Object The diagnosis and treatment of Chiari malformation Type I (CM-I) has evolved over the last few decades. The authors present their surgical experience of over 2 decades of treating children with this form of hindbrain herniation. Methods The authors conducted a retrospective review of their institutional experience with the surgical treatment of the pediatric CM-I from 1989 to 2010. Results The 2 most common presentations were headache/neck pain (40%) and scoliosis (18%). Common associated diagnoses included neurofibromatosis Type 1 (5%) and idiopathic growth hormone deficiency (4.2%). Spine anomalies included scoliosis (18%), retroversion of the odontoid process (24%), Klippel-Feil anomaly (3%), and atlantooccipital fusion (8%). Approximately 3% of patients had a known family member with CM-I. Hydrocephalus was present in 48 patients (9.6%). Syringomyelia was present in 285 patients (57%), and at operation, 12% of patients with syringomyelia were found to have an arachnoid veil occluding the fourth ventricular outlet. Fifteen patients (3%) have undergone reoperation for continued symptoms or persistent large syringomyelia. The most likely symptoms and signs to resolve following surgery were Valsalva-induced headache and syringomyelia. The average hospital stay and “return to school” time were 3 and 12 days, respectively. The follow-up for this group ranged from 2 months to 15 years (mean 5 years). Complications occurred in 2.4% of cases; there was no mortality. No patient required acute return to the operating room, and no blood transfusions were performed. Conclusions The authors believe this to be the largest reported series of surgically treated pediatric CM-I patients and hope that their experience will be of use to others who treat this surgical entity.

The authors believe this to be the largest reported series of pediatric patients who have undergone posterior fossa decompression for Chiari I malformations.

OBJECT Evidence in support of hemispherectomy stems from a multitude of retrospective studies illustrating individual institutions' experience. A systematic review of this topic, however, is lacking in the literature. METHODS A systematic review of hemispherectomy for the treatment of refractory epilepsy available up to October 2013 was performed using the following inclusion criteria: reports of a total of 10 or more patients in the pediatric age group (≤ 20 years) undergoing hemispherectomy, seizure outcome reported after a minimum follow-up of 1 year after the initial procedure, and description of the type of hemispherectomy. Only the most recent paper from institutions that published multiple papers with overlapping study periods was included. Two reviewers independently applied the inclusion criteria and extracted all the data. RESULTS Twenty-nine studies with a total of 1161 patients met the inclusion criteria. Seizure outcome was available for 1102 patients, and the overall rate of seizure freedom at the last follow-up was 73.4%. Sixteen studies (55.2%) exclusively reported seizure outcomes of a single type of hemispherectomy. There was no statistically significant difference in seizure outcome and type of hemispherectomy (p = 0.737). Underlying etiology was reported for 85.4% of patients with documented seizure outcome, and the overall distribution of acquired, developmental, and progressive etiologies was 30.5%, 40.7%, and 28.8%, respectively. Acquired and progressive etiologies were associated with significantly higher seizure-free rates than developmental etiologies (p < 0.001). Twenty of the 29 studies (69%) reported complications. The overall rate of hydrocephalus requiring CSF diversion was 14%. Mortality within 30 days was 2.2% and was not statistically different between types of hemispherectomy (p = 0.787). CONCLUSIONS Hemispherectomy is highly effective for treating refractory epilepsy in the pediatric age group, particularly for acquired and progressive etiologies. While the type of hemispherectomy does not have any influence on seizure outcome, hemispherotomy procedures are associated with a more favorable complication profile.

Appropriate literature germane to the CIM is reviewed and discussed. There is variation in the reported anatomy, outcome, and treatment for children with CIM. Based on the literature, most patients have preoperative symptoms or findings (e.g., syringomyelia) improve no matter what surgical technique is utilized. However, standardized treatment paradigms based on randomized controlled studies are still necessary to elucidate the optimal selection and treatment criteria.

