Neurosurgical strategy based on the type of occult spinal dysraphism in omphalocele-exstrophy-imperforate anus-spinal defects complex: A review of 10 cases.

Background: Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a rare, life-threatening congenital malformation primarily treated with abdominogenital repair. The optimal indication and timing of neurosurgical interventions for the associated spinal cord lesions remains insufficiently studied. We reviewed spinal dysraphism in OEIS to evaluate the best timing for neurosurgical intervention.

Expanding Ventricular Diverticulum Overlying the Cerebral Hemisphere through an Open-Lip Schizencephalic Cleft: A Report of Two Pediatric Cases.

Introduction: Open-lip-type schizencephaly is characterized by trans-cerebral clefts filled with cerebrospinal fluid (CSF) between the subarachnoid space at the hemisphere surface and the lateral ventricles. Disorders related to CSF retention, including hydrocephalus and arachnoid cysts, have reportedly been associated with open-lip schizencephaly and have induced intracranial hypertension in some cases. However, detailed neuroimaging and surgical treatment findings have rarely been described.

Retained medullary cord and caudal lipoma with histopathological presence of terminal myelocystocele in the epidural stalk.

Background: The retained medullary cord (RMC), caudal lipoma, and terminal myelocystocele (TMCC) are thought to originate from the failed regression spectrum during the secondary neurulation, and the central histopathological feature is the predominant presence of a central canal-like ependyma-lined lumen (CC-LELL) with surrounding neuroglial tissues (NGT), as a remnant of the medullary cord. However, reports on cases in which RMC, caudal lipoma, and TMCC coexist are very rare.

Case Description: We present two patients with cystic RMC with caudal lipoma and caudal lipoma with an RMC component, respectively, based on their clinical, neuroradiological, intraoperative, and histopathological findings.

Severe type of segmental spinal dysgenesis with complete disconnection of the spinal cord and vertebra associated with open neural tube defect.

Background: Severe type of segmental spinal dysgenesis (SSD) is a rare and complex anomaly in which the spinal cord completely disconnects at the portion of the spinal dysgenesis. Although closed spinal dysraphisms have been associated with SSD, to the best of our knowledge, the association between open neural tube defect (ONTD) and SSD is significantly rare, with only one case being reported to date.

Case Description: We report a case of an infant with severe SSD and a disconnected spinal cord and spinal column at the thoracolumbar junction associated with myelomeningocele (MMC) in the lumbosacral region.

Ectopic dorsal root ganglion in cauda equina mimicking schwannoma in a child.

Background: A heterotopic dorsal root ganglion (DRG) is sometimes observed in the vicinity of dysplastic neural structures during surgery for open spinal dysraphism; however, it is rarely associated with closed spinal dysraphism. Distinguish from neoplasms by preoperative imaging study is difficult. Although the embryopathogenesis of a heterotopic DRG has been speculated to be migration disorder of neural crest cells from primary neural tube, its details remain unelucidated.

Histopathological presence of dermal elements in resected margins of neural structures obtained from initial repair surgery for myelomeningocele.

Background: Development of dermoid or epidermoid cysts in myelomeningocele (MMC) sites is generally thought to occur in a delayed fashion due to implantation of dermal elements during initial repair surgery. Another theory is that dermal and dermoid elements may already be present within dysplastic neural structures at birth.

Methods: We experienced histopathological presence of dermal elements in resected tissues at initial repair surgery in four out of 18 cases with MMC who required resection of parts or margins of the neural structures to perform cord untethering.

Refractory CSF leakage following untethering surgery performed 10 months after birth for enlarging terminal myelocystocele associated with OEIS complex.

Background: Terminal myelocystocele (TMC) is an occult spinal dysraphism characterized by cystic dilatation of the terminal spinal cord in the shape of a trumpet (myelocystocele) filled with cerebrospinal fluid (CSF), which herniates into the extraspinal subcutaneous region. The extraspinal CSF-filled portion of the TMC, consisting of the myelocystocele and the surrounding subarachnoid space, may progressively enlarge, leading to neurological deterioration, and early untethering surgery is recommended.

Case Description: We report a case of a patient with TMC associated with OEIS complex consisting of omphalocele (O), exstrophy of the cloaca (E), imperforate anus (I), and spinal deformity (S).

Retained Medullary Cord Associated with Terminal Myelocystocele and Intramedullary Arachnoid Cyst.

Introduction: The retained medullary cord (RMC) is a newly defined entity of closed spinal dysraphism that is thought to originate from regression failure of the medullary cord during the last phase of secondary neurulation. The terminal myelocystocele (TMC) is an unusual type of closed spinal dysraphism, characterized by localized cystic dilatation of the terminal part of the central canal that then herniates through a posterior spinal bifida. The co-occurrence of RMC and TMC is extremely rare.

Intramedullary abscess at thoracolumbar region transmitted from infected dermal sinus and dermoid through retained medullary cord.

Background: A retained medullary cord (RMC) is a relatively newly defined entity of closed spinal dysraphism that is thought to originate from regression failure of the medullary cord during secondary neurulation. A congenital dermal sinus (CDS) may provide a pathway for intraspinal infections such as repeated meningitis. Intramedullary abscesses are the rarest but most serious complication of a CDS.

Saccular Limited Dorsal Myeloschisis with Spinal Cord Deviation out of the Spinal Canal to the Sac.

Saccular limited dorsal myeloschisis (LDM) is characterized by a fibroneural stalk linking the saccular skin lesion to the underlying spinal cord. Since untethering surgery during the early postnatal period is often indicated to prevent sac rupture, saccular LDM should be distinguished from myelomeningocele (MMC) during the perinatal period. We treated two patients with the spinal cord deviation from the spinal canal to the sac, which mimicked a prolapse of the neural placode into the MMC sac.

