Perioperative care of the pediatric patient for pial synangiosis surgery.

Moyamoya disease (MMD) is a cerebrovascular occlusive disorder which causes recurrent strokes and transient ischemic attacks in children. The arteriopathy of MMD targets the internal carotid arteries (ICA) and their branches resulting in ischemia of the ICA circulation. Small perforator branches of the ICA dilate to provide collateral perfusion to ischemic areas. This small vessel dilatation creates the characteristic angiographic appearance that gives the disease its name ("moyamoya" after the Japanese expression for "something hazy just like a puff of cigarette smoke drifting in the air"). Best medical management involves the prevention of thromboembolic events with antiplatelet agents, maintaining adequate hydration, and avoidance of hyperventilation which can contribute to cerebral vasoconstriction. Presently there are no definitive medical options to halt or correct the process of MMD. Surgical procedures include both direct and indirect revascularization. Direct revascularization involves anastomosis of a branch of the external carotid artery to a branch of the internal carotid artery (STA-MCA bipass). However, indirect procedures are often chosen in the pediatric population due to the technical difficulty of direct procedures related to small diameter blood vessels. Indirect procedures rely on neovascularization, so the increase in cerebral blood flow is delayed beyond the immediate postoperative period. One such indirect procedure is pial synangiosis, which involves suturing the adventitia of the superficial temporal artery to the pial surface of the cerebral cortex after opening of the arachnoid. The authors present two pediatric patients who required anesthetic care for pial synangiosis surgery. The perioperative care of such patients is reviewed and suggestions presented for the intraoperative anesthetic management.

Cribriform neuroepithelial tumor arising in the lateral ventricle.

Cribriform neuroepithelial tumor (CRINET) is a recently recognized central nervous system neoplasm that arises in the ventricles of young children and is characterized by primitive, non-rhabdoid SMARCB1-deficient cells with prominent cribriform architecture. We report a 14-month-old male who presented with signs of increased intracranial pressure. Neuroimaging revealed a large solid and cystic mass in the lateral ventricle. Tumor cells were small, primitive appearing, and arranged in cribriform and trabecular patterns with interspersed solid cellular areas. Rhabdoid cells were absent. Immunohistochemical staining showed no SMARCB1 (INI11, BAF47, hSNF5) expression and strong epithelial membrane antigen (EMA) immunoreactivity along luminal surfaces. Electron microscopy showed epithelial characteristics, including abundant basal lamina. Genetic analysis of the tumor revealed deletion of 1 SMARCB1 allele, while the other contained an intronic point mutation predicted to disrupt splicing. This case further illustrates the distinct histopathologic and molecular genetic features of CRINET and identifies distinctive ultrastructural features.

Endoscope-assisted strip craniectomy and postoperative helmet therapy for treatment of craniosynostosis.

OBJECT The primary goals of treatment in the infant with craniosynostosis are to correct the deformity and allow for adequate brain growth in as safe and effective a manner as possible. Herein, the authors present the results of treating craniosynostosis using an endoscope-assisted strip craniectomy and postoperative helmet therapy (EASC + PHT) in the hopes of providing further evidence of its role in the treatment of multiple different forms of craniosynostosis. This is a retrospective review of the patients treated with this technique at Children's Hospital Boston. METHODS The electronic medical records of all children with craniosynostosis treated using this technique were reviewed retrospectively. A priori, data were collected for deformity type, patient age at surgery, number of transfusions, operative time, length of hospital stay, and anthropometric measurements. RESULTS One hundred seventy-three patients (61 females and 112 males) were treated at our institution between July 2004 and March 2011 with EASC + PHT. The mean operative time was 46.30 minutes. Eight (4.6%) of the 173 patients received blood transfusions. The average length of hospital stay was 1.35 days, with the majority of patients being discharged the day after surgery. All complications and any patient who required additional craniofacial reconstructions are discussed. In addition, a subgroup analysis was done for patients who had undergone surgery and had longer than 1 year of follow-up. CONCLUSIONS The authors' growing database of patients supports the experiences described by others that early treatment of craniosynostosis with an EASC + PHT is a safe and efficacious technique. In addition, cost reduction due to decreased hospital stay and limitation of blood transfusions are demonstrable benefits associated with the use of this technique.

The management of sagittal synostosis using endoscopic suturectomy and postoperative helmet therapy.

OBJECT: Suturectomy as a treatment for craniosynostosis was largely replaced in the late twentieth century by more extensive, but predictable, cranial remodeling procedures. Recent technical innovations, such as using the endoscope combined with postoperative orthotic reshaping, have led to a resurgence of interest in suturectomy as a safer, less invasive method. METHODS: A retrospective chart review was performed for all cases of sagittal synostosis treated with endoscopic sagittal suture strip craniectomy and helmet therapy between 2004 and 2008. Data collected included gestational age, genetic evaluations and syndromic status, age at operation, duration of procedure, need for blood transfusions, length of hospital stay, preoperative and postoperative head circumference percentile and cranial index, duration of helmet use, length of follow-up, complications, and revisions. RESULTS: Fifty-six patients with isolated sagittal synostosis were treated using endoscopic suturectomy and completed helmet therapy. Mean age at time of procedure was 3.24 months. Mean operative duration was 45.32 minutes. Mean hospital stay was 1.39 days. There were 2 transfusions and no deaths. The mean length of follow-up was 2.34 years. Helmet therapy was instituted for a mean of 7.47 months. Head circumference percentile increased from 61.42% to 89.27% over 2 years of follow-up. Cranial index increased from a preoperative mean of 0.69 to 0.76 over 2 years of follow-up. Reoperations for synostosis included 1 sagittal suture refusion and 2 cases in which other sutures fused. CONCLUSIONS: Sagittal synostosis can be safely treated with endoscopic suturectomy and helmet therapy. Improvements in cranial volume and shape are comparable to open procedures and are enduring.