Fibrous hamartoma of infancy of the spinal cord resembling conus and filum, with a coexisting sacral dimple.

Fibrous hamartoma of infancy (FHI) is a rare benign soft tissue lesion of infants and young children. It usually occurs within the first 2 years of life at the superficial layer of the axilla, trunk, upper arm, and external genitalia. FHI in the central nervous system (CNS) is extremely rare. So far, only two spinal cord FHI cases have been reported. We present a case of a 1-month-old girl who presented with a skin dimple in the coccygeal area. Her MRI showed a substantial intramedullary mass in the thoracolumbar area with a sacral soft tissue mass and a track between the skin lesion to the coccygeal tip. Her normal neurological status halted immediate surgical resection. A skin lesion biopsy was first performed, revealing limited information with no malignant cells. A short-term follow-up was performed until the intramedullary mass had enlarged on the 5-month follow-up MRI. Based on the frozen biopsy result of benign to low-grade spindle cell mesenchymal tumor, subtotal resection of the mass was done, minimizing damage to the functioning neural tissue. Both the skin lesion and the intramedullary mass were diagnosed as FHI. Postoperative 5.5-year follow-up MRI revealed minimal size change of the residual mass. Despite being diagnosed with a neurogenic bladder, the patient maintained her ability to void spontaneously, managed infrequent UTIs, and continued toilet training, all while demonstrating good mobility and no motor weakness. This case is unique because the lesion resembled the secondary neurulation structures, such as the conus and the filum, along with a related congenital anomaly of the dimple.

Surgical treatment of interhemispheric arachnoid cysts.

OBJECTIVE: In children, interhemispheric arachnoid cysts (IHACs) are rare lesions often associated with corpus callosum dysgenesis. It is still controversial about surgical treatments for IHACs. We aim to report our experience with pediatric IHAC patients and evaluate surgical courses and neurological developments. METHODS: Pediatric IHACs treated between 2001 and 2021 were reviewed retrospectively. IHAC was observed until they represented rapid cyst enlargement or neurological symptoms. Cyst fenestration was done by microscope or endoscope, depending on the IHAC's location. Cyst size and corpus callosum dysgenesis were evaluated with neuroimaging. Neurological development was assessed from medical records at the last follow-up. RESULTS: Fifteen children received cyst fenestration surgery (mean age 11.4 months). Eleven patients (73.3%) under observation showed rapid cyst enlargement in a short period (median 5 months). Cysto-ventriculostomy (CVS) and cysto-cisternostomy (CCS) regressed the cyst size significantly (p = 0.003). The median follow-up duration was 51 months (range 14-178 months). Corpus callosum dysgenesis was observed in eleven patients (73.3%, complete = 5, partial = 6). Among eight patients (53.3%) having developmental delay, five patients (33.3%) showed speech delay, including one patient with intractable seizures. CONCLUSION: Pediatric IHACs frequently present within 1 year after birth, with rapid cyst enlargement. CVS and CCS were effective in regressing the cyst size. Corpus callosum dysgenesis accompanied by IHAC might have a risk of language achievement; however, development delay could rely on multifactorial features, such as epilepsy or other brain anomalies.

Intramedullary spinal capillary hemangioma with secondary neurulation defect in children.

Intramedullary spinal capillary hemangioma is a rare occurrence in pediatric patients, and only limited cases have been reported. This study presents the first two cases of spinal capillary hemangioma co-present with retained medullary cord and one case of spinal capillary hemangioma with lumbosacral lipomatous malformation. Previous literature on ten patients with this pathology was reviewed. We speculated pathogenesis, imaging features, and histopathologic findings of the disease.

Secondary Neurulation Defect: Terminal Myelocystocele, a Biological Leviathan.

Terminal myelocystocele (TMC) has been a puzzling entity of spinal dysraphism. It is found in the sacrococcygeal region usually forming a subcutaneous hump of various sizes. The wide variation of its morphology has been clarified by defining the essential and nonessential features as described in this chapter. Although it is not a common entity, TMC is attractive in that a highly plausible hypothesis on its pathoembryogenesis has been proposed based on observations on the secondary neurulation of the chick embryo. In this chapter, the embryology will be described, followed by the surgical strategy in accordance with the embryogenesis. The clinical features and prognosis will also be presented in detail.

Secondary Neurulation Defects: Retained Medullary Cord.

