External validity of the chiari severity index and outcomes among pediatric chiari I patients treated with intra- or extra-Dural decompression.

INTRODUCTION: Chiari malformation type-1 (CM-1) may be treated by intradural (ID) or extradural (ED) posterior fossa decompression, although the optimal approach is debated. The Chiari Severity Index (CSI) is a pre-operative metric to predict patient-defined improvement after CM-1 surgery. In this study, we evaluate the results of ID versus ED decompression and assess the external validity of the CSI. METHODS: We performed a retrospective cohort study of pediatric CM-1 patients undergoing decompression at a single academic children's hospital. Characteristics of headache, syrinx, and myelopathy were collected to derive CSI grade. The primary outcome measure was pre-operative symptom resolution. The proportion of patients with favorable outcome was tabulated for each of the three CSI grades and compared to previously published results. RESULTS: From 2004 to 2014, 189 patients underwent ID (48%) or ED (52%) decompression at the Children's Hospital of Philadelphia (CHOP). Follow-up ranged from 1 to 75 months. Rates of symptom resolution (58-64%) and reoperation (8%) were similar regardless of surgical approach. Although proportions of favorable outcomes differed between the CHOP and Washington University (WU) cohorts, the difference was not related to CSI grade (p = 0.63). Furthermore, there was no difference in the proportion of favorable outcomes between the two cohorts regardless of ID (p = 0.26) or ED approach (p = 0.11). CONCLUSIONS: Equivalent rates of symptom resolution and reoperation following ID and ED decompression support the ED approach as a first-line surgical option for pediatric CM-1 patients. In addition, our findings provide preliminary evidence supporting the generalizability of the CSI and its use in future comparative trials.

Myofascial Closure of Intradural Inclusion Cysts following in utero Myelomeningocele Repair.

Myelomeningocele is one of the most common congenital malformations. A randomized controlled trial, known as the Management of Myelomeningocele Study (MOMS), demonstrated that closure during the fetal period can be performed relatively safely and be of significant benefit to patients. However, postnatally, patients can develop resultant symptoms from a tethered cord and inclusion cysts; this often requires surgical treatment. Repeat surgery in this population can be challenging due to the age of the patients, the extent of surgical exposure needed and the need for resection of dermal and epidermal tissues in the midline. We describe our approach for closure of these complex defects using lateral fasciocutaneous flaps with relaxing incisions made in the posterior axillary line, in order to minimize tension and maximize soft tissue coverage of the midline.

Fetal myelomeningocele closure: technical considerations.

Myelomeningocele (MMC) is one of the most common serious congenital malformations. Typically this condition has been treated with closure of the MMC defect shortly after birth. In general, surgery for MMC aims to provide a multilayered closure to provide protection to the neural elements, prevent leakage of spinal fluid and reduce infection risks. A randomized controlled trial, the Management of Myelomeningocele Study (MOMS), has shown that closure during the fetal period can be performed relatively safely and can result in significant benefit to the child. Whereas the surgical technique of prenatal closure of an MMC defect is similar to a postnatal closure, there are some important technical differences. The goal of this paper is to describe the technique of fetal closure of MMC defects, highlight the unique steps that are needed for this surgery and delineate some potential pitfalls.

Fetal myelomeningocele repair: the post-MOMS experience at the Children's Hospital of Philadelphia.

BACKGROUND: Fetal myelomeningocele (fMMC) repair has become accepted as a standard of care option in selected circumstances. We reviewed our outcomes for fMMC repair from referral and evaluation through surgery, delivery and neonatal discharge. MATERIAL AND METHODS: All patients referred for potential fMMC repair were reviewed from January 1, 2011 through March 7, 2014. Maternal and neonatal data were collected on the 100 patients who underwent surgery. RESULTS: 29% of those evaluated met the criteria and underwent fMMC repair (100 cases). The average gestational age was 21.9 weeks at evaluation and 23.4 weeks at fMMC repair. Complications included membrane separation (22.9%), preterm premature rupture of membranes (32.3%) and preterm labor (37.5%). Average gestational age at delivery was 34.3 weeks and 54.2% delivered at ≥35 weeks. The perinatal loss rate was 6.1% (2 intrauterine fetal demises and 4 neonatal demises); 90.8% of women delivered at the Children's Hospital of Philadelphia and 3.4% received transfusions. With regard to the neonates, 2 received ventriculoperitoneal shunts prior to discharge; 71.1% of neonates had no evidence of hindbrain herniation on MRI. Of the 80 neonates evaluated, 55% were assigned a functional level of one or more better than the prenatal anatomic level. CONCLUSION: In an experienced program, maternal and neonatal outcomes for patients undergoing fMMC repair are comparable to results of the MOMS trial.

