✓ The treatment of patients with Cushing disease and without magnetic resonance (MR) imaging evidence of Cushing disease (that is, negative MR imaging) is discussed in this paper. Magnetic resonance imaging is the diagnostic modality of choice in Cushing disease, but in up to 40% of these patients negative imaging can be caused by tumor-related factors and limitations in imaging techniques. In cases in which the MR imaging is negative, it is critical to make sure that the diagnosis of Cushing disease is correct. This can be accomplished by performing a complete laboratory and imaging workup, including dexamethasone suppression tests, imaging of the adrenal glands, and inferior petrosal sinus sampling when appropriate. If these evaluations suggest a pituitary source of the hypercortisolemia, then transsphenoidal surgery remains the treatment of choice. The authors favor the endoscopic approach because it gives a wider and more magnified view of the sella and allows inspection of the medial cavernous sinus walls. Radiosurgery is an effective treatment option in patients with persistent Cushing disease. When a target cannot be found on MR imaging, one can target the entire sellar region with radiosurgery.
Jay Jagannathan, Jason P. Sheehan and John A. Jane Jr.
Jay Jagannathan, Aaron S. Dumont, John A. Jane Jr. and Edward R. Laws Jr.
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.
Case report and review of the literature
Daniel M. Prevedello, Jay Jagannathan, John A. Jane Jr., M. Beatriz S. Lopes and Edward R. Laws Jr.
Pituitary adenomas are heterogeneous in growth rate, invasiveness, and recurrence. To understand the biological behavior of the individual adenoma more fully, cell proliferation markers such as monoclonal antibodies targeted against the Ki-67 antigen have been applied. The Ki-67 antigen is a protein related to cell proliferation and is expressed in cell nuclei throughout the entire cell cycle. The authors report the case of an extremely rapidly growing pituitary adenoma with cavernous sinus invasion. The lesion, which displayed a high Ki-67 labeling index (LI; 22%), was found in a 54-year-old woman who presented with diplopia and headaches. The patient underwent three transsphenoidal operations in less than 6 months and, ultimately, was treated with fractionated intensity-modulated radiation therapy. The relationships between high Ki-67 LIs and tumor recurrence, invasiveness, and growth velocity in pituitary adenomas are reviewed.
Jay Jagannathan, Aaron S. Dumont, Daniel M. Prevedello, Christopher I. Shaffrey and John A. Jane Jr.
✓Sports-related injuries to the spine, although relatively rare compared with head injuries, contribute to significant morbidity and mortality in children. The reported incidence of traumatic cervical spine injury in pediatric athletes varies, and most studies are limited because of the low prevalence of injury. The anatomical and biomechanical differences between the immature spine of pediatric patients and the mature spine of adults that make pediatric patients more susceptible to injury include a greater mobility of the spine due to ligamentous laxity, shallow angulations of facet joints, immature development of neck musculature, and incomplete ossification of the vertebrae. As a result of these differences, 60 to 80% of all pediatric vertebral injuries occur in the cervical region. Understanding pediatric injury biomechanics in the cervical spine is important to the neurosurgeon, because coaches, parents, and athletes who place themselves in positions known to be associated with spinal cord injury (SCI) run a higher risk of such injury and paralysis. The mechanisms of SCI can be broadly subclassified into five types: axial loading, dislocation, lateral bending, rotation, and hyperflexion/hyperextension, although severe injuries often result from a combination of more than one of these subtypes. The aim of this review was to detail the characteristics and management of pediatric cervical spine injury.
Jay Jagannathan, Daniel M. Prevedello, Vivek S. Ayer, Aaron S. Dumont, John A. Jane Jr. and Edward R. Laws
In this study the authors address the efficacy and safety of frameless stereotaxy in transsphenoidal surgery.
One thousand transsphenoidal operations were performed at the authors' institution between June 2000 and July 2005. This series consists of a retrospective review of 176 patients entered in a prospectively obtained database who underwent frameless stereotactic transsphenoidal surgery in which magnetic resonance (MR) imaging, computerized tomography (CT) scanning, or fluoroscopic guidance was used. Of the 176 patients, 104 (59%) had suprasellar extension of their tumor, 70 (40%) had involvement of the visual apparatus, and 65 (37%) had cavernous sinus involvement. All patients underwent detailed pre- and postoperative neurological, endocrinological, radiographic, and ophthalmological follow-up evaluations. Records were reviewed retrospectively for intraoperative and postoperative complications related to the surgical approach.
No instances of visual deterioration, carotid artery (CA) stenosis, or stroke were observed following transsphenoidal surgery. Only one patient sustained damage to the CA intraoperatively, and this was controlled in the operating room. Five patients (3%) required an intensive care unit stay postoperatively. Intraoperative cerebrospinal fluid leakage was encountered in 112 patients (64%) and was more frequently observed in tumors with suprasellar involvement.
Frameless stereotaxy is a safe and effective modality for the treatment of recurrent or invasive sellar masses. All three frameless stereotaxy modalities provided accurate information regarding the anatomical midline and the trajectory to the sella turcica. The MR imaging, CT scanning, and fluoroscopic stereotaxy modalities all have unique advantages as well as specific limitations.
Daniel M. Prevedello, Francesco Doglietto, John A. Jane Jr., Jay Jagannathan, Joseph Han and Edward R. Laws Jr.
