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Philipp R. Aldana and Paul Steinbok

Object

Pediatricians play a vital role in the diagnosis and initial treatment of children with pediatric neurosurgical disease. Exposure of pediatrics residents to neurosurgical diseases during training is inconsistent and is usually quite limited. After residency, opportunities for pediatricians' education on neurosurgical topics are few and fall mainly on pediatric neurosurgeons. The American Association of Neurological Surgery/Congress of Neurological Surgeons Joint Section on Pediatric Neurological Surgery Committee on Education undertook a survey of practicing pediatric neurosurgeons to determine whether focused education of practicing pediatricians might lead to better patient outcomes for children with a sampling of common pediatric neurosurgical conditions.

Methods

An Internet-based 40-item survey was administered to practicing pediatric neurosurgeons from the US and Canada identified from the roster of the American Association of Neurological Surgeons/Congress of Neurological Surgeons Joint Section of Pediatric Neurological Surgery. Survey topics included craniosynostosis and plagiocephaly, occult spinal dysraphism and tethered cord, hydrocephalus and endoscopic third ventriculostomy, Chiari malformation Type I, mild or minor head injury, spastic cerebral palsy, and brain tumors. Most questions pertained to diagnosis, initial medical treatment, and referral.

Results

One hundred three (38%) of the 273 practicing pediatric neurosurgeons completed the survey. Two-thirds of the respondents had completed a pediatric neurosurgery fellowship, and two-thirds were in academic practice. Eighty-two percent of the respondents agreed that the care of pediatric neurosurgical patients could be improved with further education of pediatricians. In the opinion of the respondents, the 3 disease topics in greatest need of educational effort were craniosynostosis and plagiocephaly, occult spinal dysraphism and tethered cord, and hydrocephalus. Head injury and spasticity were given the lowest priorities.

Conclusions

This survey identified what practicing pediatric neurosurgeons perceive to be the most important knowledge deficits of their colleagues in pediatrics. These perceptions may not necessarily be congruent with the perceptions of practicing pediatricians themselves; nevertheless, the data from this survey may serve to inform conversations between neurosurgeons and planners of continuing medical education for pediatricians, pediatrics residency program directors, and medical school pediatrics faculty.

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Philipp R. Aldana, Hector E. James and Richard A. Postlethwait

Object

The authors report a clinical protocol for the application of ventriculogallbladder (VGB) shunts in children who may be unable to maintain or receive ventriculoperitoneal (VP) shunts.

Methods

Eighteen patients underwent placement of VGB shunts as an alternative to VP shunt therapy for the following reasons: malfunction of the VP shunt due to suspected failure of the peritoneum to absorb cerebrospinal fluid (17 cases) and multiple intraabdominal general surgical procedures (1 case). The patients ranged in age from 4 months to 17 years (mean 6.5 ± 6.1 years [standard deviation {SD}]). All patients underwent preoperative imaging of the gall-bladder either by ultrasonography or computed tomography scanning. A team consisting of a pediatric neurological surgeon and a pediatric general surgeon performed all operative procedures. The procedures were conducted by open laparotomy to precisely place the appropriate length of distal catheter and to anchor it to the gallbladder wall.

Results

There were 2 early shunt malfunctions, both obstructions due to “sludge” (1 in the biliary duct and 1 in the common bile duct). A late-onset (5-year) malfunction occurred secondary to gallbladder stones. In all 3 cases of malfunction, the devices were successfully converted to VP shunts. In 1 patient a conversion to a VP shunt was chosen following a general surgical intervention. There were 2 shunt infections (Staphylococcus epidermidis and Haemophilus influenzae). These were successfully treated. Two patients underwent conversion to a VGB shunt on 2 occasions. Thirteen patients had functional VGB shunts at the time of their last follow-up assessment. The follow-up for these 13 patients ranged from 1 to 8 years (mean 2.1 ± 2.0 years [SD]).

Conclusions

Ventriculogallbladder shunts may be considered for the treatment of hydrocephalus in children when the peritoneal cavity cannot be used as a distal terminus.

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Editorial

Neurosurgical education for pediatricians

Joseph H. Piatt

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Carl A. Youssef, Carmen R. Smotherman, Dale F. Kraemer and Philipp R. Aldana

OBJECT

The endoscopic endonasal approach (EEA) has been established as an alternative approach to craniovertebral junction (CVJ) pathology in adults. The authors have previously described the nasoaxial line (NAxL) as an accurate predictor of the lower limit of the EEA to the CVJ in adults. The surgical anatomy limiting the EEA to the pediatric CVJ has not been well studied. Furthermore, predicting the lower limit of the EEA in various pediatric age groups is important in surgical planning. To better understand the anatomy affecting the EEA to the CVJ, the authors examined the skull base anatomy relevant to the EEA in children of different age groups and used the NAxL to predict the EEA lower limit in children.