Retained medullary cord (RMC) is a defect resulting from impaired secondary neurulation. Intraoperatively, RMC is recognizable as an elongated cord-like structure caudal to the conus, that contains histologically confirmed neuroglial components and a lumen with an ependymal lining. It characteristically does not possess neurological function. This chapter aims to summarize (1) the mechanisms that lead to the occurrence of RMC; (2) the various forms of RMC, such as cystic RMC and 'possible RMC', and (3) the treatment strategies, especially untethering through limited exposure.

Myelomeningocele: the evolution of care over the last 50 years.

PURPOSE: Myelomeningocele (MMC) is one of the representative anomalies in the field of pediatric neurosurgery. During the 50 years of ISPN history, MMC had a tremendous changes in its incidence, clinical management and outcome with advanced understanding of its pathogenesis. We reviewed the changes in MMC during the period. METHODS: We reviewed the literature review and collected our experiences. RESULTS: During the 50 years, major changes happened in many aspects of MMC including incidence, pathoembryogenesis, folate deficiency, prevention, prenatal diagnosis, mode of delivery, treatment policy with ethical considerations, clinical treatment including fetal surgery, latex allergy, retethering, management outcome, multidisciplinary team approach, and socioeconomic and family issues. CONCLUSIONS: There was a great advance in the management and research of MMC during the 50 years. It is a monumental achievement of pediatric neurosurgeons and colleagues of the related fields.

Electrodiagnosis has a potential to identify neural damage in asymptomatic infants with closed spinal dysraphism.

PURPOSE: The presence and progression of symptoms is the basis for deciding to perform surgery in infants with closed spinal dysraphism (CSD); however, identifying symptoms could be limited, making it difficult to decide. This study investigated whether an electrodiagnostic study (EDS) can provide evidence of neural damage in asymptomatic infants with CSD. METHODS: The study group comprised infants with CSD suspected of having neural damage based on structural abnormalities in spinal ultrasound findings. The patients' medical records were reviewed retrospectively for their clinical presentation, neuroimaging findings, urodynamic study (UDS) results, EDS findings, and surgical status. RESULTS: Among 125 infants who underwent EDS and UDS, 117 (94%) had no clinical symptoms, except for cutaneous manifestations. Among these asymptomatic patients, 51 individuals (43.6%) had abnormal EDS findings; 33 subjects (28.2%) showed abnormal findings on EDS alone, while 37 (31.6%) on UDS alone, and 18 (15.4%) on both EDS and UDS. Chi-square test showed an opposite relationship between the two test results; when EDS was abnormal, UDS was often normal and vice versa (χ2 = 5.328, p = 0.021). In all cases with abnormal EDS, denervation potentials, such as fibrillation and positive sharp waves, were observed on needle electromyography. However, abnormal findings in the nerve conduction study were observed only in six cases. CONCLUSION: Subclinical neural damage was identified through EDS in asymptomatic infants with CSD. EDS could be necessary to determine whether follow-up monitoring only or surgical intervention is required for this patient group complementing UDS findings.

Electrodiagnostic findings of retethering in children with spinal dysraphism.

PURPOSE: Retethering of the cord can occur after the initial untethering surgery. Typical neurological manifestations indicative of cord tethering are often difficult to determine in pediatric patients. Patients who had a primary untethering operation are likely to present with some degree of neurological deficits from a previous tethering event, and urodynamic studies (UDSs) and spine images are frequently abnormal. Therefore, more objective tools to detect retethering are needed. This study sought to delineate the characteristics of EDS of retethering, and therefore, could support the diagnosis of retethering. METHODS: Among 692 subjects who had an untethering operation, data from 93 subjects who had been suspected of retethering clinically were retrospectively extracted. The subjects were divided into two groups, a retethered group, and a non-progression group, according to whether or not surgical interventions had been performed. Two consecutive EDSs, clinical findings, spine magnetic resonance imaging scans, and UDSs before the development of new tethering symptoms were reviewed and compared. RESULTS: In the electromyography (EMG) study, the appearance of abnormal spontaneous activity (ASA) in new muscles was prominent in the retethered group (p < 0.01). The loss of ASA was more pronounced in the non-progression group (p < 0.01). Specificity and sensitivity of EMG for retethering were 80.4 and 56.5%, respectively. In the nerve conduction study, the two groups did not show differences. The size of fibrillation potential was not different between the groups. CONCLUSIONS: To provide support for a clinician's decision on retethering, EDS could be an advantageous tool with high specificity when the results are compared to previous EDS results. Routine follow-up EDS post-operatively is recommended as a baseline for comparison at the time when retethering is clinically suspected.

