Role of Magnetic Resonance Imaging in the Screening of Closed Spinal Dysraphism.

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.

Technical evolution of pediatric neurosurgery: moyamoya disease.

Moyamoya disease (MMD) is a rare steno-occlusive disease of the bilateral internal carotid arteries that predominantly occurs in East Asia. Since the first description of the MMD by Suzuki and Takaku in 1969, significant advances have been made in both basic and clinical understanding of the disease. The incidence and prevalence of pediatric MMD have increased, potentially due to improved detection rates. The advancement of neuroimaging techniques has enabled MRI-based diagnostics and detailed visualization of the vessel wall. Various methods of surgical treatments are successful in pediatric MMD patients, and recent studies emphasize the importance of reducing postoperative complications since the goal of MMD surgery is to prevent future cerebral infarction and hemorrhage. Long-term outcomes following appropriate surgical treatment in pediatric MMD patients have shown promising results, including favorable outcomes in very young patients. Further studies with a large patient cohort are needed to establish individualized risk group stratification for determining the optimal timing of surgical treatment and to conduct multidisciplinary outcome assessments.

[Moyamoya Disease in Infants and Toddlers].

Treatment for moyamoya disease in infants and toddlers is challenging, because of the progressiveness of the disease. Revascularization surgery is the first-line therapy and should be performed as early as possible after diagnosis to prevent additional cerebral infarction. This review describes in detail the changes in the new "Diagnostic Criteria 2021 for moyamoya disease", and discusses the concept of treatment including indirect and combined bypass surgery in infants and toddlers. Surgical technique and perioperative management based on our experience are also precisely described.

Ocular Ischemic Syndrome due to Internal Carotid Artery Occlusion with Neurofibromatosis Type 1.

Neurofibromatosis type 1 (NF-1) is associated with multiple vascular abnormalities, including internal carotid artery (ICA) stenosis/occlusion. Depending on the site of stenosis/occlusion of the ICA, both cerebral circulation and ocular circulation can be impaired. We describe a rare pediatric case of ocular ischemic syndrome (OIS) due to ICA occlusion in NF-1. The patient diagnosed with NF-1 suffered right ICA occlusion at 12 years of age, and developed right dense vitreous hemorrhage due to OIS at 13 years of age. The patient underwent right cerebral revascularization surgery to improve cerebral and ocular ischemia, but the visual acuity of the right eye did not improve. This case suggests that attention should be paid not only to cerebral ischemia but also to ocular ischemia in patients with ICA stenosis/occlusion of NF-1. Surgical interventions such as cerebral revascularization surgery should be considered in the early stages of OIS to protect visual function, and careful follow-up is required.

Retethering risk in pediatric spinal lipoma of the conus medullaris.

OBJECTIVE: Lipoma of the conus medullaris (LCM) causes neurological symptoms known as tethered cord syndrome (TCS). The symptoms can be seen at diagnosis and during long-term follow-up. In this report, pediatric patients with LCMs who underwent untethering surgery, under the policy of performing surgery if diagnosed regardless of symptoms, were retrospectively reviewed to evaluate long-term surgical outcomes. Possible risk factors for retethered cord syndrome (ReTCS) were evaluated in the long-term follow-up period. METHODS: A total of 51 consecutive pediatric patients with LCMs who underwent a first untethering surgery and were followed for > 100 months were retrospectively analyzed. The surgery was performed with the partial removal technique. Pre- and postoperative clinical and radiological data were reviewed to analyze the outcomes of surgery and identify potential risk factors for ReTCS. RESULTS: During follow-up, 12 patients experienced neurological deterioration due to ReTCS. The overall 10-year and 15-year progression-free survival rates were 82.3% and 75.1%, respectively. On univariate analysis, a lipoma type of lipomyelomeningocele (OR 11, 95% CI 2.50-48.4; p = 0.0014), patient age at the time of surgery (OR 0.41, 95% CI 0.14-1.18; p = 0.0070), and the mean patient growth rate after surgery (OR 2.00, 95% CI 1.12-3.41; p = 0.0040) were significant factors associated with ReTCS. Cox proportional hazard models showed that a lipoma type of lipomyelomeningocele (HR 5.16, 95% CI 1.54-20.1; p = 0.010) and the mean growth rate after surgery (HR 1.88, 95% CI 1.00-3.50; p = 0.040) were significantly associated with the occurrence of ReTCS. CONCLUSIONS: More complex lesions and a high patient growth rate after surgery seemed to indicate increased risk of ReTCS. Larger prospective studies and registries are needed to define the risks of ReTCS more adequately.

