Hydrocephalus in patients with achondroplasia is thought to be due to increased dural sinus venous pressure resulting from narrowing of the jugular foramen. In this setting, where hydrocephalus is presumed to be “vascular” in origin and therefore communicating, endoscopic third ventriculostomy (ETV) would seem contraindicated. The authors describe 3 patients in whom ETV was successfully performed, resulting in MR imaging–documented decreases in ventricle size. The patients were 11 months, 33 months, and 13 years at the time of surgery. All patients had serial preoperative MR images demonstrating progressive hydrocephalus in a “triventricular” pattern with a small fourth ventricle but an open aqueduct. All patients had undergone suboccipital decompression for foramen magnum stenosis prior to the treatment of hydrocephalus. Preoperative retrograde venography revealed variable pressure gradients across the jugular foramen. It is postulated that the increase in intracranial venous pressure resulting from jugular foramen stenosis may lead to disproportionate venous engorgement of the cerebellum and some degree of obstructive hydrocephalus amenable to ETV. The authors discuss the role of suboccipital decompression in the progression of hydrocephalus in patients with achondroplasia.
Dale Swift, Laszlo Nagy and Brian Robertson
Frederick H. Sklar, Laszlo Nagy and Brian D. Robertson
Headaches are common in children with shunts. Headaches associated with over-shunting are typically intermittent and tend to occur later in the day. Lying down frequently makes the headaches better. This paper examines the efficacy of using abdominal binders to treat over-shunting headaches.
Over an 18-year period, the senior author monitored 1027 children with shunts. Office charts of 483 active patients were retrospectively reviewed to identify those children with headaches and, in particular, those children who were thought to have headaches as a result of over-shunting. Abdominal binders were frequently used to treat children with presumed over-shunting headaches, and these data were analyzed.
Of the 483 patients undergoing chart review, 258 (53.4%) had headache. A clinical diagnosis of over-shunting was made in 103 patients (21.3% overall; 39.9% of patients with headache). In 14 patients, the headaches were very mild (1–2 on a 5-point scale) and infrequent (1 or 2 per month), and treatment with an abdominal binder was not thought indicated. Eighty-nine patients were treated with a binder, but 19 were excluded from this retrospective study for noncompliance, interruption of the binder trial, or lack of follow-up.
The remaining 70 pediatric patients, who were diagnosed with over-shunting headaches and were treated with abdominal binders, were the subjects of a more detailed retrospective study. Significant headache improvement was observed in 85.8% of patients. On average, the patients wore the binders for approximately 1 month, and headache relief usually persisted even after the binders were discontinued. However, the headaches eventually did recur in many of the patients more than a year later. In these patients, reuse of the abdominal binder was successful in relieving headaches in 78.9%.
The abdominal binder is an effective, noninvasive therapy to control over-shunting headaches in most children. This treatment should be tried before any surgery is considered. It is suggested that the abdominal binder may modulate abnormally increased intracranial pulse pressures associated with over-shunting. Interactions with the cerebrovascular bed are suspected to account for persistent headache relief after the binder is discontinued.
Preston D’Souza, Erin K. Barr, Seshadri D. Thirumala, Roy Jacob and Laszlo Nagy
Pigmented epithelioid melanocytomas (PEMs) are low-grade, intermediate-type borderline melanocytic tumors with limited metastatic potential. To date, PEMs have been treated via gross-total resections. Postoperative recurrence and mortality are rare. This case highlights a unique presentation of a PEM that involved bone destruction and intradural infiltration, which required a subtotal resection. To the authors’ knowledge, this is the first report of a PEM extending through the dura and necessitating subtotal resection, which is contrary to the standard of care, gross-total resection. Surveillance imaging 10 months after resection remained negative for clinical and radiological recurrence.