✓ A case is reported of eosinophilic granuloma at the cervicothoracic junction presenting with profound quadriparesis preoperatively. The patient underwent excision via an anterior approach, with splitting of the sternum to gain access to the T-1 vertebra. Postoperatively, he has had an excellent return of function.
Thomas A. Sweasey and Robert C. Dauser
Robert C. Dauser, Gerald F. Tuite, and Charles W. McCluggage
✓ A variety of surgical procedures have been proposed for the treatment of moyamoya disease, but few have used the potential of the middle meningeal artery to any great extent. During the period of spontaneous collateral formation, patients with this disease are at risk for the development of transient ischemic attacks and strokes. Surgical treatments aimed at increasing collateral flow to the brain from the external carotid system have included both direct and indirect anastomotic methods. In this report, the authors describe a technique that used the middle meningeal artery circulation as a source of collateral blood supply by inverting dural flaps that are located on a large meningeal vessel, allowing the richly vascularized outer dural surface to contact a large surface area of the ischemic cortex. An extensive degree of revascularization was observed.
David M. Katz, Jonathan D. Trobe, Karin M. Muraszko, and Robert C. Dauser
✓ Four patients who developed increased intracranial pressure from ventricular shunt failure suffered a delay in diagnosis because magnetic resonance imaging of the brain did not show ventriculomegaly and because ophthalmic findings were initially overlooked or misinterpreted. None of the patients had the conventional manifestations of shunt failure: severe headache, nausea, vomiting, and depressed consciousness. Three patients suffered marked, permanent vision loss from chronic papilledema. These cases affirm that increased intracranial pressure may occur in shunt dependency without producing either conventional clinical symptoms or signs on imaging of the brain. Because ophthalmic manifestations may be the major clues to diagnosis, and because irreversible loss of vision is possible if these clues are overlooked, consideration should be given to periodic ophthalmological examination of shunt-dependent patients.
Michael N. Bucci, John A. Feldenzer, William A. Phillips, Stephen S. Gebarski, and Robert C. Dauser
✓ An unusual case of atlanto-axial rotational limitation secondary to an osteoid osteoma of the axis is presented. Transoral microsurgical resection followed by physical therapy improved the clinical symptoms. This case illustrates several unique problems within the cervical spine as well as the efficacy of the transoral approach to the axis.
Nisha Gadgil, Sandi Lam, Monika Pyarali, Michael Paldino, I-Wen Pan, and Robert C. Dauser
Numerous surgical procedures facilitate revascularization of the ischemic brain in patients with moyamoya disease. Dural inversion is a technique in which flaps of dura mater centered around the middle meningeal artery are inverted, encouraging the formation of a rich collateral blood supply. This procedure has been used in combination with encephaloduroarteriosynangiosis for more than 20 years at the authors’ institution for the treatment of pediatric moyamoya disease. The objective of this study was to describe the clinical and radiographic outcomes for a cohort of consecutive pediatric moyamoya patients undergoing dural inversion.
Clinical and radiographic data on patients who had undergone dural inversion in the period from 1997 to 2016 were reviewed. Univariate and multivariate logistic regression and Kaplan-Meier analyses were performed to assess the risk of postoperative stroke, functional outcome, and the angiographic degree of revascularization.
Dural inversion was performed on 169 hemispheres in 102 patients. Median follow-up was 4.3 years. Six patients (3.6% of hemispheres) suffered postoperative ischemic or hemorrhagic stroke. Overall mortality was 1.0%. Good postoperative neurological status (modified Rankin Scale [mRS] score ≤ 2) was observed in 90 patients (88%); preoperative and postoperative mRS scores showed significant improvement (p < 0.001). Eighty-six percent of hemispheres had Matsushima grade A or B revascularization. Younger age was associated with postoperative stroke and poor functional outcome. Patients with secondary moyamoya syndrome had a significantly worse radiographic outcome. The cumulative 5-year Kaplan-Meier risk for stroke was 6.4%.
Dural inversion is a useful technique of cerebral revascularization in pediatric moyamoya disease. A 20-year experience demonstrates the safety and efficacy of this technique with a relatively low rate of postoperative stroke, good functional outcomes, and favorable angiographic results.
