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Raheel Ahmed and Arnold H. Menezes

Clinical presentation of craniovertebral junction disorders may range from acute catastrophic neurological deficits to insidious signs and symptoms that may mask the underlying etiology. Prompt recognition and treatment is essential to avert long-term neurological morbidity. Proatlas segmentation disorders are a rare group of developmental disorders involving the craniocervical junction. Abnormal bony segmentation leads to malformed bony structures that can in turn lead to neurological deficits through bony compression of the cervicomedullary junction. This report details a proatlas segmentation defect presenting as palatal myoclonus, a rare movement disorder. The clinical presentation, surgical management, and neuroanatomical basis for the disorder is presented. This report highlights the myriad clinical presentations of craniovertebral disorders and emphasizes a rare but treatable etiology for palatal myoclonus.

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Arnold H. Menezes and Raheel Ahmed

Object

Atlantoaxial tumors account for a substantial proportion of primary bone tumors in children. Before resection, surgeons must consider the complex regional anatomy, the potential for neurological compromise, craniocervical instability, and the question of tumor resectability in a growing spine. Using current technology, the authors analyzed surgical cases in this light and present outcomes and treatment recommendations after long-term patient follow-up.

Methods:

The authors reviewed clinical records for 23 children whose primary atlantoaxial bone tumors were treated from 1996 through 2010.

Results

Pathological lesions among the 23 patients were 4 aneurysmal bone cysts, 2 osteochondromas, 5 chordomas, 4 osteoblastomas, 3 fibrous dysplasias, 4 eosinophilic granulomas, and 1 Ewing's sarcoma. Clinical presentation consisted of neck pain (n = 23), headaches and occipital pain (n = 16), myelopathy (n = 8), and torticollis (n = 4). Selective angiography and coil embolization were undertaken for all patients with aneurysmal bone cysts and osteoblastomas, 2 patients with chordomas, 1 patient with fibrous dysplasia, and 1 patient with Ewing's sarcoma. Primary embolization treatment of radiation-induced aneurysmal bone cyst of the atlas showed complete reossification. Results of CT-guided needle biopsy were diagnostic for 1 patient with eosinophilic granuloma and 1 with Ewing's sarcoma. Needle biopsies performed before referral were associated with extreme blood loss for 1 patient and misdiagnosis for 2 patients. Surgery involved lateral extrapharyngeal, transoral, posterior, and posterolateral approaches with vertebral artery rerouting. Complete resection was possible for 9 patients (2 with osteochondroma, 3 with fibrous dysplasia, 2 with chordoma, and 2 with osteoblastoma). Decompression and internal fusion were performed for 3 patients with aneurysmal bone cysts. Of the 23 patients, 7 underwent dorsal fusion and 4 underwent ventral fusion of the axis body. Chemotherapy was necessary for the patients with eosinophilic granuloma with multifocal disease and for the patient with Ewing's sarcoma. There was no morbidity, and there were no deaths. All patients with benign lesions were free of disease at the time of the follow-up visit (mean ± SD follow-up 8.8 ± 1.1 years; range 2–18 years). Chordomas received proton or LINAC irradiation, and as of 4–15 years of follow-up, no recurrence has been noted.

Conclusions

Because most atlantoaxial tumors in children are benign, an intralesional procedure could suffice. Vascular control and staged resection are critical. Ventral transoral fusion or lateral extrapharyngeal fusion has been successful. Resection with ventral fusion and reconstruction are essential for vertebral body collapse. Management of eosinophilic granulomas must be individualized and might require diagnosis through needle biopsy.

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Raheel Ahmed, Arnold H. Menezes, and James C. Torner

OBJECTIVE

Surgical excision is the mainstay treatment for resectable low-grade intramedullary spinal cord tumors (IMSCTs) in the pediatric age group. Chemotherapy and radiation treatments are generally reserved for progressive or recurrent disease. Given the indolent nature of low-grade tumors and the potential side effects of these approaches, their long-term treatment benefits are unclear. The aim of the study was to determine long-term disease outcomes and the therapeutic roles of surgery and adjuvant therapies in pediatric patients with low-grade IMSCTs over an extended follow-up period.

METHODS

Case records for all pediatric patients (< 21 years of age) with a histopathological diagnosis of low-grade IMSCT were selected over a period from January 1975 to January 2010. Outcome variables including McCormick functional grade, overall survival (OS), and progression-free survival (PFS) were analyzed with respect to demographic and treatment variables.