A randomized trial of prenatal versus postnatal repair of myelomeningocele.

BACKGROUND: Prenatal repair of myelomeningocele, the most common form of spina bifida, may result in better neurologic function than repair deferred until after delivery. We compared outcomes of in utero repair with standard postnatal repair. METHODS: We randomly assigned eligible women to undergo either prenatal surgery before 26 weeks of gestation or standard postnatal repair. One primary outcome was a composite of fetal or neonatal death or the need for placement of a cerebrospinal fluid shunt by the age of 12 months. Another primary outcome at 30 months was a composite of mental development and motor function. RESULTS: The trial was stopped for efficacy of prenatal surgery after the recruitment of 183 of a planned 200 patients. This report is based on results in 158 patients whose children were evaluated at 12 months. The first primary outcome occurred in 68% of the infants in the prenatal-surgery group and in 98% of those in the postnatal-surgery group (relative risk, 0.70; 97.7% confidence interval [CI], 0.58 to 0.84; P<0.001). Actual rates of shunt placement were 40% in the prenatal-surgery group and 82% in the postnatal-surgery group (relative risk, 0.48; 97.7% CI, 0.36 to 0.64; P<0.001). Prenatal surgery also resulted in improvement in the composite score for mental development and motor function at 30 months (P=0.007) and in improvement in several secondary outcomes, including hindbrain herniation by 12 months and ambulation by 30 months. However, prenatal surgery was associated with an increased risk of preterm delivery and uterine dehiscence at delivery. CONCLUSIONS: Prenatal surgery for myelomeningocele reduced the need for shunting and improved motor outcomes at 30 months but was associated with maternal and fetal risks. (Funded by the National Institutes of Health; ClinicalTrials.gov number, NCT00060606.).

Direct cardiac ventriculoatrial shunt: technical note.

There are a number of choices for placement of the distal catheter during ventricular shunting for hydrocephalic patients. In very rare instances, patients with multiple revisions can no longer have their shunt placed in the routine locations. We describe the placement of the distal catheter into the atrium through direct cardiac access, a technique described decades ago but rarely needed in clinical practice. This can be a useful location in the limited number of patients who have exhausted other more routine locations.

The role of concurrent fusion to prevent spinal deformity after intramedullary spinal cord tumor excision in children.

OBJECTIVES: Spinal deformity is a common development after laminectomy and resection of pediatric intramedullary spinal cord tumors. Our objective is to compare the occurrence of postlaminectomy spinal deformity in children with intramedullary spinal cord tumors that underwent decompression with fusion at the time of surgery to those that did not undergo fusion. METHODS: A retrospective chart review of 255 children with spinal cord tumors treated at 2 tertiary pediatric cancer centers between was performed. Of these, 52 patients with a biopsy-proven intramedullary spinal cord tumor had complete clinical records and radiographic data. Preoperative spinal alignment, surgical treatment, postoperative deformity, and risk factors for deformity were evaluated. All patients had at least 2-year follow-up. RESULTS: There were 18 females and 34 males with an average age of 8.1 ± 4.1 years. The average time to latest follow-up was 7.6 ± 5.3 years. Moderate or severe postresection spinal deformity (scoliosis > 25 degrees and/or sagittal plane abnormality > 20 degrees requiring bracing or surgery) developed in 57% (21/37) of resections without fusion (laminectomy or laminoplasty alone), and in 27% (4/15) of those with fusion (P = 0.05). Among skeletally mature children, 18 of 28 (64%) developed deformity after laminectomies and laminoplasties, compared with 22% (2/9) of the patients in the fusion group (P = 0.03). Removal of >3 lamina (P = 0.04) was associated with development of postoperative deformity. CONCLUSIONS: In the surgical treatment of patients with intramedullary spinal cord tumors, those that undergo instrumentation or in situ fusion at the time of spinal cord tumor excision are significantly less likely to develop postresection spinal deformity. LEVEL OF EVIDENCE: 3, Retrospective comparative study.

Energy expenditure in obesity associated with craniopharyngioma.

