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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.

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Hannah E. Goldstein, Justin A. Neira, Matei Banu, Philipp R. Aldana, Bruno P. Braga, Douglas L. Brockmeyer, Michael L. DiLuna, Daniel H. Fulkerson, Todd C. Hankinson, Andrew H. Jea, Sean M. Lew, David D. Limbrick, Jonathan Martin, Joshua M. Pahys, Luis F. Rodriguez, Curtis J. Rozzelle, Gerald F. Tuite, Nicholas M. Wetjen and Richard C. E. Anderson

OBJECTIVE

The long-term effects of surgical fusion on the growing subaxial cervical spine are largely unknown. Recent cross-sectional studies have demonstrated that there is continued growth of the cervical spine through the teenage years. The purpose of this multicenter study was to determine the effects of rigid instrumentation and fusion on the growing subaxial cervical spine by investigating vertical growth, cervical alignment, cervical curvature, and adjacent-segment instability over time.

METHODS

A total of 15 centers participated in this multi-institutional retrospective study. Cases involving children less than 16 years of age who underwent rigid instrumentation and fusion of the subaxial cervical spine (C-2 and T-1 inclusive) with at least 1 year of clinical and radiographic follow-up were investigated. Charts were reviewed for clinical data. Postoperative and most recent radiographs, CT, and MR images were used to measure vertical growth and assess alignment and stability.

RESULTS

Eighty-one patients were included in the study, with a mean follow-up of 33 months. Ninety-five percent of patients had complete clinical resolution or significant improvement in symptoms. Postoperative cervical kyphosis was seen in only 4 patients (5%), and none developed a swan-neck deformity, unintended adjacent-level fusion, or instability. Of patients with at least 2 years of follow-up, 62% demonstrated growth across the fusion construct. On average, vertical growth was 79% (4-level constructs), 83% (3-level constructs), or 100% (2-level constructs) of expected growth. When comparing the group with continued vertical growth to the one without growth, there were no statistically significant differences in terms of age, sex, underlying etiology, surgical approach, or number of levels fused.

CONCLUSIONS

Continued vertical growth of the subaxial spine occurs in nearly two-thirds of children after rigid instrumentation and fusion of the subaxial spine. Failure of continued vertical growth is not associated with the patient’s age, sex, underlying etiology, number of levels fused, or surgical approach. Further studies are needed to understand this dichotomy and determine the long-term biomechanical effects of surgery on the growing pediatric cervical spine.

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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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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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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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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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Emanuele La Corte, Philipp R. Aldana, Paolo Ferroli, Jeffrey P. Greenfield, Roger Härtl, Vijay K. Anand and Theodore H. Schwartz

OBJECT

The endoscopic endonasal approach (EEA) provides a minimally invasive corridor through which the cervicomedullary junction can be decompressed with reduced morbidity rates compared to those with the classic transoral approaches. The limit of the EEA is its inferior extent, and preoperative estimation of its reach is vital for determining its suitability. The aim of this study was to evaluate the actual inferior limit of the EEA in a surgical series of patients and develop an accurate and reliable predictor that can be used in planning endonasal odontoidectomies.

METHODS

The actual inferior extent of surgery was determined in a series of 6 patients with adequate preoperative and postoperative imaging who underwent endoscopie endonasal odontoidectomy. The medians of the differences between several previously described predictive lines, namely the nasopalatine line (NPL) and nasoaxial line (NAxL), were compared with the actual surgical limit and the hard-palate line by using nonparametric statistics. A novel line, called the rhinopalatine line (RPL), was established and corresponded best with the actual limit of the surgery.

RESULTS

There were 4 adult and 2 pediatric patients included in this study. The NPL overestimated the inferior extent of the surgery by an average (± SD) of 21.9 ± 8.1 mm (range 14.7-32.5 mm). The NAxL and RPL overestimated the inferior limit of surgery by averages of 6.9 ± 3.8 mm (range 3.7-13.3 mm) and 1.7 ± 3.7 mm (range −2.8 to 8.3 mm), respectively. The medians of the differences between the NPL and NAxL and the actual surgery were statistically different (both p = 0.0313). In contrast, there was no statistically significant difference between the RPL and the inferior limit of surgery (p = 0.4375).

CONCLUSIONS

The RPL predicted the inferior limit of the EEA to the craniovertebral junction more accurately than previously described lines. The use of the RPL may help surgeons in choosing suitable candidates for the EEA and in selecting those for whom surgery through the oropharynx or the facial bones is the better approach.

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Editorial

Neurosurgical education for pediatricians

Joseph H. Piatt