Longstanding overt ventriculomegaly diagnosed in adolescents, not adults: a pediatric case report.

BACKGROUND: Longstanding overt ventriculomegaly in adults (LOVA) is a new form of progressive hydrocephalus characterized by onset in early childhood and gradual progression into adulthood. Patients with LOVA are usually asymptomatic in childhood. The diagnosis of LOVA in adolescence has not been reported. CASE REPORT: A patient with macrocephaly and mild ventriculomegaly from infancy developed headache exacerbation and cognitive dysfunction at the age of 11 years. Brain magnetic resonance imaging showed mild tri-ventriculomegaly with no radiological aggravation compared to imaging at the age of 8 years. No papilledema was observed. Drainage of 15 ml of spinal fluid via a lumbar puncture relieved the headache and cognitive dysfunction. Based on repeated improvements in cognitive function and headaches after spinal fluid drainage, we diagnosed the patient with LOVA with symptom onset in early adolescence. A ventriculoperitoneal shunt was placed, and the headaches disappeared completely. The full-scale intellectual quotient, verbal comprehension, and working memory improved significantly. CONCLUSIONS: LOVA may manifest as early as adolescence. The clinical presentation, age, clinical, radiological features, and management vary, and a spinal tap exam is useful for diagnosing LOVA, even in children. The spinal tap exam may be indicated in children with longstanding ventriculomegaly and deteriorating neurological symptoms to diagnose this "treatable intellectual disability."

A neonatal purely prepontine arachnoid cyst: a case report and review of the literature.

Purely prepontine arachnoid cysts not extending into the suprasellar region in neonates are rare. Herein, we report a purely prepontine arachnoid cyst in a neonate which caused central apnea and was successfully treated with microscopic cyst fenestration and C1 laminectomy.

[Intrathecal Baclofen Therapy for Spastic Paralysis].

Intrathecal baclofen therapy(ITB)is a recognized treatment for spastic paralysis. Direct injection of baclofen into the intrathecal space through a catheter from a battery-loaded pump implanted in the abdomen allows effective administration of the drug at much lower doses than oral administration. ITB therapy is indicated for patients with severe spastic paraparesis that do not respond sufficiently to first-line medical therapy or in whom the side effects complicate treatment. A pump implantation is considered in cases where spasticity is improved after intrathecal infection of a small amount of baclofen. Postoperative complications include CSF leakage, infection and device malfunction. Postoperatively, the patient requires baclofen refills every 2-3 months and pump replacement surgery every 6-7 years. Currently, ITB therapy is not widespread in Japan, although further popularization is expected.

Endoscopic third ventriculostomy and cerebrospinal fluid shunting for pure communicating hydrocephalus in children.

PURPOSE: Recent reports regarding endoscopic third ventriculostomy (ETV) for pediatric hydrocephalus revealed that ETV could avoid cerebrospinal fluid (CSF) shunting in certain types of hydrocephalus. However, the effectiveness of ETV for "pure" communicating hydrocephalus that has no obstruction through CSF pathway is still unknown. In this study, we report clinical outcome of ETV and CSF shunting for communicating hydrocephalus and discuss the efficacy of ETV for pure communicating hydrocephalus. METHODS: Children less than 15 years old who underwent ETV or CSF shunting for communicating hydrocephalus were retrospectively reviewed. The absence of obstruction through CSF circulation was confirmed by CT cisternography or cine-contrast image in MRI. RESULTS: Sixty-three patients (45 CSF shunting and 18 ETV) were included. The mean follow-up period was 6.1 years. The success rate was 60% in CSF shunting and 67% in ETV at the last visit (p = 0.867). Normal development was observed in 24 patients (53%) in CSF shunting and 12 patients (67%) in ETV (p = 0.334). There was a significant difference in the mean time to failure (CSF shunting: 51.1 months, ETV 3.6 months, p = 0.004). The factor that affected success rate in ETV was the age at surgery (success 21.6 months, failure 4.4 months, p = 0.024) and ETV success score (success 66.7, failure 50.0, p = 0.047). CONCLUSION: Clinical outcomes of ETV were not inferior to those of CSF shunting in patients with communicating hydrocephalus. Further studies is required to elucidate to establish the consensus of ETV as a treatment option for communicating hydrocephalus.