BACKGROUND AND PURPOSE: Obesity is a common yet incompletely understood complication of childhood craniopharyngioma. We hypothesized that craniopharyngioma is associated with specific defects in energy balance compared to obese control children. METHODS: Eleven craniopharyngioma patients were recruited for a study on body composition and energy balance. Eight subjects were obese. The obese craniopharyngioma patients had a mean age (+/-SD) of 11.2 +/- 1.7 years. The average body mass index z score was 2.33 (+/-0.32). A previously studied group of obese children (BMI z score 2.46 +/- 0.46) served as controls. Resting energy expenditure (REE) was determined by indirect calorimetry and body composition by dual energy X-ray absorptiometry in all children. RESULTS: Obese craniopharyngioma patient subjects had increased mean (+/-standard error) fat-free mass compared to obese controls (57% +/- 0.88 % vs 50.0% +/- 0.87%, p = 0.02). The obese craniopharyngioma patients had a 17% lower REE compared to values expected from the World Health Organization equation (1,541 +/- 112.6 vs 1,809 +/- 151.8 kcal; p = 0.01). In contrast, the obese control children had measured REE within 1% of predicted (1,647 +/- 33.2 vs. 1,652 +/- 40.2; p = 0.8). In a linear regression model, REE remained significantly lower than predicted after controlling for FFM. CONCLUSIONS: Lower REE may be a factor contributing to obesity in children with craniopharyngioma. Further study is needed into the mechanisms for reduced energy expenditure in patients with craniopharyngioma.

Lower extremity neuromotor function and short-term ambulatory potential following in utero myelomeningocele surgery.

OBJECTIVE: To evaluate lower extremity neuromotor function (LENF) and short-term ambulatory potential following fetal myelomeningocele (fMMC) closure. METHODS: Retrospective chart review of 54 children that underwent fMMC closure at our institution prior to the NIHCD-MOMS trial. Neonatal LENF was compared to predicted function based on spinal lesion level assigned by prenatal ultrasound. Ambulatory status was classified as independent walkers (walks without assistive appliances), assisted walker (requires walking aid), and non-ambulatory (wheelchair bound). RESULTS: Thoracic, lumbar, and sacral level lesions were present in 4, 44 and 6 fMMC infants, respectively. 31/54 of fMMC children (57.4%; median: 2 levels, range: 1-5) had better than predicted, 13/54 (24.1%) same as predicted and 10/54 (18.5%; median: 1 level, range: 1-2) worse than predicted LENF at birth. At a median follow-up age of 66 months (36-113), 37/54 (69%) walk independently, 13/54 (24%) are assisted walkers, and 4/54 (7%) are wheelchair dependent. The strongest factors predicting a lower likelihood to walk independently were higher-level lesion (>L4, p = 0.001) and the development of clubfoot deformity after fetal intervention (p = 0.026). Despite the observed improved ambulatory status, structured evaluation of coordinative skills revealed that the majority of independent ambulators and all children that require assistive devices to walk experience significant deficits in lower extremity coordination. CONCLUSIONS: We observed that fMMC surgery in this highly selective population results in better than predicted LENF at birth and short-term ambulatory status. However, fMMC toddlers continue to demonstrate deficits in movement coordination that are characteristic for children with spina bifida.

Fetal surgery for neural tube defects.

Open spina bifida remains a major source of disability despite an overall decrease in incidence. It is frequently diagnosed prenatally and can thus - potentially - be treated by fetal surgery. Animal studies and preliminary human studies strongly suggest that at least a portion of the neurological abnormalities seen in these patients are secondary, and occur in mid-gestation. It is estimated that approximately 400 fetal operations have now been performed for myelomeningocele world wide. Despite this large experience, the technique remains of unproven benefit. Preliminary results suggest that fetal surgery results in reversal of hindbrain herniation (the Chiari II malformation), a decrease in shunt-dependent hydrocephalus, and possibly improvement in leg function, but these findings might be explained by selection bias and changing management indications. A randomized prospective trial (the MOMS trial) is currently being conducted by three centers in the United States, and is estimated to be completed in 2009.

Does a myelomeningocele sac compared to no sac result in decreased postnatal leg function following maternal fetal surgery for spina bifida aperta?