METHODS

Axial brain CT scans of 39 children with normal skull base anatomy were reconstructed sagittally. Children were divided into 4 groups according to age: 3–6, 7–10, 11–14, and 15–18 years old. The intersection of the NAxL with the odontoid process of C-2 was described for each group. Analyses of variance were used to estimate the effect of age, sex, interaction between age and sex on different anatomical parameters relevant to the endonasal corridor (including the length of the hard palate [HPLe]), dimensions of choana and piriform aperture, and the length of the NAxL to C-2. The effect of the HPLe on the working distance of NAxL to the odontoid was also estimated using analysis of covariance, controlling for age, sex, and their interaction.

RESULTS

The NAxL extended to the odontoid process in 38 of the 39 children. Among the 39 children, the NAxL intersected the upper third of the odontoid process in 25 while intersecting the middle third in the remaining 13 children. The measurements of the inferior limits did not differ with age, varying between 9 and 11 mm below the hard palate line at the ventral surface of C-2. Significant increases in the size of the piriform aperture and choana and the HPLe were observed after age 10. The HPLe predicted the length of the NAxL (p < 0.0001).

CONCLUSIONS

The caudal limit of the EEA extends as far as the middle third of the odontoid process in children, as predicted by the NAxL. The most prominent increase in the size of the choana and piriform aperture occurs after age 10. The HPLe is a significant predictor of the working distance to C-2. Utilizing the NAxL preoperatively may help in planning the EEA to the CVJ in children.

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Amer Dastgir, Nathan J. Ranalli, Theresa L. MacGregor and Philipp R. Aldana

The authors report an unusual case of intrathecal baclofen withdrawal due to the perforation and subsequent leakage of a baclofen pump catheter in a patient with spastic cerebral palsy. A 15-year-old boy underwent an uncomplicated placement of an intrathecal baclofen pump for the treatment of spasticity due to cerebral palsy. After excellent control of symptoms for 3 years, the patient presented to the emergency department with increasing tremors following a refill of his baclofen pump. Initial evaluation consisted of radiographs of the pump and catheter, which appeared normal, and a successful aspiration of CSF from the pump’s side port. A CT dye study revealed a portion of the catheter directly overlying the refill port and extravasation of radiopaque dye into the subfascial pocket anterior to the pump. During subsequent revision surgery, a small puncture hole in the catheter was seen to be leaking the drug. The likely cause of the puncture was an inadvertent perforation of the catheter by a needle during the refilling of the pump. This case report highlights a unique complication in a patient with an intrathecal baclofen pump. Physicians caring for these patients should be aware of this rare yet potential complication in patients presenting with baclofen withdrawal symptoms.

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Hector E. James, Anthony A. Perszyk, Teresa L. MacGregor and Philipp R. Aldana

OBJECT

The cranium is documented to grow from birth through adolescence. The standard of practice in primary care is measuring head circumference and plotting growth using curves that stop at 36 months. The authors report the importance of their experience with measuring head circumference in the child and same-sex parent beyond 36 months.

METHODS

In the University of Florida genetics and pediatric neurosurgery clinics, head circumference is measured and plotted on growth charts through 18 years of age. Circumference and rate of growth over time are compared with those of the same-sex parent. A diagnostic workup is initiated if there is a discrepancy with the patient's head circumference or if there is significant change in the growth rate of the cranium.

RESULTS

Between January 2004 and December 2007, the lead author examined 190 patients referred by pediatricians and/or pediatric subspecialists because of the concerns regarding head size of the child. Neuroimaging was performed in 70% of the patients prior to referral. None of the patients had their head size compared with that of their same-sex parent prior to referral. On assessing referring physician responses as to why the same-sex parents, head measurements were not pursued prior to imaging or referral to the specialists, the results were: 1) only have head circumference sheets to 36 months of age (n = 28); 2) the American Academy of Pediatrics does not recommend it (n = 3); and 3) the head stops growing at 36 months of age (n = 2).

CONCLUSIONS

Pediatricians and pediatric subspecialists need instruction on head circumference measurement in children from infancy through adolescence, and when indicated, in comparison with the head size of the same-sex parent. This measurement may be an effective and inexpensive assessment tool.