Infantile intraorbital Ewing's sarcoma: case report and review of the literature.

We reported a 7-month-old female with intraorbital Ewing's sarcoma. Infantile Ewing's sarcoma is rare and its prognosis is poor. Ewing's sarcoma of orbital origin is even rare. There has been only 1 case of infantile intraorbital Ewing's sarcoma reported, and only 5 infantile primary orbital ESFTs (Ewing's sarcoma family of tumors) have been reported. Among these 5 cases, 2 infants who did not receive multimodal therapy died, whereas 3 who received multimodal therapy demonstrated long-term survival. The present case was also treated with multimodal therapy consisting of surgery, chemotherapy, and proton beam radiotherapy. There is no recurrence at 15 months follow-up. No specific treatment strategies have been established yet, and accumulation of cases is necessary. Ewing's sarcoma should be included in the differential diagnosis of infantile intraorbital tumors.

Surgical outcomes by sectioning a filum terminale in patients with terminal syringomyelia.

BACKGROUND: Although terminal syringomyelia suggests a tethered spinal cord, a consensus has still not been established for surgical indications, when there are no associated abnormalities or only minor associated abnormalities. We analyzed surgical outcomes of sectioning a filum terminale in patients with terminal syringomyelia to elucidate the significance of untethering surgery. METHODS: Fifty-seven pediatric patients with terminal syringomyelia who underwent untethering via sectioning the filum terminale between 2007 and 2018 were retrospectively analyzed. Postoperative outcomes of symptoms and MRI findings were evaluated. RESULTS: Of 57 patients, 40 had other associated abnormalities (25 with filar lipoma and 29 with low conus medullaris) that could be responsible for tethered cord. Nineteen patients had symptoms. The mean age at the time of surgery was 18 months (3-96 months). The mean follow-up period was 3.3 years (1-9 years). Preoperative symptoms improved in 9 patients (47%) after surgery. At 1 year after the surgery, the size of syringomyelia decreased in 19 (33%), increased in 4 (8%), and did not change in 34 (69%) patients. Of 28 patients who were followed up for more than 3 years, the size decreased in 17 (61%), increased in 1 (4%), and did not change in 10 (35%). CONCLUSION: When untethering was performed by sectioning a filum terminale, the size of terminal syringomyelia decreased in more than half of patients in the long term. Sectioning the filum terminale demonstrated the possibility of radiological and clinical improvement in patients with terminal syringomyelia.

Neoadjuvant chemotherapy for atypical teratoid rhabdoid tumors (AT/RTs).

PURPOSE: Atypical teratoid rhabdoid tumor (AT/RT) is a rare, clinically highly malignant tumor. The extent of the surgical resection may affect survival. However, it is often difficult to perform gross total resection (GTR) at an initial surgery because of the large tumor size and high vascularity. Neoadjuvant chemotherapy may reduce not only the size but also the vascularity. We report our experience, review the literature, and analyze its effectiveness. METHODS: A retrospective chart review of patients who underwent neoadjuvant chemotherapy and second-look surgery was performed. Demographic data, treatment courses, changes in tumor after the chemotherapy, extent of resection and estimated blood loss (EBL) during the second-look surgery, and outcome of each children were evaluated. RESULTS: There are 4 cases. The average age at diagnosis was 13.3 months (2-30 months). Two to 4 courses of neoadjuvant chemotherapy were performed. MRI after the chemotherapy showed reduction of tumor volume, and tumor vascularity at the second-look surgery decreased in all cases. GTR was achieved in 3 cases, and NTR in 1 case. The mean EBL/estimated blood volume (EBL/EBV) was 21.3% (1.5-39%). The mean follow-up period was 23 months (2-48 months). At the last follow-up, 2 patients were alive without recurrence of the tumor (the follow-up periods were 48 and 16 months). CONCLUSIONS: Neoadjuvant chemotherapy for AT/RTs might reduce both tumor size and vascularity, which enabled the maximal tumor resection. It may contribute to improve the prognosis of AT/RT through facilitating the tumor resection.