Spinal cord deformity with aggravation of tethering in saccular limited dorsal myeloschisis during the first 2 months of life.

Background: Although the optimal timing of prophylactic untethering surgery for limited dorsal myeloschisis (LDM) with intact or subtle neurological findings diagnosed at birth remains undetermined, intentional delayed surgery is commonly used for flat and tail-like LDM. Conversely, for saccular LDM, early surgery is indicated during the postnatal period because it prevents rupture of the sac. We treated a saccular LDM patient, in whom intentional delayed surgery was selected because the sac was thickly covered with normal skin.

Surgical histopathology of a filar anomaly as an additional tethering element associated with closed spinal dysraphism of primary neurulation failure.

Background: Closed spinal dysraphism of primary neurulation failure could be associated with filar anomalies, such as filar lipoma or thickened and tight filum terminale (TFT), resulting from impaired secondary neurulation. Retained medullary cord (RMC) is a remnant of the cavitary medullary cord originating from the secondary neurulation failure. Some filar lipomas are known to contain a central canal-like ependyma-lined lumen with surrounding neuroglial tissues (E-LC w/NGT), that is, a characteristic histopathology of RMC.

Osteosarcoma of the temporal bone occurring 40 years after radiotherapy: A technical case report presenting resection of intra- and extracranial lesions followed by a one-stage reconstruction.

Background: Osteosarcoma (OS) is a malignant tumor of the bone, which rarely occurs in the head-and-neck regions as a primary or a secondary malignancy. Adequate surgical resection is currently the mainstay of treatment for head-and-neck OS; however, resection and reconstruction can be difficult because the anatomies of these regions are complex. We present a case of an OS arising from the temporal bone 40 years after radiation therapy, which was successfully treated with resection and a one-stage reconstruction using intraoperative tissue expansion technique.

Measuring Quality of Care for Ischemic Stroke Treated With Acute Reperfusion Therapy in Japan - The Close The Gap-Stroke.

Background: In Japan there is no consensus on how to efficiently measure quality indicators (QIs), defined as a standard of care, for acute ischemic stroke (AIS). Using information from a health insurance claims database and electronic medical records, we evaluated the feasibility and validity of measuring QIs for AIS patients who received intravenous recombinant tissue plasminogen activator (IV rt-PA) or endovascular therapy (EVT).

Methods and results: AIS patients receiving rt-PA or EVT between 2013 and 2015 were identified.

Temporal trends and geographical disparities in comprehensive stroke centre capabilities in Japan from 2010 to 2018.

Objectives: Comprehensive stroke centre (CSC) capabilities are associated with reduced in-hospital mortality due to acute stroke. However, it remains unclear whether there are improving trends in the CSC capabilities or how hospital-related factors determine quality improvement. This study examined whether CSC capabilities changed in Japan between 2010 and 2018 and and whether any changes were influenced by hospital characteristics.

Intraoperative Tissue Expansion Using a Foley Catheter for a Scalp Defect: Technical Note.

Background: Primary closure of the surgical wound during neurosurgical procedures is sometimes difficult because of limited ability to expand the scalp, or because the skin defect is large. Hence, our institution recently adopted the technique of intraoperative tissue expansion using a Foley catheter for these cases. We describe this easily accomplished, readily available, effective, economical technique and describe our experience performing the technique.

Effect of treatment modality and cerebral vasospasm agent on patient outcomes after aneurysmal subarachnoid hemorrhage in the elderly aged 75 years and older.

Objective: We sought to examine whether the effect of treatment modality and drugs for cerebral vasospasm on clinical outcomes differs between elderly and non-elderly subarachnoid hemorrhage (SAH) patients in Japan.

Methods: We analyzed the J-ASPECT Study Diagnosis Procedure Combination database (n = 17,343) that underwent clipping or coiling between 2010 and 2014 in 579 hospitals. We stratified patients into two groups according to their age (elderly [≥75 years old], n = 3,885; non-elderly, n = 13,458).

Effects of case volume and comprehensive stroke center capabilities on patient outcomes of clipping and coiling for subarachnoid hemorrhage.

Objective: Improved outcomes in patients with subarachnoid hemorrhage (SAH) treated at high-volume centers have been reported. The authors sought to examine whether hospital case volume and comprehensive stroke center (CSC) capabilities affect outcomes in patients treated with clipping or coiling for SAH.

Methods: The authors conducted a nationwide retrospective cohort study in 27,490 SAH patients who underwent clipping or coiling in 621 institutions between 2010 and 2015 and whose data were collected from the Japanese nationwide J-ASPECT Diagnosis Procedure Combination database.

Ruptured dermoid cyst of the conus medullaris in the myelomeningocele sac revealed at the initial repair surgery.

Background: Dermoid cysts in the myelomeningocele (MMC) site are thought to arise in a delayed fashion because of iatrogenic implantation of dermoid elements at the time of the initial repair surgery. However, there have been few reports on dermoid elements already present at birth.

Clinical Presentation: We report a patient, in whom dermoid cyst was located at dorsal aspect of the conus medullaris in the MMC sac.

National trends in outcomes of ischemic stroke and prognostic influence of stroke center capability in Japan, 2010-2016.

Background: Limited national-level information on temporal trends in comprehensive stroke center capabilities and their effects on acute ischemic stroke patients exists.

Aims: To examine trends in in-hospital outcomes of acute ischemic stroke patients and the prognostic influence of temporal changes in comprehensive stroke center capabilities in Japan.

Methods: This retrospective study used the J-ASPECT Diagnosis Procedure Combination database and identified 372,978 acute ischemic stroke patients hospitalized in 650 institutions between 2010 and 2016.