Thoracic outlet syndrome (TOS) is a condition arising from compression of the subclavian vessels and/or brachial plexus as the structures travel from the thoracic outlet to the axilla. Despite the significant pathology associated with TOS, there remains some general disagreement among experts on the specific anatomy, etiology, and pathophysiology of the condition, presumably because of the wide variation in symptoms that manifest in presenting patients, and because of lack of a definitive gold standard for diagnosis. Symptoms associated with TOS have traditionally been divided into vascular and neurogenic categories, a distinction based on the underlying structure(s) implicated. Of the two, neurogenic TOS (nTOS) is more common, and typically presents as compression of the brachial plexus; primarily, but not exclusively, involving its lower trunk. Vascular TOS (vTOS) usually involves compression of the vessel, most commonly the subclavian artery or vein, or is secondary to thrombus formation in the venous vasculature. Any anatomical anomaly in the thoracic outlet has the potential to predispose a patient to TOS. Common anomalies include variations in the insertion of the anterior scalene muscle (ASM) or scalenus minimus muscle, the presence of a cervical rib or of fibrous and muscular bands, variations in insertion of pectoralis minor, and the presence of neurovascular structures, which follow an atypical course. A common diagnostic technique for vTOS is duplex imaging, which has generally replaced more invasive angiographic techniques. In cases of suspected nTOS, electrophysiological nerve studies and ASM blocks provide guidance when screening for patients likely to benefit from surgical decompression of TOS. Surgeons generally agree that the transaxillary approach allows the greatest field of view for first rib excision to relieve compressed vessels. Alternatively, a supraclavicular approach is favored for scalenotomies when the ASM impinges on surrounding structures. A combined supraclavicular and infraclavicular approach is preferred when a larger field of view is required. The future of TOS management must emphasize the improvement of available diagnostic and treatment techniques, and the development of a consensus gold standard for diagnosis. Helical computed tomography offers a three-dimensional view of the thoracic outlet, and may be valuable in the detection of anatomical variations, which may predispose patients to TOS. This review summarizes the history of TOS, the pertinent clinical and anatomical presentations of TOS, and the commonly used diagnostic and treatment techniques for the condition.

Thyroglossal duct cysts are the most frequently encountered congenital abnormality of the neck, and are described extensively in the literature. The high incidence of this abnormality and its presence in all age groups requires clinicians to be aware of the clinical features, etiology, and current treatment options. Because it is readily accessible, relatively inexpensive, and non-invasive, sonography is arguably the ideal initial investigation. Computerized tomography and fine needle aspiration biopsy are often utilized as supplementary techniques for confirmation of the diagnosis. The treatment of choice for thyroglossal duct cysts continues to be the classic Sistrunk procedure, developed in 1920, although several modifications have since been employed. In this review, we aim to explore the embryological development, clinical presentation and diagnostic features of thyroglossal duct cysts. In addition, a useful adaptation to the Sistrunk procedure and the recent application of OK-432 therapy as a promising alternative for treatment of thyroglossal duct cysts will also be discussed.

Object. The quantitative analysis of odontoid process angulation has had scant attention in the Chiari I malformation population. In this study the authors sought to elucidate the correlation between posterior angulation of the odontoid process and patients with Chiari I malformation. Methods. Magnetic resonance images of the craniocervical junction obtained in 100 children with Chiari I malformation and in 50 children with normal intracranial anatomy (controls) were analyzed. Specific attention was focused on measuring the degree of angulation of the odontoid process and assigning a score to the various degrees. Postoperative outcome following posterior cranial fossa decompression was then correlated to grades of angulation. Other measurements included midsagittal lengths of the foramen magnum and basiocciput, the authors' institutions' previously documented pB—C2 line (a line drawn perpendicular to one drawn between the basion and the posterior aspect of the C-2 body), level of the obex from a midpoint of the McRae line, and the extent of tonsillar herniation. Higher grades of odontoid angulation (retroflexion) were found to be more frequently associated with syringomyelia and particularly holocord syringes. Higher grades of angulation were more common in female patients and were often found to have obices that were caudally displaced greater than three standard deviations below normal. Conclusions. These results not only confirm prior reports of an increased incidence of a retroflexed odontoid process in Chiari I malformation but quantitatively define grades of inclination. Grades of angulation were not found to correlate with postoperative outcome. It is the authors' hopes that these data add to our current limited understanding of the mechanisms involved in hindbrain herniation.

Tuberculum sellae meningiomas frequently extend into the optic canals. Radical tumor resection including the involved dural attachment, underlying hyperostotic bone, and intracanalicular tumor in the optic canal offers the best chance of a Simpson Grade I resection to minimize recurrence. Decompression of the optic canal with removal of the intracanalicular tumor also improves visual outcome since this portion of the tumor is usually the cause of asymmetrical visual loss. The purely endoscopic endonasal extended transsphenoidal approach offers a direct midline trajectory and immediate access to tuberculum sellae meningiomas without brain retraction and manipulation of neurovascular structures. Although the endoscopic approach has been previously criticized for its inability to remove tumor within the optic canals, complete Simpson Grade I tumor removal including intracanalicular tumor, dural attachment, and involved hyperostotic bone can be achieved in properly selected patients. Excellent visualization of the suprasellar region and the inferomedial aspects of both optic canals allows for extracapsular, extraarachnoid dissection of the tumor from the critical structures using bimanual microsurgical dissection. In this report, the authors describe the operative nuances for removal of tuberculum sellae meningiomas with optic canal involvement using a purely endoscopic endonasal extended transsphenoidal (transplanum transtuberculum) approach. They specifically highlight the technique for endonasal bilateral optic nerve decompression and removal of intracanalicular tumor to improve postoperative visual function, as demonstrated in 2 illustrative cases. Special attention is also given to cranial base reconstruction to prevent CSF leakage using the vascularized pedicled nasoseptal flap.