Radiologically occult cervical intradural dermal sinus tract: a case report and review of literature.

We report the unusual case of a 7-month-old girl presenting with congenital cervical dermal sinus tract in which the intradural tract was not detected on preoperative imaging and was identified intraoperatively. Considering possible devastating sequelae of infection, excision of dermal sinus tract might be justified even in the case with radiologically undetected intradural tract.

Pediatric nodular fasciitis at the roof of the optic canal causing decreased vision: case report and review of the literature.

We present a 3-year-old girl with decreased visual acuity of the left eye. Radiological studies revealed a mass lesion at the roof of the left optic canal with bony erosion, which compressed the left optic nerve. Gross total resection of the mass and decompression of the optic canal were performed. Histopathological study was consistent with nodular fasciitis. This is the first report of nodular fasciitis at the roof of the optic canal in a young child. Nodular fasciitis compressing the optic nerve should be included in differential diagnoses of optic neuropathy in young children.

Pediatric meningiomas: a report of 5 cases and review of literature.

PURPOSE: The purpose of this paper is to provide novel insight into the rare pediatric meningiomas. METHODS: We retrospectively analyzed pediatric surgical cases of meningioma during 2002 to 2017 in our institution. The preoperative, intraoperative, and the postoperative status were collected to find any unique features that has not reported in the past. RESULTS: Nine surgeries out of 5 patients were identified. The mean age was 7 years old (range 1-14 years old). Four patients were females. The mean tumor diameter was 52 mm (range 23-81 mm). The tumor locations were optic nerve sheath, Sylvian fissure, parasagittal, trigone of the lateral ventricle, and cerebellopontine angle. The Sylvian fissure meningioma without dural attachment (MWODA) was found in a 15-month-old female. A relapsed parasagittal meningioma showed regression in histological grade and residual tumor demonstrated spontaneous regression. In the initial surgeries, Simpson grade 1 resection was achieved in 2 cases. The pathological diagnoses were 1 meningothelial, 1 metaplastic, 2 atypical, and 1 clear cell meningiomas. The mean postoperative follow-up period was 71 months. Three patients experienced recurrence of the tumor. At the latest follow-up, all patients were free of radiological tumor recurrence or regrowth with a mean follow-up of 4 years (range 1-6.9 years). All patients were in the modified Rankin scale of 0-1. CONCLUSIONS: MWODA is not considered to be rare in pediatric meningioma and should be included in the differential diagnosis. We presented a histologically regressed relapsed meningioma, which spontaneously regressed after subtotal resection. In the case of recurrent meningioma, surgical resection and adjuvant radiation therapy could be effective for long-term control of the tumor.

Pediatric intracranial lower cranial nerve schwannoma unassociated with neurofibromatosis type 2: case report and review of the literature.

INTRODUCTION: Pediatric schwannomas are rare, and most of them are associated with neurofibromatosis type 2 (NF2) and usually located in the vestibular nerve. Herein, we present the first pediatric case of intracranial schwannoma derived from the IX/X nerve complex unrelated to NF2. CASE REPORT: The patient was a 9-year-old boy who presented with a 3-month history of headache and nausea. There was no family history of NF2. Imaging studies revealed a cystic lesion with enhanced wall in the left cerebellomedullary fissure. During the operation, the IX/X nerve complex was strongly adhered to the tumor at the jugular foramen. The tumor was totally excised, and the postoperative MRI demonstrated no residual tumor. Histopathological diagnosis was schwannoma. Genetic analysis revealed no mutation associated with NF2 and schwannomatosis. CONCLUSION: We reported the first case of pediatric lower cranial nerve schwannoma which was not associated with NF2. The schwannoma should be included as differential diagnosis of pediatric posterior fossa tumors.

Long-term outcome and neuroradiologic changes after multiple hippocampal transection combined with multiple subpial transection or lesionectomy for temporal lobe epilepsy.