✓The history of the endoscope exemplifies the manner in which technological advances influence medicine and surgery. Endoscopic systems have evolved and improved, and they currently provide detailed visualization of a variety of deep organ structures. Otorhinolaryngological surgeons have used the endoscope for more than 30 years. In the 1990s, a number of influential neurosurgeons and otorhinolaryngological surgeons began performing purely endoscopic pituitary surgery. Endoscopic transsphenoidal operations are now extending beyond the sella. The collaboration between otorhinolaryngologists and neurosurgeons has produced a new subspecialty of “endoscopic skull base surgery.” There is a great deal of progress still to be made in developing skills, instruments, and improving skull base repair. The extended skull base approaches allow surgical exposures from the olfactory groove to C-2 and to the infratemporal region and jugular fossa laterally. This article discusses the history of the endoscope, the pivotal technological advances, and the key figures in the burgeoning field of endoneurosurgery.
John A. Jane Jr., Joseph Han, Daniel M. Prevedello, Jay Jagannathan, Aaron S. Dumont and Edward R. Laws Jr.
Sellar tumors are most commonly approached through the transsphenoidal corridor, and tumor resection is most often performed using the operating microscope. More recently the endoscope has been introduced for use either as an adjunct to or in lieu of the microscope. Both the microscopic and endoscopic transsphenoidal approaches to sellar tumors allow safe and effective tumor resection. The authors describe their current endoscopic technique and elucidate the advantages and disadvantages of the pure endoscopic adenomectomy compared with the standard microscopic approach.
Jay Jagannathan, David O. Okonkwo, Hian Kwang Yeoh, Aaron S. Dumont, Dwight Saulle, Julie Haizlip, Jeffrey T. Barth, John A. Jane Sr. and John A. Jane Jr.
The management strategies and outcomes in pediatric patients with elevated intracranial pressure (ICP) following severe traumatic brain injury (TBI) are examined in this study.
This study was a retrospective review of a prospectively acquired pediatric trauma database. More than 750 pediatric patients with brain injury were seen over a 10-year period. Records were retrospectively reviewed to determine interventions for correcting ICP, and surviving patients were contacted prospectively to determine functional status and quality of life. Only patients with 2 years of follow-up were included in the study.
Ninety-six pediatric patients (age range 3–18 years) were identified with a Glasgow Coma Scale score < 8 and elevated ICP > 20 mm Hg on presentation. The mean injury severity score was 65 (range 30–100). All patients were treated using a standardized head injury protocol. The mean time course until peak ICP was 69 hours postinjury (range 2–196 hours). Intracranial pressure control was achieved in 82 patients (85%). Methods employed to achieve ICP control included maximal medical therapy (sedation, hyperosmolar therapy, and paralysis) in 34 patients (35%), ventriculostomy in 23 patients (24%), and surgery in 39 patients (41%). Fourteen patients (15%) had refractory ICP despite all interventions, and all of these patients died. Seventy-two patients (75%) were discharged from the hospital, whereas 24 (25%) died during hospitalization. Univariate and multivariate analysis revealed that the presence of vascular injury, refractory ICP, and cisternal effacement at presentation had the highest correlation with subsequent death (p < 0.05). Mean follow-up was 53 months (range 11–126 months). Three patients died during the follow-up period (2 due to infections and 1 committed suicide). The mean 2-year Glasgow Outcome Scale score was 4 (median 4, range 1–5). The mean patient competency rating at follow-up was 4.13 out of 5 (median 4.5, range 1–4.8). Univariate analysis revealed that the extent of intracranial and systemic injuries had the highest correlation with long-term quality of life (p < 0.05).
Controlling elevated ICP is an important factor in patient survival following severe pediatric TBI. The modality used for ICP control appears to be less important. Long-term follow-up is essential to determine neurocognitive sequelae associated with TBI.
Jay Jagannathan, David O. Okonkwo, Aaron S. Dumont, Hazem Ahmed, Abbas Bahari, Daniel M. Prevedello, John A. Jane Sr. and John A. Jane Jr.
The authors examine the indications for and outcomes following decompressive craniectomy in a single-center pediatric patient population with traumatic brain injury (TBI).
A retrospective review of data was performed using a prospectively acquired database of patients who underwent decompressive craniectomy at the authors' institution between January 1995 and April 2006. The patients' neuroimages were examined to evaluate the extent of intracranial injury, and the patients' records were reviewed to determine the admission Glasgow Coma Scale (GCS) score, the extent of systemic injuries, the time to craniectomy, and the indications for craniectomy. Long-term functional outcome and independence levels were evaluated using the Glasgow Outcome Scale (GOS) and a Likert patient quality-of-life rating scale.
Twenty-three craniectomies were performed in children during the study period. The mean patient age at craniectomy was 11.9 years (range 2–19 years). In all patients, the computed tomography scans obtained at presentation revealed pathological findings, with diffuse axonal injury and traumatic contusions being the most common abnormalities. The median presenting GCS score was 4.6 (range 3–9). Nineteen patients (83%) suffered from other systemic injuries. One patient (4%) died intraoperatively and six patients (26%) died postoperatively. Postoperative intracranial pressure (ICP) control was obtained in 19 patients (83%); an ICP greater than 20 mm Hg was found to have the strongest correlation with subsequent brain death (p = 0.001). The mean follow-up duration was 63 months (range 11–126 months, median 49 months). The mean GOS score at the 2-year follow-up examination was 4.2 (median 5). At the most recent follow-up examination, 13 (81%) of 16 survivors had returned to school and only three survivors (18%) were dependent on caregivers.
Although the mortality rate for children with severe TBI remains high, decompressive craniectomy is effective in reducing ICP and is associated with good outcomes in surviving patients.