were no significant differences between the scores of the ETV group and shunt group (all p≥0.11). Conclusions This is the first study to provide a meaningful comparison of QOL after ETV and shunt in children. These preliminary results suggest that there is no obvious difference in QOL after ETVand shunt.

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Limits of endoscopic treatment of sylvian arachnoid cysts in children

Childs Nerv Syst (2010) 26:155–162. DOI 10.1007/s00381-009-0977-5

The optimal surgical management of sylvian arachnoid cysts is debated. We present our experience in children who were treated endoscopically, focusing on the limits and complications of this approach.

Materials and methods Seventeen children with a temporosylvian arachnoid cyst have been treated using a purely temporal endoscopic approach.

Results In all but one case, the recognition of anatomical landmarks was obtained at the insertion of the endoscope. In one child, the orientation and opening of the basal cisterns were more difficult due to the thickness and opacity of the membranes. Nevertheless, it was possible to perform the cysto-cisternostomy endoscopically in all children with several stomies in 13. In one child, a venous bleeding occurred. There were no other intraoperative complications. There were no postoperative cerebrospinal fluid leaks. In two patients, a symptomatic subdural collection developed which required a transient subdural-peritoneal shunt. No subdural collection was noted on a delayed follow-up (mean, 23 months). Preoperative symptoms related to the mass effect exerted by the cyst resolved in all children. Two children presented a recurrence, 12 and 20 months after endoscopic surgery, respectively. At the redo-endoscopy, the stomies were found to be closed. Their reopening resulted in the disappearance of the symptoms.

Conclusions An endoscopic approach can be used safely in the management of sylvian arachnoid cysts. An effective opening of the deep arachnoid membranes into the basal cisterns could be performed in all patients with the resolution of the preoperative symptoms. However, the long-term efficacy of the stoma needs to be assessed.

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