OBJECTIVE: Multiple hippocampal transection (MHT) is a surgical procedure developed to avoid postoperative memory decline. Its efficacy has been documented in only a few small series with relatively short observation periods. We prospectively evaluated the long-term seizure and cognitive outcomes of MHT combined with multiple subpial transection or lesionectomy (MHT + MST/L). Moreover, we quantitatively evaluated the structural and metabolic neuroradiologic changes after the procedure to elucidate the anatomofunctional correlates of memory preservation. METHODS: Twenty-four patients underwent MHT + MST/L for treatment of drug-resistant mesial temporal lobe epilepsy (mTLE) and were followed for more than 5 years. Indications for the procedure were the following: (1) verbally dominant-sided surgery in patients with a radiologically normal hippocampus or normal/near normal memory, and (2) surgery for patients with concomitant epileptic activity on the contralateral side, that is, when the surgery was considered a high risk for severe postoperative memory decline. Seizure outcome was evaluated using Engel's classification 1, 2, and 3 years after surgery, and at the last visit (LV). Three subgroups were evaluated as well: magnetic resonance imaging (MRI) negative (MN), hippocampal sclerosis (HS), and normal hippocampus with extrahippocampal lesion (NHEL). The long-term cognitive outcome was followed through to LV in patients who underwent verbally dominant-sided surgery. Hippocampal volume (HV), diffusion tensor parameters (DTP), and glucose utilization (GU) were determined from MRI and fluorodeoxyglucose-positron emission tomography (FDG-PET) studies performed before and >6 months after surgery. RESULTS: Whereas the rate of Engel class I as a whole was 71% at 1 year and 67% at LV, the rates in the MN, HS, and NHEL groups were 60%, 67%, and 100% at 1 year, respectively, and 70%, 56%, and 80% at LV, respectively. Memory indices after verbally dominant-sided surgery transiently declined at 1 month but recovered to and remained at the preoperative level through LV. The HV, DTP of the fornix, and GU of the temporal lobe on the treated side showed pathologic changes even when the transiently declined memory indices had recovered to the preoperative level. SIGNIFICANCE: The long-term outcome for complex partial seizures after MHT + MST/L was comparable to that seen after anterior temporal lobectomy. The long-term cognitive outcome was favorable, even for patients with a high risk of severe postoperative memory decline. MHT + MST/L may be a treatment option for mTLE in which resective surgery carries a risk of postoperative memory decline, particularly in patients without MRI lesion. A discrepancy between the preserved memory and the pathologic neuroradiologic changes indicates the necessity for further studies including functional MRI.

Cerebrospinal fluid collections in sagittal suture synostosis.

PURPOSE: The premature fusion of the sagittal suture (scaphocephaly) may be associated with intracranial cerebrospinal fluid (CSF) dynamics alterations resulting in pericerebral effusion (PE) and/or ventriculomegaly. However, the etiology and development of such collections are not fully elucidated. The aims of this study are to clarify the characteristics of intracranial CSF collection in scaphocephaly, consider the role of surgery, and analyze the prognostic factor on CSF dynamics. METHOD: The pre- and postoperative cerebral imaging of 63 children (48 boys, 15 girls) with scaphocephaly who underwent craniotomy with decompression of the superior sagittal sinus (SSS) before 6 months of age between 2008 and 2015 were retrospectively studied. The PE was compared to the estimated value corrected for age (normal control). Evans' index (EI) was calculated. Correlations among these values and indices were analyzed. RESULT: Preoperatively, 36 of 63 patients (57 %) had larger PE than expected, and 36 of 63 patients (57 %) had pathological EI (EI > 0.3), and among them, 26 (46 %) had both. Patients with large PE tended to have pathological EI (p = 0.005). The PE and the EI were significantly decreased postoperatively (p < 0.001). The PE decreased faster than the expected natural course in 59 of 63 patients (92 %) postoperatively. CONCLUSION: The precise comparison with estimated PE prevalence corrected for age showed that PE can be found in more than half of the children with scaphocephaly, associated with ventriculomegaly. Surgery has a significant efficacious role in decreasing those CSF collections. The long-term significance of such CSF collections in scaphocephaly is still to be elucidated.

Spinal lipoma of the filum terminale: review of 174 consecutive patients.