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Antonio M. Aguilera, David L. Wood, Cortney Keeley, Hector E. James and Philipp R. Aldana

OBJECT

The transition of the young adult with spina bifida (YASB) from pediatric to adult health care is considered a priority by organized pediatrics. There is a paucity of transition programs and related studies. Jacksonville Health and Transition Services (JaxHATS) is one such transition program in Jacksonville, Florida. This study’s purpose was to evaluate the health care access, utilization, and quality of life (QOL) of a group of YASBs who have transitioned from pediatric care.

METHODS

A survey tool addressing access to health care and quality of health and life was developed based on an established survey. Records of the Spinal Defects Clinic held at Wolfson Children’s Hospital and JaxHATS Clinic were reviewed and YASBs (> 18 and < 30 years old) were identified.

RESULTS

Ten of the 12 invited YASBs in the Jacksonville area completed the surveys. The mean age of respondents was 25.1 years. All reported regular medical home visits, 8 with JaxHATS and 2 with other family care groups. All reported easy access to medical care and routine visits to spina bifida (SB) specialists; none reported difficulty or delays in obtaining health care. Only 2 patients required emergent care in the last year for an SB-related medical problem. Seven respondents reported very good to excellent QOL. Family, lifestyle, and environmental factors were also examined.

CONCLUSIONS

In this small group of YASBs with a medical home, easy access to care for medical conditions was the norm, with few individuals having recent emergency visits and almost all reporting at least a good overall QOL. Larger studies of YASBs are needed to evaluate the positive effects of medical homes on health and QOL in this population.

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Philipp R. Aldana, Saswata Roy, Richard A. Postlethwait and Hector E. James

Object

Bioresorbable implant systems have been used in neurosurgery for the rigid fixation of cranial and facial bones. A relatively recent advancement has been the fixation of these implants using an ultrasonic device. The experience with such a device in neurosurgical practice has been limited. The authors report on their experience with ultrasound-aided fixation of bioresorbable implants in pediatric neurosurgical practice.

Methods

The study consisted of 2 parts. The retrospective portion consisted of a chart review of pertinent clinical information, complications, and outcomes after the use of a commercially available ultrasound-aided bioresorbable implant system (SonicWeld Rx, KLS Martin L.P.). Follow-up was obtained in all patients via clinical examination or telephone interview. The prospective portion of the study consisted of video analysis of the implantation technique in a routine craniotomy. Implantation times were measured, and delays during treatment were noted.

Results

Over a period of 2 years, 28 consecutive patients underwent placement of these implants for bone fixation during craniotomies or craniofacial reconstructions. The only complication was seen in a child with Crouzon syndrome, who had a wound infection caused by Serratia sepsis from a central venous line infection. There were no repeated operations for implant-related swelling, and no cases of premature plate resorption, bone instability, or settling. In vivo, the average time required to implant a resorbable pin with this system was 22 seconds.

Conclusions

The use of a bioresorbable implant system with ultrasound-aided pin fixation in pediatric neurosurgery cases achieved adequate stability with few complications. This system was easy to use and provided rapid fixation of implants.

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Alexandra D. Beier, Gemi E. Jannotta, E. Dayan Sandler, Harry S. Abram, Raj D. Sheth and Philipp R. Aldana

Hemiconvulsion-hemiplegia-epilepsy (HHE) is an uncommon epileptic syndrome that affects young children. Typical management includes early initiation of benzodiazepines to abate the initial seizure activity quickly. Patients in whom epilepsy develops require prolonged use of antiepileptic agents. Herniation due to diffuse cerebral edema from HHE is rare; however, decompressive craniectomy has been described as a lifesaving measure. The authors present the case of a patient in whom a decompressive craniectomy was performed. They advocate a proactive approach in the detection and management of cerebral edema in HHE causing intracranial hypertension. In HHE cases that exhibit radiographic evidence of malignant cerebral edema (although not previously described in this disease, but similar to the setting of stroke and trauma), the authors advocate early neurosurgical consultation and evaluation for insertion of an intracranial pressure monitor for those patients who do not have a reliable neurological examination (i.e., Glasgow Coma Scale score ≤ 8).

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Nicole Prendergast, Philipp R. Aldana, Ronny L. Rotondo, Lournaris Torres-Santiago and Alexandra D. Beier

Tumors involving the sella are commonly craniopharyngiomas, optic pathway gliomas, or pituitary adenomas. Functioning adenomas are expected, with prolactinomas topping the differential. The authors present the case of a silent corticotroph adenoma, which has not been described in the pediatric population, and they detail the use of proton therapy, which is also novel.