Preoperative Risks of Cerebral Infarction in Pediatric Moyamoya Disease.

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 months­17 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, 0.57­0.82]; P<0.0001) and the magnetic resonance angiography (MRA) score (OR, 2.29 [95% CI, 1.40­3.75]; P=0.001). Univariate analysis showed that risk factors for infarction while waiting for surgery were age at diagnosis (OR, 0.61 [95% CI, 0.46­0.80]; P<0.0001), the MRA score (OR, 1.75 [95% CI, 1.26­2.41]; P=0.0003), and onset of infarction (OR, 40.4 [95% CI, 5.08­322.3]; 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.

Reconversion to ventriculoperitoneal shunt following ventriculoatrial shunt malfunction in children.

PURPOSE: To analyze the long-term efficacy of the ventriculoatrial shunt (VAS) in pediatric patients with hydrocephalus, focusing on the atrial catheter and suitable revision procedures of the distal catheter following VAS malformation performed at our institution. METHODS: The authors retrospectively analyzed data of 28 pediatric patients under the age of 10 years who were treated with VAS for hydrocephalus and who had a follow-up period of at least 5 years. RESULTS: A total of 42 atrial tube revision procedures were performed in 28 patients during the study period. The median atrial tube survival time due to atrial tube obstruction was 2.32 years (n = 31, range: 0.4-8.08 years). Atrial tube survival time was shorter in younger children (p < 0.0001) and in children who were shorter in height (p = 0.0001). As a revision procedure following atrial tube malfunction, 22 (78.6%) out of the 28 patients who had an inserted VAS had the VAS reconversion into a VPS at the last follow-up. CONCLUSIONS: VAS can be a useful alternative to VPS, but it requires frequent atrial tube revisions, especially in younger children. Reconversion to VPS after VAS malfunction is a reasonable option and is associated with longer shunt survival time despite its previously observed difficulties.

Progressive Hydrocephalus after Endoscopic Third Ventriculostomy in Pediatric Patients with Blake's Pouch Cyst.

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.

Prosthesis Used in Microvascular Decompressions: A Multicenter Survey in Japan Focusing on Adverse Events.

OBJECTIVE: To investigate the characteristics of materials used as prostheses for microvascular decompression surgery (MVDs) in Japan and their possible adverse events (AEs) to determine preferable materials for MVDs. METHODS: A questionnaire was sent to all members of the Japanese Society for MVDs, and answers were obtained from 59 institutions. RESULTS: Among a total of 2789 MVDs, 1088 operations for trigeminal neuralgia, 1670 for hemifacial spasm, and 31 others, including 117 reoperations, were performed between April 2011 and March 2014. Nonabsorbable material was used in 96.5% of MVDs, including polytetrafluoroethylene (PTFE) (80.5%), polyurethane (11.9%), expanded PTFE (2.1%), and silk thread (1.47%). The use of absorbable materials, including fibrin glue (87.5%), cellulose (13.5%), gelatin (4,77%), and collagen (1.76%), was reported. The major combinations were PTFE with fibrin glue (58.7%) followed by PTFE alone (7.60%). Eighty-eight AEs in 85 (3.2%) cases were reported among 2672 first operations. AEs included 51 central nervous system dysfunctions, 15 wound infections/dehiscence, and 10 others, which were presumed to be related to the intraoperative procedure. Among relatively high-, moderate-, and low-volume centers, there were no significant differences in the frequency of AEs (P = 0.077). Tissue-prosthesis adhesion and/or granuloma formation were reported in 13 cases of 117 reoperations. The incidence of adhesion-related recurrence was 11.1% of all reoperations. CONCLUSIONS: The number of AEs was quite low in this survey, and intradural use of any prosthesis reported in this paper might be justified; however, further development of easily handled and less-adhesive prosthesis materials is awaited.