RESULTS

Case records of 37 patients with low-grade IMSCTs were identified, with a mean follow-up duration of 12.3 ± 1.4 years (range 0.5–37.2 years). Low-grade astrocytomas were the most prevalent histological subtype (n = 22, 59%). Gross-total resection (GTR) was achieved in 38% of patients (n = 14). Fusion surgery was required in 62% of patients with pre- or postoperative deformity (10 of 16). On presentation, functional improvement was observed in 87% and 46% of patients in McCormick Grades I and II, respectively, and in 100%, 100%, and 75% in Grades III, IV, and V, respectively. Kaplan-Meier PFS rates were 63% at 5 years, 57% at 10 years, and 44% at 20 years. OS rates were 92% at 5 years, 80% at 10 years, and 65% at 20 years. On multivariate analysis, shunt placement (hazard ratio [HR] 0.33, p = 0.01) correlated with disease progression. There was a trend toward improved 5-year PFS in patients who received adjuvant chemotherapy and radiation therapy (RT; 55%) compared with those who did not (36%). Patients who underwent subtotal resection (STR) were most likely to undergo adjuvant therapy (HR 7.86, p = 0.02).

CONCLUSIONS

This extended follow-up duration in patients with low-grade IMSCTs beyond the first decade indicates favorable long-term OS up to 65% at 20 years. GTR improved PFS and was well tolerated with sustained functional improvement in the majority of patients. Adjuvant chemotherapy and RT improved PFS in patients who underwent STR. These results emphasize the role of resection as the primary treatment approach, with adjuvant therapy reserved for patients at risk for disease progression and those with residual tumor burden.

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Kimberly Hamilton, Susan Rebsamen, Shahriar Salamat, and Raheel Ahmed

An extraosseous intradural presentation for a sacral chordoma in the pediatric age group has not been reported to date. This is a report on an 11-year-old boy who presented with an extraosseous, intradural sacral chordoma. He underwent gross-total resection and received adjuvant proton beam therapy. Neoplastic transformation of the notochord is reviewed to illustrate the developmental basis for the surgical anatomy and pathogenesis of the classic chordoma variant. Clinical and pathological features are reviewed to differentiate this chordoma presentation from classic osseous chordomas and ecchordosis physaliphora, a related benign developmental notochordal lesion. Finally, the role of developmental signaling in the pathogenesis of chordomas from postembryonic notochordal tissue is discussed.

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Raheel Ahmed, Meryl A. Severson III, and Vincent C. Traynelis

Object

Hyperbaric oxygen therapy (HBO) is used as primary and/or adjunctive therapy in the treatment of various clinical conditions complicated by local hypoxia. It may have therapeutic potential in the treatment of neurosurgical infections such as spinal osteomyelitis that are associated with significant morbidity rates. The purpose of this study was to evaluate the efficacy of HBO therapy in the treatment of spinal osteomyelitis.

Methods

The clinical records of patients diagnosed with spinal osteomyelitis who received HBO therapy during their treatment at the authors' institution over the past 10 years were retrospectively reviewed. Six adult patients were identified. Four patients had recently undergone spinal surgery and secondary spinal osteomyelitis had developed. These patients received adjunctive HBO therapy due to significant comorbidities and risk factors for poor healing.

Results

All patients remained symptom and infection free over the subsequent follow-up period. Two patients had primary spinal osteomyelitis that had recurred despite a full course of appropriate antimicrobial therapy. Infection control was achieved after HBO therapy in 1 patient. The mean follow-up period for the study group was 2.9 years (range 5 months to 5 years).

Conclusions

Hyperbaric oxygen therapy enabled infection cure in 5 of 6 patients with spinal osteomyelitis complicated by medical comorbidities or the failure of primary therapy. These results show that HBO may be a useful adjunctive therapeutic modality in the treatment of spinal osteomyelitis, particularly when there are medical comorbidities that increase the risk of poor healing. Hyperbaric oxygen therapy may also be beneficial in patients with relapsing primary spinal osteomyelitis after standard therapy has failed.

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Raheel Ahmed, Arnold H. Menezes, Olatilewa O. Awe, and James C. Torner

Object

Radical resection is recommended as the first-line treatment for pediatric intramedullary spinal cord tumors (IMSCTs), but it is associated with morbidity, including risk of neurological decline and development of postoperative spinal deformity. The authors report long-term data on clinical and treatment determinants affecting disease survival and neurological outcomes.

Methods

Case records for pediatric patients (< 21 years of age at presentation) who underwent surgery for IMSCTs at the authors' institution between January 1975 and January 2010 were analyzed. The patients' demographic and clinical characteristics (including baseline neurological condition), the treatment they received, and their disease course were reviewed. Long-term disease survival and functional outcome measures were analyzed.

Results

A total of 55 patients (30 male and 25 female) were identified. The mean duration of follow-up (± SEM) was 11.4 ± 1.3 years (median 9.3 years, range 0.2–37.2 years). Astrocytomas were the most common tumor subtype (29 tumors [53%]). Gross-total resection (GTR) was achieved in 21 (38%) of the 55 patients. At the most recent follow-up, 30 patients (55%) showed neurological improvement, 17 (31%) showed neurological decline, and 8 (15%) remained neurologically stable. Patients presenting with McCormick Grade I were more likely to show functional improvement by final follow-up (p = 0.01) than patients who presented with Grades II–V. Kaplan-Meier actuarial tumor progression-free survival rates at 5, 10, and 20 years were 61%, 54%, and 44%, respectively; the overall survival rates were 85% at 5 years, 74% at 10 years, and 64% at 20 years. On multivariate analysis, GTR (p = 0.04) and tumor histological grade (p = 0.02) were predictive of long-term survival; GTR was also associated with improved 5-year progression-free survival (p = 0.01).

