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Closed spinal dysraphism (CSD) encompasses a heterogeneous group of spinal cord deformities, which can be accompanied by several types of skin stigmata. These skin stigmata may include inconspicuous features, such as sacral dimples and deformed gluteal clefts, but the association between such mild skin stigmata and CSD is uncertain. This study aimed to reevaluate the indication for magnetic resonance imaging (MRI) in patients with skin stigmata while considering the indication for surgery. A retrospective analysis was conducted on magnetic resonance images of 1255 asymptomatic children with skin stigmata between 2003 and 2015. Skin stigmata classification was based on medical chart data. All subtypes of CSDs except for filum terminale lipomas (FTL), FTL thicker than 2 mm or with low conus medullaris, were considered to meet the surgical indication. CSD prevalence was estimated while considering the surgical indications and assessed after excluding all FTL cases. Skin stigmata were classified into seven types, dimple, deformed gluteal cleft, hair, subcutaneous mass, appendage, discoloration, and protruding bone, and included 1056 isolated and 199 complex ones. The prevalence of CSD was 19.5%, 6.8%, and 0.5% among patients with isolated dimples (n = 881) and 13.9%, 5.8%, and 0.7% among those with isolated deformed gluteal clefts (n = 136) for all cases, surgical indications, and patients without FTL, respectively. Dimples and deformed gluteal clefts had a low prevalence of CSD requiring surgical intervention, and cases without FTL were rare. Asymptomatic patients with mild skin stigmata may not require immediate MRI.

Background and Purpose: In pediatric moyamoya disease, there have been few reports of the risk factors for preoperative cerebral infarction, especially during the waiting period before surgery. The clinical and radiological findings of surgically treated pediatric moyamoya patients were evaluated to analyze the risk factors for cerebral infarction seen from onset to surgery., Methods: Between August 2003 and September 2019, 120 hemispheres of 71 patients under 18 years of age with moyamoya disease were surgically treated by direct and indirect bypass procedures. The mean age of all surgical hemispheres at diagnosis was 6.7±3.9 years (6 months–17 years). The potential risk factors for preoperative infarction were examined statistically., Results: Multivariate logistic regression analysis showed that risk factors for infarction at the time of diagnosis were age at diagnosis (odds ratio [OR], 0.68 [95% CI, 0.57–0.82]; P<0.0001) and the magnetic resonance angiography (MRA) score (OR, 2.29 [95% CI, 1.40–3.75]; P=0.001). Univariate analysis showed that risk factors for infarction while waiting for surgery were age at diagnosis (OR, 0.61 [95% CI, 0.46–0.80]; P<0.0001), the MRA score (OR, 1.75 [95% CI, 1.26–2.41]; P=0.0003), and onset of infarction (OR, 40.4 [95% CI, 5.08–322.3]; P<0.0001). Multiple comparisons showed that patients under 4 years of age were at a significantly high risk of infarction at the time of diagnosis and while waiting for surgery. Time from diagnosis to surgery of >2 months was a significant risk factor for infarction while waiting for surgery in patients under 6 years of age., Conclusions: Young age at diagnosis and a high MRA score may be associated with rapid disease progression and result in preoperative infarction. We recommend that surgery be performed within 2 months of diagnosis for the patients under 4 years of age with a high MRA score (>5) and cerebral infarction. Further study is needed to define the optimal timing of surgery.

The pathophysiology and optimal treatment for hydrocephalus with Blake's pouch cyst (BPC) remain controversial. The authors present two pediatric cases of hydrocephalus associated with BPC, in which the patients' hydrocephalus progressed after endoscopic third ventriculostomy (ETV), despite a patent stoma of the third ventricular floor. Case 1: A 4-year-old girl with delayed gait development was diagnosed with BPC-associated hydrocephalus and received ETV. Postoperatively, the patient presented headaches and nausea. Computed tomography (CT) scans demonstrated larger ventricles than those observed on the preoperative images. Because phase-contrast cine magnetic resonance imaging (MRI) and constructive interference in steady state (CISS) MRI revealed patent cerebrospinal fluid (CSF) flow at the third ventricular floor level, a ventriculoperitoneal shunt (VPS) was placed using a programmable pressure valve to treat the hydrocephalus. Case 2: A 6-year-old girl with newly developed repeated convulsive seizures was diagnosed with BPC-associated hydrocephalus and received ETV. Phase-contrast cine MRI on the 5th postoperative day showed hyperdynamic CSF flow at the third ventricular floor level. She also developed vomiting and headache 6 weeks after ETV. CT scans demonstrated much larger tetraventricular hydrocephalus than that observed on the preoperative images. VPS placement improved her hydrocephalus. Referencing the previous literature, we discuss the CSF dynamics and the mechanism of BPC-associated hydrocephalus, focusing on the third ventricular floor bulging. We hope our experience will help elucidate the pathophysiology and treatment strategies for BPC-associated hydrocephalus., Competing Interests: Conflicts of Interest The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper., (© 2020 The Japan Neurosurgical Society.)

Filum terminale lipoma (FTL) causes various spinal symptoms known as tethered cord syndrome. The treatment for FTL is surgical untethering by sectioning the FTL, which can prevent symptom progression and often results in improvement of symptoms. This report describes a minimally invasive surgical strategy that we have introduced for FTL sectioning. The pediatric patients with FTL since 2007 were treated using this minimally invasive surgical strategy, which we refer to as an interlaminar approach (ILA). In summary, the surgical technique involves: minimal skin incision to expose the unilateral ligamentum flavum in the lower lumbar region; ligamentum flavum incision to expose the dural sac, and dural incision followed by identification and sectioning of the filum. Postoperatively, no bed rest was required. Prior to introducing ILA, we had used standard one level laminectomy/laminotomy (LL) with more than 1 week of postsurgical bed rest until 2007, providing an adequate control group for the benefit of the ILA. A total of 49 consecutive patients were treated using ILA. While 37 patients were treated using LL. Surgical complications that need surgery were seen only in one patient, who developed cerebrospinal fluid (CSF) leak in LL patients. No retethering or additional neurological symptoms were seen during follow-up. All patients complained of minimal postsurgical back pain, but no patients required postoperative bed rest in ILA patients, while LL patients need postsurgical bed rest because of back pain. The ILA strategy provides the advantage of a minimal tissue injury, associated with minimal postoperative pain, blood loss, and bed rest.