OBJECTIVE: A fetus with large sac S1 myelomeningocele (MMC) but bilateral talipes prompted the question, 'Does the presence or size of an MMC sac affect postnatal leg function?' STUDY DESIGN: An MMC database with prenatal, birth, and a minimum of 1-year follow-up evaluation was reviewed. All fetuses had in-utero MMC repair at 20 + 0 to 25 + 6 weeks at a single institution. Fifty-four fetuses had prenatal evaluation, with 48 children completing a birth and a 1-year evaluation of leg function. RESULTS: An MMC sac was present in 38/54 (70%) of fetuses evaluated in-utero and had been present in 35/48 (73%) of children evaluated at 1 year of age. Although leg function evaluated at 1 year was better than expected in the 'no sac' group (p = 0.059), this did not reach statistical significance. CONCLUSION: The presence of an MMC sac may increase postnatal lower limb morbidity.

Anticancer effects of fenretinide in human medulloblastoma.

N-(4-hydroxyphenyl) retinamide (4-HPR, fenretinide) a synthetic retinoid is in clinical trials for the treatment of several malignancies. However, its biological effects and therapeutic value in childhood brain tumor medulloblastoma (MB) has not been investigated. In this study, we report for the first time that fenretinide (2.5-10 microM) induces apoptotic cell death in human MB cells. We observed significant inhibition of cell survival in four MB cell lines (D425MED, D458MED, D283MED and D341MED) as determined by MTT assays. These results were further supported by inhibition of anchorage-independent colony formation in soft agar. Fenretinide-induced decrease in cell viability was in part due to activation of caspase-3 dependent cell death, which was further supported by the cleavage of poly(ADP-ribose) polymerase-1 (PARP-1), a caspase-3 substrate. Cell death was partially prevented by the antioxidant, l-ascorbic acid suggesting that free radical intermediates might be involved in fenretinide effects. These results suggest that pharmacologically achievable concentrations of fenretinide are effective in killing MB cells and thus show its therapeutic potential to treat human MB.

Maternal-fetal surgery for myelomeningocele: neurodevelopmental outcomes at 2 years of age.

OBJECTIVE: This study was undertaken to examine short-term neurodevelopmental outcomes in children with myelomeningocele (MMC) who underwent in utero neurosurgical closure. STUDY DESIGN: Between 1998 and 2002, 51 fetuses underwent in utero MMC closure at our Center. Thirty (63%) of these children have returned for neurodevelopmental testing at 2 years of age using the Bayley Scales of Infant Development and Preschool Language Scales. RESULTS: Overall shunt rate was 43% in this group. Neurodevelopmental testing found 67% with cognitive language and personal-social skills in the normal range, 20% with mild delays, and 13% with significant delays. Children with shunted hydrocephalus scored lower than those with unshunted ventriculomegaly. CONCLUSION: Children who have undergone fetal MMC closure have characteristic neurodevelopmental deficits that do not appear worsened by fetal surgery, and developmental outcomes may be improved by decreasing the need for ventriculoperitoneal shunting.

Brain tissue oxygen monitoring in pediatric patients with severe traumatic brain injury.

OBJECT: Intracranial pressure (ICP) and cerebral perfusion pressure (CPP) monitoring are fundamental to the management of severe traumatic brain injury (TBI). In adults, brain tissue oxygen monitoring (specifically PO2) and treatment have been shown to be safe additions to conventional neurocritical care and are associated with improved outcome. Brain tissue oxygen monitoring, however, has not been described in pediatric patients with TBI. In this report, the authors present preliminary experience with the use of ICP and PO2 monitoring in this population. METHODS: Pediatric patients (age <18 years) with severe TBI (Glasgow Coma Scale score <8) admitted to a Level 1 trauma center who underwent ICP and PO2 monitoring were evaluated. Therapy was directed at maintaining ICP below 20 mm Hg and age-appropriate CPP (> or =40 mm Hg). Data obtained in six patients (two girls and four boys ranging in age from 6-16 years) were analyzed. Brain tissue oxygen levels were significantly higher (p < 0.01) at an ICP of less than 20 mm Hg (PO2 29.29 +/- 7.17 mm Hg) than at an ICP of greater than or equal to 20 mm Hg (PO2 22.83 +/- 13.85 mm Hg). Significant differences (p < 0.01) were also measured when CPP was less than 40 mm Hg (PO2 2.53 +/- 7.98 mm Hg) and greater than or equal to 40 mm Hg (PO2 28.97 +/- 7.85 mm Hg). CONCLUSIONS: Brain tissue oxygen monitoring may be a safe and useful addition to ICP monitoring in the treatment of pediatric patients with severe TBI.