Progressive Cerebral Ischemia and Intracerebral Hemorrhage after Indirect Revascularization for a Patient with Cerebral Proliferative Angiopathy.

We previously reported a patient with cerebral proliferative angiopathy (CPA) who showed cerebral ischemia in resting and acetazolamide-stressed N-isopropyl-p-[123I] iodoamphetamine single-photon emission computed tomography (123I-IMP-SPECT). At onset, the patient was treated conservatively. However, during the 2 years following initial onset, his hemiparesis and aphasia had gradually aggravated and his IQ scores were markedly decreased. MRI revealed progressive vascular proliferation and brain atrophy. 123I-IMP-SPECT showed more severely impaired cerebral blood flow (CBF) and cerebrovascular reactivity over the affected hemisphere. We performed an indirect revascularization to augment CBF; however, his neurological deficits were not improved and new arteriovenous shunts via extracranial-intracranial bypass were developed, followed by an asymptomatic small intracerebral hemorrhage. There are no reports on CPA patients who have shown cerebral hemorrhage after indirect revascularization. Treatments for CPA are still challenging and controversial. Cases with severe stenosis of the proximal arteries may benefit from indirect revascularization. But indirect bypass should not be indicated for such patients without main arterial stenosis, even if they have persistent ischemia.

Posterior cerebral artery stenosis and posterior circulation revascularization surgery in pediatric patients with moyamoya disease.

OBJECTIVE Some pediatric patients with moyamoya disease (MMD) present with posterior cerebral artery (PCA) stenosis before and after anterior circulation revascularization surgery and require posterior circulation revascularization surgery. This study evaluated the factors associated with PCA stenosis and assessed the efficacy of posterior circulation revascularization surgery, including occipital artery (OA)-PCA bypass, in pediatric patients with MMD. METHODS The presence of PCA stenosis before and after anterior circulation revascularization surgery and its clinical characteristics were investigated in 62 pediatric patients (< 16 years of age) with MMD. RESULTS Twenty-three pediatric patients (37%) with MMD presented with PCA stenosis at the time of the initial diagnosis. A strong correlation between the presence of infarction and PCA stenosis before anterior revascularization was observed (p < 0.001). In addition, progressive PCA stenosis was observed in 12 patients (19.4%) after anterior revascularization. The presence of infarction and a younger age at the time of initial diagnosis were risk factors for progressive PCA stenosis after anterior revascularization (p < 0.001 and p = 0.002, respectively). Posterior circulation revascularization surgery, including OA-PCA bypass, was performed in 9 of the 12 patients with progressive PCA stenosis, all of whom showed symptomatic and/or radiological improvement. CONCLUSIONS PCA stenosis is an important clinical factor related to poor prognosis in pediatric MMD. One should be aware of the possibility of progressive PCA stenosis during the postoperative follow-up period and consider performing posterior circulation revascularization surgery.

Minimally Invasive Surgical Approach to Filum Lipoma.

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.

Chemokine Ligand 5 (CCL5) Derived from Endothelial Colony-Forming Cells (ECFCs) Mediates Recruitment of Smooth Muscle Progenitor Cells (SPCs) toward Critical Vascular Locations in Moyamoya Disease.