The Outcomes of Endoscopic Suturectomy in Syndromic Craniosynostosis.

OBJECTIVE: Endoscopic suturectomy is a widely practiced surgical option for infants with craniosynostosis. But the efficacy and safety of the procedure remain unclear in syndromic patients. This study aims to evaluate the efficacy and safety of endoscopic suturectomy for patients with syndromic craniosynostosis. METHODS: From January 2013 to December 2020, 242 patients underwent endoscopic suturectomy at our institution. The surgical outcome was determined to be favorable or unfavorable based upon the necessity of an additional cranial surgery upon the last follow-up. First, we analyzed the outcomes of 26 syndromic craniosynostosis patients who have followed up for over a year. Second, we compared the outcomes between the syndromic (N=12) and nonsyndromic (N=11) patients with bilateral coronal synostosis who have followed up for over a year. RESULTS: Twenty-three out of 26 syndromic craniosynostosis patients (88%) showed favorable outcomes without significant complications. In the analysis for bilateral coronal synostosis patients, 11 of 12 syndromic patients (92%) presented favorable outcomes, and all nonsyndromic patients showed favorable outcomes. No significant differences were observed in various anthropometric indices (cranial index, intracranial volume, anterior cranial height, anterior cranial base length, and cranial height-length index) and surgical outcomes between syndromic and nonsyndromic groups. CONCLUSIONS: Endoscopic suturectomy has the potential to be a surgical option for syndromic craniosynostosis. Even for patients with unfavorable outcomes, endoscopic suturectomy could serve as a bridge treatment for infants to counter cranial deformation before additional extensive surgery.

Three-dimensional visualization of secondary neurulation in chick embryos using microCT.

BACKGROUND: Defects in secondary neurulation play an important role in neural tube defects. Researchers have investigated the processes of secondary neurulation and caudal body formation mainly by microscopic observations and molecular experiments. Although conventional histology is a powerful tool for observing the details of morphology, it has limitations in the presentation of gross three-dimensional (3D) configurations of small embryos. The goal of this study was to visualize secondary neurulation and related structures in chick embryos in Hamburger and Hamilton (HH) stages 10-22 using microCT. RESULTS: The gross morphology of the chick embryo of various developmental stages was well visualized using microCT. Also, the detailed structures of the caudal cell mass (CCM) were presented starting from HH stage 12 to stage 16. The spatiotemporal relationship of CCM with the floor plate of the neural tube and notochord was shown. The dynamic changes of the chordoneural hinge, the cavitation of the secondary neural tube, and the primitive streak were described throughout the early stages of secondary neurulation. CONCLUSIONS: By utilizing the advantages of the microCT technique, our study shed light on the secondary neurulation in early-stage chick embryos and this can be the 3D reference for related structures.

Neurotoxicity of phenylalanine on human iPSC-derived cerebral organoids.

Phenylketonuria (PKU) is a common genetic metabolic disorder that causes phenylalanine accumulation in the blood. The most serious symptoms are related to the brain, as intellectual disability, seizure, and microcephaly are commonly found in poorly treated PKU patients and the babies of maternal PKU. However, the mechanism of hyperphenylalaninemia on human neurodevelopment is still unclear. Here we utilized human induced pluripotent stem cell (iPSC)-derived cerebral organoids to investigate the neurotoxicity of hyperphenylalaninemia. Cerebral organoids at days 40 or 100 were treated with different concentrations of phenylalanine for 5 days. After phenylalanine treatments, the cerebral organoids displayed alterations in organoid size, induction of apoptosis, and depletion of neural progenitor cells. However, phenylalanine did not have an impact on neurons and glia, including astrocytes, immature oligodendrocytes, and mature oligodendrocytes. Remarkably, a reduction in the thickness of the cortical rosettes and a decrease in myelination at the intermediate zone were inspected with the elevated phenylalanine concentrations. RNA-seq of phenylalanine-treated organoids revealed that gene sets related to apoptosis, p53 signaling pathway, and TNF signaling pathway via NF-kB were enriched in upregulated genes, while those related to cell cycle and amino acid metabolism were enriched in downregulated genes. In addition, there were several microcephaly disease genes, such as ASPM, LMNB1, and CENPE, ranked at the top of the downregulated genes. These findings indicate that phenylalanine exposure may contribute to microcephaly, abnormal cortical expansion, and myelination lesions in the developing human brain.

