The diagnosis and management of pediatric sellar lesions is discussed in this paper. Craniopharyngiomas account for the majority of pediatric sellar masses, and pituitary adenomas are extremely uncommon during childhood. The diagnosis of sellar lesions involves a multidisciplinary effort, and detailed endocrinological, ophthalmological, and neurological testing is critical in the evaluation of a new sellar mass. The management of pituitary adenomas varies depending on the entity. For most tumors other than prolactinomas, transsphenoidal resection remains the mainstay of treatment. Less invasive methods, such as endoscopic transsphenoidal surgery and stereotactic radiosurgery, have shown promise as primary and adjuvant treatment modalities, respectively.
Jay Jagannathan, Aaron S. Dumont, John A. Jane Jr. and Edward R. Laws Jr.
Edward R. Laws, Adam S. Kanter, John A. Jane Jr. and Aaron S. Dumont
Jason P. Sheehan, Ajay Niranjan, Jonas M. Sheehan, John A. Jane Jr., Edward R. Laws, Douglas Kondziolka, John Flickinger, Alex M. Landolt, Jay S. Loeffler and L. Dade Lunsford
Object. Pituitary adenomas are very common neoplasms, constituting between 10 and 20% of all primary brain tumors. Historically, the treatment armamentarium for pituitary adenomas has included medical management, microsurgery, and fractionated radiotherapy. More recently, radiosurgery has emerged as a viable treatment option. The goal of this research was to define more fully the efficacy, safety, and role of radiosurgery in the treatment of pituitary adenomas.
Methods. Medical literature databases were searched for articles pertaining to pituitary adenomas and stereotactic radiosurgery. Each study was examined to determine the number of patients, radiosurgical parameters (for example, maximal dose and tumor margin dose), duration of follow-up review, tumor growth control rate, complications, and rate of hormone normalization in the case of functioning adenomas.
A total of 35 peer-reviewed studies involving 1621 patients were examined. Radiosurgery resulted in the control of tumor size in approximately 90% of treated patients. The reported rates of hormone normalization for functioning adenomas varied substantially. This was due in part to widespread differences in endocrinological criteria used for the postradiosurgical assessment. The risks of hypopituitarism, radiation-induced neoplasia, and cerebral vasculopathy associated with radiosurgery appeared lower than those for fractionated radiation therapy. Nevertheless, further observation will be required to understand the true probabilities. The incidence of other serious complications following radiosurgery was quite low.
Conclusions. Although microsurgery remains the primary treatment modality in most cases, stereotactic radiosurgery offers both safe and effective treatment for recurrent or residual pituitary adenomas. In rare instances, radiosurgery may be the best initial treatment for patients with pituitary adenomas. Further refinements in the radiosurgical technique will likely lead to improved outcomes.
A historical perspective
Adam S. Kanter, Aaron S. Dumont, Ashok R. Asthagiri, Rod J. Oskouian, John A. Jane Jr. and Edward R. Laws Jr.
Over the last century, the transsphenoidal approach has evolved into the first-line method of treatment for sellar as well as select groups of parasellar and suprasellar lesions. The journey to its current popularity has been marked by controversy and near abandonment in the late 1920s, followed by its renaissance in the late 1960s. Despite the profound skepticism with which this procedure was viewed, several visionary neurosurgeons persevered through its nadir in popularity, preserving this surgical corridor to the skull base. Advances in medical and surgical techniques, paralleling an improved understanding of pituitary pathophysiology, contributed to its resurgence. The transsphenoidal procedures now performed stem from an array of modifications and refinements accumulated through nearly 100 years of medical and surgical evolution. This era's critical innovations and neurosurgical personalities are the topic of this historical overview.