The etiology and pathogenesis of moyamoya disease (MMD) are still obscure. Previous studies indicated that angiogenic chemokines may play an important role in the pathogenesis of the disease. Recently, it was discovered that peripheral blood-derived endothelial colony-forming cells (ECFCs) and smooth muscle progenitor cells (SPCs) have defective functions in MMD patients. Therefore, the interaction of ECFCs and SPCs, the precursors of two crucial cellular components of vascular walls, with some paracrine molecules is an intriguing subject. In this study, co-culture of ECFCs and SPCs from MMD patients and healthy normal subjects revealed that MMD ECFCs, not SPCs, are responsible for the defective functions of both ECFCs and SPCs. Enhanced migration of SPCs toward MMD ECFCs supported the role for some chemokines secreted by MMD ECFCs. Expression arrays of MMD and normal ECFCs suggested that several candidate cytokines differentially produced by MMD ECFCs. We selected chemokine (C-X-C motif) ligand 6 (CXCR6), interleukin-8 (IL8), chemokine (C-C motif) ligand 2 (CCL2), and CCL5 for study, based on the relatively higher expression of these ligands in MMD ECFCs and their cognate receptors in MMD SPCs. Migration assays showed that only CCL5 significantly augmented the migration activities of SPCs toward ECFCs. Treatment with siRNA for the CCL5 receptor (CCR5) abrogated the effect, confirming that CCL5 is responsible for the interaction of MMD ECFCs and SPCs. These data indicate that ECFCs, not SPCs, are the major players in MMD pathogenesis and that the chemokine CCL5 mediates the interactions. It can be hypothesized that in MMD patients, defective ECFCs direct aberrant SPC recruitment to critical vascular locations through the action of CCL5.

Shunt placement after cyst fenestration for middle cranial fossa arachnoid cysts in children.

OBJECT Some pediatric patients with middle cranial fossa arachnoid cysts present with symptoms of increased intracranial pressure (ICP) and require shunt placement after a cyst fenestration. However, factors concerning increased ICP after fenestration followed by shunt placement have not been elucidated. This study evaluated factors that are associated with shunt placement following cyst fenestration in pediatric patients with middle cranial fossa arachnoid cysts. METHODS Twenty-six pediatric patients with middle cranial fossa arachnoid cysts who were surgically treated at a single institution between 2004 and 2013 were retrospectively identified. The surgical indications for middle cranial fossa arachnoid cysts were as follows: 1) arachnoid cysts associated with symptoms such as headache and abnormally enlarging head circumference; 2) progressively expanding arachnoid cysts; and 3) large arachnoid cysts such as Galassi Type III. A cyst fenestration was performed as a first-line treatment, and shunt placement was required if symptoms associated with increased ICP were found following fenestration. The risk factors evaluated included age, sex, presenting symptoms, the presence of head enlargement, progressive cyst expansion, and subdural hematoma/hygroma. RESULTS Four patients (15.4%) required shunt placement after cyst fenestration. Younger age, abnormal head enlargement, and progressive cyst expansion before fenestration were significantly associated with the need for shunt placement following fenestration. Arachnoid cysts decreased in size in 22 patients (84.6%) after fenestration and/or shunt placement. The presence of symptoms was not associated with postoperative cyst size in this study. CONCLUSIONS In this study, younger age, abnormal head enlargement, and progressive cyst expansion were risk factors for shunt placement after cyst fenestration in pediatric patients with middle cranial fossa arachnoid cysts. It is important to consider that cyst fenestration may not be effective because of a latent derangement of CSF circulation in patients with these risk factors.

Awareness of folic acid use increases its consumption, and reduces the risk of spina bifida.

The majority of neural tube defects were believed to be folic acid (FA)-preventable in the 1990s. The Japanese government recommended women planning pregnancy to take FA supplements of 400 µg/d in 2000, but the incidence of spina bifida has not decreased. We aimed to evaluate the OR of having an infant with spina bifida for women who periconceptionally took FA supplements and the association between an increase in supplement use and possible promoters for the increase. This is a case-control study which used 360 case women who gave birth to newborns afflicted with spina bifida, and 2333 control women who gave birth to healthy newborns during the first 12 years of this century. They were divided into two 6-year periods; from 2001 to 2006 and from 2007 to 2012. Logistic regression analyses were conducted to compute OR between cases and controls. The adjusted OR of having an infant with spina bifida for supplement users was 0.48 in the first period, and 0.53 in the second period. The proportion of women who periconceptionally consumed supplements significantly increased from 10 % in the first period to 30 % in the second period. Awareness of the preventive role of FA was a promoter for an increase in supplement use, and thus an FA campaign in high school seems rational and effective. The failure of the current public health policy is responsible for an epidemic of spina bifida. Mandatory food fortification with FA is urgent and long overdue in Japan.