PURPOSE: Spinal lipoma of the filum terminale (LFT) is a congenital lumbosacral anomaly that can cause tethered cord syndrome. Purposes of this study are to clarify preoperative characteristics of LFT, to elucidate surgical effects, and to discuss the rationale of prophylactic surgery for LFT. METHODS: Medical data of 174 children (2008-2014) who underwent section of LFT were prospectively recorded for prevalence of symptoms, skin stigmas, and associated malformations, motivator of diagnosis, conus level, and surgical outcome. Mean age at surgery was 4.1 ± 4.2 years (37 days to 17.7 years). RESULTS: Ninety-four children (54.0 %) had skin stigmas and 60 (34.5 %) had certain perineal malformations. Seventy-nine children (45.4 %) were symptomatic. The most common motivator for diagnosis was skin stigmas (44.3 %), followed by associated malformations (33.3 %), and symptoms (20.1 %). The age at surgery was significantly older in symptomatic patients than in asymptomatic patients (p < 0.001). Surgery improved symptoms in 50 % of patients at 2.1-year follow-up period. Of 85 asymptomatic patients, all except one remained asymptomatic postoperatively and none of the symptomatic patients deteriorated. The presence of associated malformations and the conus level did not affect surgical outcome. Postoperative complications developed in nine patients (5.2 %): seven transient local problems, one definitive urological deterioration, and one transient respiratory problem. CONCLUSIONS: Surgery for LFT was a simple and safe procedure. It improved half of symptomatic patients and stopped the deterioration of the others. Even if only one of the asymptomatic patients deteriorated at maximum follow-up, the role of prophylactic surgery remains a point of discussion.

Two Cases of Children With Black Hairy Tongue and Tooth Discoloration Caused by Antibacterial Agents.

Black hairy tongue (BHT) is a lesion in which the filiform papillae of the tongue are significantly extended by hyperkeratosis, thereby giving the tongue a hairy appearance. Here, we report two rare cases of children with BHT and tooth discoloration caused by antimicrobial agents. Case 1: A four-year-old female patient received intravenous linezolid after spinal surgery, and BHT developed on day eight of treatment. Subsequently, the patient developed teeth discoloration. Linezolid was continually administered for 50 days, and BHT and teeth discoloration improved 10 days after the end of linezolid treatment. Case 2: A two-year-old male patient with a brain abscess received intravenous meropenem and vancomycin. On the fourth day of treatment, BHT developed, and teeth discoloration was subsequently observed. Antibiotic therapy was continued for 82 days, and BHT and tooth discoloration improved 20 days after the treatment was discontinued.

Spinal Subarachnoid Hematoma After Cardiac Angiography in an Infant: A Case Report.

Spinal subarachnoid hematoma may result in sequelae such as bilateral lower extremity paralysis and vesicorectal disturbances. Although spinal subarachnoid hematoma is rare in infants, early intervention has been suggested to improve neurological prognosis. Therefore, clinicians are encouraged to make early diagnosis and surgical intervention. A 22-month-old boy was prescribed aspirin for a congenital heart disease. A routine cardiac angiography was performed under general anesthesia. Fever and oliguria developed on the next day, followed by flaccid paralysis of the lower limbs four days later. Five days later, he was diagnosed with spinal subarachnoid hematoma and associated spinal cord shock. Even after emergent posterior spinal decompression, hematoma removal, and rehabilitation, the patient remained with bladder rectal disturbance and flaccid paralysis of both lower limbs. Diagnosis and treatment of this case were delayed mainly because of his difficulty to complain of back pain and paralysis. The neurogenic bladder was one of the first neurological symptoms in our case, so it may be important to consider spinal cord involvement in infants with bladder compromise. Risk factors for spinal subarachnoid hematoma in infants are largely unknown. The patient had undergone a cardiac angiography the day before the onset of the symptoms, which may be related to subarachnoid hematoma. However, similar reports are scarce, with only one case of spinal subarachnoid hematoma reported in an adult following cardiac catheter ablation. Accumulation of evidence regarding risk factors for subarachnoid hematoma in infants is warranted.

Two cases of spontaneous temporal encephalocele.