Spina bifida.

Spina bifida results from failure of fusion of the caudal neural tube, and is one of the most common malformations of human structure. The causes of this disorder are heterogeneous and include chromosome abnormalities, single gene disorders, and teratogenic exposures. However, the cause is not known in most cases. Up to 70% of spina bifida cases can be prevented by maternal, periconceptional folic acid supplementation. The mechanism underlying this protective effect is unknown, but it is likely to include genes that regulate folate transport and metabolism. Individuals with spina bifida need both surgical and medical management. Although surgical closure of the malformation is generally done in the neonatal period, a randomised clinical trial to assess in utero closure of spina bifida has been initiated in the USA. Medical management is a lifelong necessity for individuals with spina bifida, and should be provided by a multidisciplinary team.

All-trans-retinoic acid-induced apoptosis in human medulloblastoma: activation of caspase-3/poly(ADP-ribose) polymerase 1 pathway.

Current treatments for childhood brain tumor medulloblastoma (MB), radiation and chemotherapy, lead to undesirable side effects. Identification of antitumor agents that reduce the toxicity will thus have significant therapeutic value. In this study, we investigated all-trans-retinoic acid (ATRA) as an antitumor agent. Although high concentrations (1-10 microM) of retinoic acid derivatives are generally needed for significant antitumor effects in many cancer cells, we observed that pharmacologically relevant concentrations of ATRA were effective in inducing cell death in human MB cells. Using 10-fold lower concentrations (100-500 nM), we found that ATRA inhibits MB (DAOY, D283, D425, and D458) cell proliferation as determined by cell viability [3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide] and bromodeoxyuridine incorporation assays. Furthermore, 100 nM ATRA was potent in inhibiting the anchorage-independent growth of the sensitive cell lines (D283, D425, and D458) in soft agar assays. We also demonstrate that the ATRA-induced decrease in cell viability was due to increased cell death by apoptosis, which was accompanied by a 20-fold induction of caspase-3 activity in the most sensitive cell line, D458. By contrast, induction of caspase-3 was only 2-fold in the relatively insensitive DAOY cells. Furthermore, ATRA-induced cell death in D283, D425, and D458 cells was accompanied by activation of caspase-3, a key executioner of apoptosis. We also demonstrate that activated caspase-3 resulted in cleavage of 116-kDa poly(ADP-ribose) polymerase 1 to its signature fragments (85 and 29 kDa). Pretreatment with a specific caspase-3 inhibitor, DEVD-CHO, significantly reduced ATRA-induced apoptotic cell death. Thus, we demonstrate for the first time that low concentrations of ATRA inhibit MB cell proliferation and induce apoptotic cell death in part by activating caspase-3/poly(ADP-ribose) polymerase 1 effector pathway, and we show that retinoic acids and novel retinoids are potential antitumor agents in MB therapy.

Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement.