Invited submission from the Joint Section Meeting on Disorders of the Spine and Peripheral Nerves, March 2004
John A. Jane Sr., Jonathan H. Sherman, Paul T. Boulos, Craig Luce and Aaron S. Dumont
✓ Although its management continues to evolve, lumbar stenosis remains a common societal problem. The present article is based on an invited lecture at the 2004 Annual Meeting of the Congress of Neurological Surgeons/American Association of Neurological Surgeons Joint Section on Disorders of the Spine and Peripheral Nerves. In it the authors provide a historical overview of lumbar stenosis and describe how the senior author's treatment of this condition has evolved over the past four decades. Within each era of treatment, the reasons for modification of treatment methods and relevant outcome measures are outlined. Additionally, specific subsets of patients with lumbar stenosis are also discussed to emphasize unique characteristics that affect treatment strategies. The authors' present technique for management of lumbar stenosis is also illustrated.
John A. Jane Jr., Mary Lee Vance, C. J. Woodburn and Edward R. Laws Jr.
Surgical and medical therapies successfully achieve biochemical remission in the majority of patients with secretory pituitary adenomas. Nevertheless, continued hypersecretion after first-line therapy occurs and requires adjunctive therapy to prevent morbidity and premature mortality. For patients in whom medical and surgical therapy have failed, gamma knife surgery (GKS) is performed with the goal of controlling tumor growth and excess growth hormone (GH) production. The authors report their experience with GKS in patients in whom surgical and medical therapies failed.
The neuroendocrine service at the University of Virginia has treated 220 patients with secretory adenomas. The authors evaluated the biochemical results in patients with acromegaly followed for greater than 18 months (64 patients) as well as those with Cushing disease (45 patients), Nelson syndrome (14 patients with adequate follow up [27 overall]), and prolactinomas (19 patients) followed for at least 12 months posttreatment. Biochemical remission occurred in 36% of patients with GH-secreting adenomas, 73% of those with Cushing disease, 14% of those with Nelson syndrome, and 11% of those harboring prolactinomas. Recurrence after biochemical remission was documented in four patients with Cushing disease. New hormonal deficits have occurred in 28% of patients with acromegaly, 31% with Cushing disease, 36% with Nelson syndrome, and 21% with prolactinomas. Minor visual deterioration developed in one patient with Cushing disease.
Gamma knife surgery offers an important treatment modality in patients with secretory adenomas refractory to surgical and medical interventions.
Case report and review of the literature
John W. McDonald, Daniel Becker, Cristina L. Sadowsky, John A. Jane Sr., Thomas E. Conturo and Linda M. Schultz
✓ The authors of this prospective, single-case study evaluated the potential for functional recovery from chronic spinal cord injury (SCI). The patient was motor complete with minimal and transient sensory perception in the left hemibody. His condition was classified as C-2 American Spinal Injury Association (ASIA) Grade A and he had experienced no substantial recovery in the first 5 years after traumatic SCI. Clinical experience and evidence from the scientific literature suggest that further recovery would not take place. When the study began in 1999, the patient was tetraplegic and unable to breathe without assisted ventilation; his condition classification persisted as C-2 ASIA Grade A. Magnetic resonance imaging revealed severe injury at the C-2 level that had left a central fluid-filled cyst surrounded by a narrow donutlike rim of white matter. Five years after the injury a program known as “activity-based recovery” was instituted. The hypothesis was that patterned neural activity might stimulate the central nervous system to become more functional, as it does during development. Over a 3-year period (5–8 years after injury), the patient's condition improved from ASIA Grade A to ASIA Grade C, an improvement of two ASIA grades. Motor scores improved from 0/100 to 20/100, and sensory scores rose from 5–7/112 to 58–77/112. Using electromyography, the authors documented voluntary control over important muscle groups, including the right hemidiaphragm (C3–5), extensor carpi radialis (C-6), and vastus medialis (L2–4). Reversal of osteoporosis and an increase in muscle mass was associated with this recovery. Moreover, spasticity decreased, the incidence of medical complications fell dramatically, and the incidence of infections and use of antibiotic medications was reduced by over 90%. These improvements occurred despite the fact that less than 25 mm2 of tissue (approximately 25%) of the outer cord (presumably white matter) had survived at the injury level.