Efficacy of superficial temporal artery-middle cerebral artery double anastomoses in a patient with rapidly progressive moyamoya disease: case report.

Moyamoya disease can be associated with a rapidly progressive course in young patients. This report describes a patient with moyamoya disease who experienced rapid disease progression, resulting in cerebral infarction and a wide area of diminished cerebral perfusion. Double superficial temporal artery (STA)-middle cerebral artery (MCA) anastomoses were utilized to immediately increase cerebral perfusion in the affected area. This case involved a 5-year-old girl who had been diagnosed with moyamoya disease and had undergone STA-MCA anastomosis with indirect bypass in the right hemisphere at the age of 3. At the time of presentation, magnetic resonance (MR) imaging showed cerebral infarction at the left frontal lobe, and MR angiography showed rapidly progressive narrowing of the left MCA that had not been present 3 months prior. N-isopropyl-p-[I123] iodoamphetamine single-photon emission computed tomography (IMP-SPECT) showed markedly decreased uptake in the left hemisphere. She underwent emergent STA-MCA double anastomoses with indirect bypass on the left side. IMP-SPECT showed marked increase in uptake in the left hemisphere. The anterior cerebral artery (ACA) territory adjacent to the cerebral infarction also showed increased uptake on the SPECT. Postoperatively, there were no clinical or radiographic indications of ischemic or hemorrhagic complications. Double anastomoses are effective in quickly and significantly increasing blood flow. The postoperative course in this case was uneventful. Double anastomoses are a surgical option for patients with moyamoya disease who show rapid disease progression, even in those in the acute phase of cerebral infarction.

123I-IMP-SPECT in a patient with cerebral proliferative angiopathy: a case report.

Cerebral proliferative angiopathy (CPA) is a new clinical entity demonstrating a diffuse network of densely enhanced vascular abnormalities with intermingled normal brain parenchyma and is distinguishable from classical arteriovenous malformations by specific clinical and imaging markers. However, the pathophysiological nature of this disease is unclear, and there is no consensus on the treatment. We describe cerebral perfusion abnormalities in a patient with CPA by using N-isopropyl-p-[123I] iodoamphetamine single-photon emission computed tomography (123I-IMP-SPECT) and perfusion-weighted magnetic resonance imaging. The patient, a 13-year-old boy, had reversible focal neurological deficits unrelated to cerebral hemorrhage. 123I-IMP-SPECT at resting state showed preserved uptake within the vascular lesion, yet lower uptake in the area adjacent to the lesion. In addition, acetazolamide-stressed 123I-IMP-SPECT exhibited severely impaired cerebrovascular reactivity over the affected hemisphere, suggesting that his focal neurological deficits were related to the cerebral ischemia. The perfusion abnormalities on 123I-IMP-SPECT in a CPA patient have never been previously reported. The concept of vascular malformation-related hypoperfusion is discussed.

Surgical treatment of scaphocephaly with sinus pericranii.

A 1-year-old female was admitted with a subcutaneous, pulsatile soft mass in the midline parietal region, and abnormal head shape. Fundus examination showed papilledema, suggesting elevated intracranial pressure. Radiological findings showed sagittal suture craniosynostosis with sinus pericranii. Magnetic resonance venography showed that the drainage through the sinus pericranii was not essential for the venous outflow from the brain. The patient underwent surgical resection of the sinus pericranii and total cranial remodeling. Ligation of the stalk-like orifice attached to the superior sagittal sinus with resection of the sinus pericranii and total cranial reconstruction were performed concurrently. The postoperative course was uneventful, and her papilledema resolved. No recurrence of the sinus pericranii has occurred for 3 years. This case describes a unique one-staged operation to treat sinus pericranii with sagittal suture craniosynostosis.