OBJECT: The Management of Myelomeningocele Study (MOMS) was a multicenter randomized trial comparing the safety and efficacy of prenatal and postnatal closure of myelomeningocele. The trial was stopped early because of the demonstrated efficacy of prenatal surgery, and outcomes on 158 of 183 pregnancies were reported. Here, the authors update the 1-year outcomes for the complete trial, analyze the primary and related outcomes, and evaluate whether specific prerandomization risk factors are associated with prenatal surgery benefit. METHODS: The primary outcome was a composite of fetal loss or any of the following: infant death, CSF shunt placement, or meeting the prespecified criteria for shunt placement. Primary outcome, actual shunt placement, and shunt revision rates for prenatal versus postnatal repair were compared. The shunt criteria were reassessed to determine which were most concordant with practice, and a new composite outcome was created from the primary outcome by replacing the original criteria for CSF shunt placement with the revised criteria. The authors used logistic regression to estimate whether there were interactions between the type of surgery and known prenatal risk factors (lesion level, gestational age, degree of hindbrain herniation, and ventricle size) for shunt placement, and to determine which factors were associated with shunting among those infants who underwent prenatal surgery. RESULTS: Ninety-one women were randomized to prenatal surgery and 92 to postnatal repair. The primary outcome occurred in 73% of infants in the prenatal surgery group and in 98% in the postnatal group (p < 0.0001). Actual rates of shunt placement were only 44% and 84% in the 2 groups, respectively (p < 0.0001). The authors revised the most commonly met criterion to require overt clinical signs of increased intracranial pressure, defined as split sutures, bulging fontanelle, or sunsetting eyes, in addition to increasing head circumference or hydrocephalus. Using these modified criteria, only 3 patients in each group met criteria but did not receive a shunt. For the revised composite outcome, there was a difference between the prenatal and postnatal surgery groups: 49.5% versus 87.0% (p < 0.0001). There was also a significant reduction in the number of children who had a shunt placed and then required a revision by 1 year of age in the prenatal group (15.4% vs 40.2%, relative risk 0.38 [95% CI 0.22-0.66]). In the prenatal surgery group, 20% of those with ventricle size < 10 mm at initial screening, 45.2% with ventricle size of 10 up to 15 mm, and 79.0% with ventricle size ≥ 15 mm received a shunt, whereas in the postnatal group, 79.4%, 86.0%, and 87.5%, respectively, received a shunt (p = 0.02). Lesion level and degree of hindbrain herniation appeared to have no effect on the eventual need for shunting (p = 0.19 and p = 0.13, respectively). Similar results were obtained for the revised outcome. CONCLUSIONS: Larger ventricles at initial screening are associated with an increased need for shunting among those undergoing fetal surgery for myelomeningocele. During prenatal counseling, care should be exercised in recommending prenatal surgery when the ventricles are 15 mm or larger because prenatal surgery does not appear to improve outcome in this group. The revised criteria may be useful as guidelines for treating hydrocephalus in this group.

Craniospinal radiation in the treatment of biopsy-proven intracranial germinomas: twenty-five years' experience in a single center.

PURPOSE: The optimal treatment for intracranial germinomas remains controversial. We report on our 25-year experience using craniospinal irradiation (CSI) for this disease. METHODS AND MATERIALS: Between September 1976 and May 2001, 39 patients with biopsy-proven intracranial germinomas seen at the Children's Hospital of Philadelphia/Hospital of the University of Pennsylvania received CSI. Thirteen of 36 patients (36%) had evidence of spinal dissemination. Median doses to the whole brain, primary site, and spine were 36 Gy (range, 18-44.2 Gy), 50.4 Gy (range, 44-55.8 Gy), and 30.6 Gy (range, 18-40 Gy), respectively. RESULTS: With a median follow-up of 7.1 years (range: 1.5-20.2 years), there have been no documented relapses. This includes 5 patients without spinal dissemination who received 18-19.8 Gy to the craniospinal axis; for these patients, the median length of follow-up was 5.5 years (range, 1.3-6.8 years). One patient, who had no evidence of disease 12.9 years after CSI, died of unknown causes 4 months later. CONCLUSIONS: Our treatment of intracranial germinomas with CSI has yielded outstanding results with no known relapses during a long follow-up period. These results must be considered when evaluating other approaches, such as chemotherapy only or local field irradiation.

Surgery with or without radiation therapy in the management of craniopharyngiomas in children and young adults.

PURPOSE: The optimal management of craniopharyngiomas remains controversial, especially in children and young adults. This study reports a single institution's experience with such patients. METHODS AND MATERIALS: Between 1974 and 2001, 76 patients were treated for craniopharyngioma at the Children's Hospital of Philadelphia and the Hospital of University of Pennsylvania (HUP). Of these, 75 patients (97%) were evaluable with long-term follow-up. Although all patients underwent attempted gross total resection, 27 had documentation of less than total resection with 18 of these patients receiving immediate postoperative radiotherapy (RT). An additional 22 patients received RT at HUP after failing surgery alone. RESULTS: Median follow-up for all patients was 7.6 years. The 10-year actuarial overall survival, relapse-free survival, and local control (LC) rates for all patients were 85%, 48%, and 53%, respectively. When comparing the 57 patients treated with surgery alone to the 18 treated with subtotal resection (STR) followed by RT, a significant difference in LC rates at 10 years (42% vs. 84%, respectively; p = 0.004) was noted. However, no statistically significant difference in overall survival was found between the two groups, because RT was highly effective as salvage therapy. Twenty-two patients at HUP treated with RT after relapse had a 10-year ultimate LC rate comparable to that of patients who received RT immediately after STR. CONCLUSION: RT given either immediately after STR or at relapse is effective in controlling craniopharyngiomas.