This is a report of two cases of spontaneous temporal encephalocele: one was anteroinferior and presented with epilepsy; the other was posteroinferior and presented with facial neuritis and labyrinthitis. Spontaneous temporal encephalocele is relatively rare and apparently not familiar to a majority of primary physicians. It may present with a variety of symptoms according to its anatomical location, including cerebrospinal fluid fistulas, recurrent meningitis, chronic otitis media, hearing loss, facial nerve palsy and medically intractable epilepsy. Attention should be paid to this disease entity, as it is easily overlooked in imaging studies and can leave serious neurological deficits.

Is the Postoperative Horizontal Decubitus Position Following Transection of a Tight Filum Terminale in Pediatric Patients Necessary? - A Retrospective Cohort Study.

After untethering surgery of a tethered spinal cord of a tight filum terminale, patients are usually kept in the horizontal decubitus position to prevent cerebrospinal fluid (CSF) leakage. However, the optimal period for keeping these patients in this position has not been established yet. Surgical results in two groups of pediatric patients with a tight filum terminale were retrospectively analyzed. Group A was maintained in the horizontal decubitus position for 72 h and group B was managed without being kept in this position postoperatively. A total of 313 patients underwent sectioning of a tight filum terminale. Of these patients, 144 were maintained horizontally for 72 h postoperatively (group A) and 169 were managed without this position (group B). Among the patients who were maintained horizontally for 72 h, one (0.7%) developed pseudomeningocele. No patients experienced CSF leakage in this group. Among the patients who were not horizontal, one (0.6%) developed CSF leakage and one (0.6%) developed pseudomeningocele. Maintaining patients without restriction of their position does not appear to change the rate of postoperative CSF leakage or pseudomeningocele. This suggests that maintaining patients horizontally after transection of a tight filum terminale is not necessary for preventing CSF leakage.

Basal encephalocele: surgical strategy and functional outcomes in the Tokyo experience.

OBJECTIVE: The basal encephalocele (BEC) is the rarest form of encephalocele, with an incidence of about 1/35,000 live births. The incidence of its subtype, sphenoidal BEC, is even lower at about 1/700,000 live births. The aim of this study was to propose the optimal surgical approach to repairing BEC, with special attention to the reconstruction of the skull base bone defect. METHODS: Fourteen consecutive pediatric patients with BEC who underwent surgical repair between March 2004 and March 2020 (10 boys and 4 girls, age 25 days to 7 years, median age 4 months) were enrolled. The follow-up period of the surviving patients ranged from 53 to192 months (mean 119.8 months). The patient demographics, BEC subtypes, preoperative clinical condition, radiographic findings, surgical procedures, and postoperative course were retrospectively analyzed. RESULTS: There were 4, 8, and 2 cases of sphenoidal BEC, sphenoethmoidal BEC, and ethmoidal BEC, respectively. The size of the bone defect was small in 3 patients, medium in 7, and large in 4 patients. All the patients with sphenoethmoidal and ethmoidal BEC showed associated congenital anomalies other than cleft palate. In total, 25 operations were performed. Two patients underwent multiple operations, whereas the remaining 9 patients received only 1 operation. The transoral transpalatal approach was the initial procedure used in all 14 patients. The transfrontobasal approach was applied as an additional procedure in 2 patients and as part of a 1-stage combined operation in 2 patients. Autograft bone alone was used for skull base reconstruction in 17 early operations. A titanium mesh/plate was used in the remaining 8 operations without any perioperative complications. All BECs were successfully repaired. Three patients died during the clinical course due to causes unrelated to their surgery. All but one of the surviving patients started growth hormone replacement therapy before school age. CONCLUSIONS: Based on the authors' limited experience, the key to successful BEC repair involves circumferential dissection of the BEC and a firm reconstruction of the skull base bone defect with a titanium plate/mesh. The transoral transpalatal approach is a promising, reliable procedure that may be used in the initial operation. When a cleft palate is absent, transnasal endoscopic repair is recommended. The transfrontobasal approach should be reserved for cases with a huge BEC and other anomalies. Long-term prognosis is apparently favorable in survivors.