✓ Neoplastic angioendotheliosis is a rare disorder characterized by intravascular neoplastic proliferation of endothelial cells within vessels of all caliber in the meninges and neuropil. Ischemic infarcts of brain and spinal cord result from occlusion of the lumina by neoplastic cells or fibrin thrombi. Transition from reactive to neoplastic endothelium can be identified in many vessels. Steroid therapy can be beneficial in reduction of severity of symptoms, but cannot alter the course of disease. Therapeutic intervention must be undertaken promptly after the diagnosis is confirmed by meningeal and cortical biopsy if the inexorable course of the disease is to be altered.
Linda Ansbacher, Nancy Low, David Beck, David Boarini, Charles Jacoby and Pasquale A. Cancilla
Lester Lee, Sharon Low, David Low, Lee Ping Ng, Colum Nolan and Wan Tew Seow
The introduction of ventriculoperitoneal shunts changed the way hydrocephalus was treated. Whereas much is known about the causes of shunt failure in the first few years, there is a paucity of data in the literature regarding the cause of late shunt failures. The authors conducted a study to find out the different causes of late shunt failures in their institution.
A 10-year retrospective study of all the patients who were treated in the authors' hospital between 2006 and 2015 was conducted. Late shunt failures included those in patients who had to undergo shunt revision more than 5 years after their initial shunt insertion. The patient's notes and scans were reviewed to obtain the age and sex of the patient, the time it took for the shunt to fail, the reason for failure, and the patient's follow-up.
Forty-six patients in the authors' institution experienced 48 late shunt failures in the last 10 years. Their ages ranged from 7 to 26 years (12.23 ± 4.459 years [mean ± SD]). The time it took for the shunts to fail was between 6 and 24 years (mean 10.25 ± 3.77 years). Reasons for failure resulting in shunt revision include shunt fracture in 24 patients (50%), shunt blockage in 14 patients (29.2%), tract fibrosis in 6 patients (12.5%), shunt dislodgement in 2 patients (4.2%), and shunt erosion in 2 patients (4.2%). Postoperative follow-up for the patients ranged from 6 to 138 months (mean 45.15 ± 33.26 months).
Late shunt failure is caused by the effects of aging on the shunt, and the complications are different from early shunt failure. A large proportion are complications associated with shunt calcification. The authors advocate a long follow-up for pediatric patients with shunts in situ to monitor them for various causes of late shunt failure.
Eamon J. McLaughlin, Gregory G. Heuer, Robert G. Whitmore, John K. Birknes, Jean Belasco, Daniel Sterman, David W. Low and Phillip B. Storm
The authors report the case of a 14-year-old girl with a residual malignant peripheral nerve sheath tumor after thoracotomy, chemotherapy, and radiation therapy. The residual tumor, which involved the intercostal muscles, aorta, and neural foramina of T4–10, was completely resected through a costotransversectomy and multiple hemilaminotomies with the patient in the prone position and was stabilized using a T1–12 pedicle screw fusion. Postoperatively, the patient developed several infections requiring multiple washouts and prolonged antibiotics. Thirty months after surgery, she developed a bronchocutaneous fistula. The hardware was removed, and a vascularized latissimus dorsi free flap was placed over the lung. She continued to have an air leak and presented 3 weeks later with a 40° left thoracic curve. She returned to the operating room for a T2–L2 fusion with a vascularized fibular graft. On postoperative Day 1, she underwent a bronchoscopy and had her left lower lobe airways occluded with multiple novel one-way endobronchial valves. She is now 5 years out from her tumor resection and 3 years out from her definitive fusion. She has no evidence of residual tumor, infection, or pseudarthrosis and continues to remain asymptomatic.