Leonardo Rangel-Castilla, Steven W. Hwang, Andrew Jea, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen, and Robert C. Dauser
Multiple-suture synostosis is typically associated with syndromic craniosynostosis but has been occasionally reported in large series of nonsyndromic children. The diagnosis of multiple fused sutures usually occurs at the same time, but rarely has the chronological development of a secondary suture synostosis been noted. The development of secondary bicoronal suture synostosis requiring surgical intervention has only been reported, to date, after surgical intervention and is hypothesized to arise from a disruption of inhibitory factors from the dura. The disinhibition of these factors permits the sutures to then fuse at an early stage. The authors report on a patient who developed secondary unilateral coronal synostosis after the diagnosis of an isolated sagittal synostosis. The secondary synostosis was identified at the time of the initial surgical intervention and ultimately required a second procedure of a frontoorbital advancement.
The clinical appearance of this phenomenon may be subtle, and surgeons should monitor for the presence of secondary synostosis during surgery as it may require intervention. Failure to identify the secondary synostosis may necessitate another surgery or result in a poor cosmetic outcome. The authors recommend close clinical follow-up for the short term in patients with isolated sagittal synostosis.
Gregory S. McLoughlin, Jed G. Nuchtern, Robert C. Dauser, Daniel M. Sciubba, Ziya L. Gokaslan, and Jean-Paul Wolinsky
✓ Lymphangiomas are benign collections of blind-ended lymphatic and vascular channels. Lesions typically occur in the soft tissues of the head and neck, although any region of the body can be affected. Involvement of the spine is very rare. A complete resection is generally curative. On rare occasions, these tumors are complicated by infection or hemorrhage. The authors present an unusual case of a hemorrhagic lymphangioma in a 1-year-old male child. The lesion originated in the mediastinum and extended into the cervicothoracic epidural space via a neural foramen. This resulted in an acute epidural hematoma and quadriparesis. Emergency decompression resulted in full neurological recovery. This may be the first report of a lymphangioma resulting in an acute epidural hematoma and quadriparesis.
Hidehiro Takei, Yummy Nguyen, Vidya Mehta, Murali Chintagumpala, Robert C. Dauser, and Adekunle M. Adesina
Medulloblastoma (MB) is a malignant embryonal tumor of the cerebellum. Amplification of c-myc or N-myc is infrequently identified and, when present, is often associated with the large cell/anaplastic (LC/A) phenotype. The frequency of low-level copy gain of myc oncogenes and its relationship to prognosis of MB has not been explored.
Archival cases of MB were histologically reviewed and classified into 3 major subtypes: classic, nodular, and LC/A. Using quantitative real-time polymerase chain reaction (PCR), the authors analyzed 58 cases with a pure histological subtype for the copy number (CN) of myc (c-myc and N-myc) oncogenes. Cases with > 5-fold CN were further analyzed using the fluorescent in situ hybridization (FISH) assay. Kaplan-Meier survival analysis was performed.
A > 5-fold myc CN was noted in 5 (20.8%) of 24 LC/A, 1 (5.3%) of 19 classic, and 2 (13.3%) of 15 nodular subtypes. In a significant number of tumors (14 [56%] of 24 LC/A, 13 [68%] of 19 classic, and 10 [67%] of 15 nodular MBs) the CN was > 2-fold but < 5-fold. High-level amplification, defined as > 10-fold CN, was only seen in the LC/A subtype (5 cases), although moderate amplification (> 5-fold but < 10-fold) could be detected in other histological subtypes. Fluorescence in situ hybridization readily detected most cases corresponding to tumors with > 5-fold amplicon CN by quantitative real-time PCR, and could detect all 5 cases with > 10-fold CN by quantitative real-time PCR. The group of patients with > 5-fold myc amplicon CN showed significantly shorter survival than those with < 5-fold CN (p = 0.045), independent of histological subtype.
Since FISH could easily detect most cases in the moderate-to-high myc gene amplification (> 5-fold CN) group, the FISH assay has utility in detecting subsets of MB with poorer prognosis.