The primary novelty of this report is the demonstration that substantial recovery of function (two ASIA grades) is possible in a patient with severe C-2 ASIA Grade A injury, long after the initial SCI. Less severely injured (lower injury level, clinically incomplete lesions) individuals might achieve even more meaningful recovery. The role of patterned neural activity in regeneration and recovery of function after SCI therefore appears a fruitful area for future investigation.
Jason P. Sheehan, Jonas M. Sheehan, M. Beatriz Lopes and John A. Jane Sr.
✓ Diastematomyelia is a rare entity in which some portion of the spinal cord is split into two by a midline septum. Most cases occur in childhood, but some develop in adulthood. A variety of concurrent spinal anomalies may be found in patients with diastematomyelia.
The authors describe a 38-year-old right-handed woman who presented with a 7-month history of lower-extremity pain and weakness on the right side. She denied recent trauma or illness. Sensorimotor deficits, hyperreflexia, and a positive Babinski reflex in the right lower extremity were demonstrated on examination.
Neuroimaging revealed diastematomyelia extending from T-1 to T-3, an expanded right hemicord from T-2 to T-4, and a C6–7 syrinx. The patient underwent T1–3 total laminectomies, resection of the septum, untethering of the cord, and excision of the hemicord lesion. The hemicord mass was determined to be an intramedullary epidermoid cyst; on microscopic evaluation the diastematomyelia cleft was shown to contain fibroadipose connective tissue with nerve twigs and ganglion cells. Postoperatively, the right lower-extremity pain, weakness, and sensory deficits improved.
Diastematomyelia can present after a long, relatively asymptomatic period and should be kept in the differential diagnosis for radiculopathy, myelopathy, tethered cord syndrome, or cauda equina syndrome. Numerous spinal lesions can be found in conjunction with diastematomyelia. To the authors' knowledge, this is the first case in which a thoracic epidermoid cyst and cervical syrinx occurred concurrently with an upper thoracic diastematomyelia. Thorough neuraxis radiographic evaluation and surgical treatment are usually indicated.
Jason P. Sheehan, Gregory A. Helm, Jonas M. Sheehan and John A. Jane Sr.
Lumbar spinal stenosis can be effectively treated by performing an extensive ipsilateral spinal decompression, including a partial pediculotomy, and contralateral posterior bone fusion. Infrequently, complications can arise following radical decompression to alleviate symptoms of stenosis, and one such complication is a pedicle fracture. Three reports of pedicle fractures following extensive spinal decompression and contralateral posterior fusion are detailed. This complication is emphasized, and interventions are discussed.
Three patients presented with symptoms attributable to lumbar stenosis; they were initially treated with an ipsilateral decompression, achieved in part, through a partial pediculotomy followed by contralateral autologous bone fusion. Initially, all three patients improved postoperatively; however, they later developed neurological symptoms ipsilateral to the side of spinal decompression. Computerized tomography scanning demonstrated pedicle fractures on the decompressed side. This complication has not yet been reported in association with decompression and fusion for lumbar stenosis.
Two of the patients developed leg pain necessitating reoperation whereas the third experienced only mild transient symptoms. The fractured pedicle was removed in one patient; laminar and spinous process fusion was performed again. Another patient underwent a total laminectomy, removal of the fractured pedicle, and bilateral transverse process fusion. Reoperation yielded satisfactory outcomes. The third patient's symptoms resolved without intervention.
Pedicle fractures are a potential complication of extensive lumbar decompression and contralateral posterior fusion. Loading forces from the facets or transverse processes are possibly the cause of such fractures. Removal of the fractured pedicle, additional decompression, and enhanced bone fusion are recommended when the symptoms warrant surgical intervention.