Conclusions

The prognosis for pediatric IMSCTs is favorable with sustained functional improvement expected in a significant proportion of patients on long-term follow-up. Long-term survival at 10 years (75%) and 20 years (64%) is associated with aggressive resection. Gross-total resection was also associated with improved 5-year progression-free survival (86%). Hence, the treatment benefits of GTR are sustained on extended follow-up.

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Raheel Ahmed, Arnold H. Menezes, Olatilewa O. Awe, Kelly B. Mahaney, James C. Torner, and Stuart L. Weinstein

Object

Spinal deformity in pediatric patients with intramedullary spinal cord tumors (IMSCTs) may be either due to neurogenic disability or due to secondary effects of spinal decompression. It is associated with functional decline and impairment in health-related quality-of-life measures. The authors sought to identify the long-term incidence of spinal deformity in individuals who had undergone surgery for IMSCTs as pediatric patients and the risk factors and overall outcomes in this population.

Methods

Treatment records for pediatric patients (age < 21 years) who underwent surgical treatment for histology-proven primary IMSCTs between 1975 and 2010 were reviewed. All patients were evaluated in consultation with the pediatric orthopedics service. Clinical records were reviewed for baseline and follow-up imaging studies, surgical fusion treatment, and long-term skeletal and disease outcomes.

Results

The authors identified 55 patients (30 males and 25 females) who were treated for pediatric IMSCTs between January 1975 and January 2010. The mean duration of follow-up (± SEM) was 11.4 ± 1.3 years (median 9.3 years, range 0.2–37.2 years). Preoperative skeletal deformity was diagnosed in 11 (20%) of the 55 patients, and new-onset postoperative deformity was noted in 9 (16%). Conservative management with observation or external bracing was sufficient in 8 (40%) of these 20 cases. Surgical fusion was necessary in 11 (55%). Posterior surgical fusion was sufficient in 6 (55%) of these 11 cases, while combined anterior and posterior fusion was undertaken in 5 (45%). Univariate and multivariate analysis of clinical and surgical treatment variables indicated that preoperative kyphoscoliosis (p = 0.0032) and laminectomy/laminoplasty at more than 4 levels (p = 0.05) were independently associated with development of spinal deformity that necessitated surgical fusion. Functional scores and 10-year disease survival outcomes were similar between the 2 groups.

Conclusions

Long-term follow-up is essential to monitor for delayed development of spinal deformity, and regular surveillance imaging is recommended for patients with underlying deformity. The authors' extended follow-up highlights the risk factors associated with development of spinal deformity in patients treated for pediatric IMSCTs. Surgical fusion allows patients who develop progressive deformity to achieve long-term functional and survival outcomes comparable to those of patients who do not develop progressive deformity.

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Newton Cho, Vincent D. W. Nga, Raheel Ahmed, Jerry C. Ku, Pablo M. Munarriz, Prakash Muthusami, James T. Rutka, and Peter Dirks

OBJECTIVE

Pediatric rolandic arteriovenous malformations (AVMs) present a treatment challenge given the lifetime risk of hemorrhage, rehemorrhage, and associated long-term morbidity. Microsurgical resection has been recommended as the optimal treatment for AVMs in general, but there is no dedicated literature on the outcomes of resection of pediatric rolandic AVMs. Here, the study objective was to review the outcomes of microsurgical resection of pediatric rolandic AVMs in the modern era, together with the utilization of surgical adjuncts including navigation, intraoperative angiography, and neurophysiological monitoring.

METHODS

The authors performed a retrospective review of patients 18 years of age and younger with cerebral AVMs microsurgically treated between January 2000 and May 2016 at The Hospital for Sick Children. Only those patients with an AVM whose nidus was located within the rolandic region were analyzed. A descriptive analysis was performed to identify patient demographics, preoperative AVM characteristics, and postoperative obliteration rates and neurological complications.

RESULTS

A total of 279 AVMs were evaluated in the study period. Twenty-three of these AVMs were rolandic, and the median age in the 11 microsurgically treated cases was 11 years (range 1–17 years). AVM hemorrhage was the most common presentation, occurring in 8 patients (73%). Lesions were either Spetzler-Martin grade II (n = 8, 73%) or grade III (n = 3, 27%). The postoperative obliteration rate of AVMs was 100%. The mean imaging follow-up duration was 33 months (range 5–164 months). There was no documented recurrence of an AVM during follow-up. One patient developed a transient postoperative hemiparesis, while another patient developed right fingertip hyperesthesia.

CONCLUSIONS

Microsurgical resection of rolandic pediatric AVMs yields excellent AVM obliteration with minimal neurological morbidity in selected patients. The incorporation of surgical adjuncts, including neurophysiological monitoring and neuronavigation, allows accurate demarcation of functional cortex and enables effective resection.