Disorders of Secondary Neurulation: Suggestion of a New Classification According to Pathoembryogenesis.

Recently, advanced knowledge on secondary neurulation and its application to the clinical experience have led to the deeper insight into the pathoembryogenesis of secondary neurulation with new classifications of the caudal spinal dysraphic entities. Here, we summarize the dynamic changes in the concepts of disordered secondary neurulation over the last two decades. In addition, we suggest our new pathoembryogenetic explanations for a few entities based on the literature and the data from our previous animal research. Disordered secondary neurulation at each phase may cause various corresponding lesions, such as (1) failed junction with the primary neural tube (junctional neural tube defect and segmental spinal dysgenesis), (2) dysgenesis or duplication of the caudal cell mass associated with disturbed activity of caudal mesenchymal tissue (caudal agenesis and caudal duplication syndrome), (3) abnormal continuity of medullary cord to the surrounding layers, namely, failed ingression of the primitive streak to the caudal cell mass (myelomeningocele), focal limited dorsal neurocutaneous nondisjunction (limited dorsal myeloschisis and congenital dermal sinus), and neuro-mesenchymal adhesion (lumbosacral lipomatous malformation), and (4) regression failure spectrum of the medullary cord (thickened filum and filar cyst, retained medullary cord and low-lying conus, terminal myelocele, and terminal myelocystocele). It seems that almost every anomalous entity of the primary neural tube may occur in the area of secondary neurulation. Furthermore, the close association of the caudal cell mass with the activity of caudal mesenchymal tissue involves a wider range of surrounding structures in secondary neurulation than in primary neurulation. Although the majority of the data are from animals and many theories are still conjectural, these changing concepts of normal and disordered secondary neurulation will provoke further advancements in our management strategies as well as in the pathoembryogenetic understanding of anomalous lesions in this area.

Retained medullary cord and terminal myelocystocele as a spectrum: case report.

The caudal portion of the spinal cord, the medullary cord, is formed by secondary neurulation. One of the distinctive features of secondary neurulation compared to primary neurulation is that the medullary cord normally degenerates into a filum in humans. Various anomalies have been known to originate from degenerating process errors. One anomaly is terminal myelocystocele (TMCC), which is a closed spinal dysraphism with an elongated caudal spinal cord. The terminal part is filled with cerebrospinal fluid (CSF) and protrudes into the dorsal extradural space. Another anomaly is the retained medullary cord (RMC), which is a nonfunctioning cord-like structure extending to the cul-de-sac. In a 1-month-old boy, we identified an RMC with cystic dilatation of the caudal end extending to the epidural space at the very bottom of the cul-de-sac, resembling a degenerating terminal balloon, which is an essential feature of TMCC. Hence, this case may be considered an intermediate form between TMCC and RMC. This case provides clinical evidence that TMCC and RMC share the same pathoembryogenic origin, namely, failure of the regression phase of secondary neurulation.

The transcriptomic landscape of caudal cell mass in different developmental stages of the chick embryo.

INTRODUCTION: The caudal cell mass (CCM) is an aggregate of undifferentiated pluripotent cells and the main player in secondary neurulation. Previous studies have elucidated the dynamic fate of the multipotent cell lineages, with a recent interest in the neuromesodermal progenitors. However, a transcriptomic analysis of the CCM during secondary neurulation has not been performed yet. METHODS: We analyzed RNA sequencing data of CCM samples at three different developmental stages of chicken embryos; HH16 (largest CCM phase), HH20 (secondary neural tube formation phase), and HH28 (degeneration phase). RESULTS: The transcriptomic profiles were clearly distinguishable according to developmental stage, and HH20 was shown to have not only intermediate, but also unique properties in secondary neurulation. A total of 10,666 differentially expressed genes, including FGF18 and GDF11, were identified and enriched in several gene ontologies related to embryogenesis or organogenesis. We also found that genes encoding transcription factors, such as TWIST2, IRX4, HOXB4, HOXD13, LIN28A, CDX4, and Brachyury, were among the top-ranked differentially expressed genes. CONCLUSION: Through transcriptomic profiling, we provided a picture of the developmental process of the CCM. We identified several key molecules or pathways involved in secondary neurulation and the pathogenesis of related diseases.

Occult tethered cord syndrome: a rare, treatable condition.

PURPOSE: Occult tethered cord syndrome (OTCS) is an entity that shows tethered cord syndrome (TCS) with normal spinal MRI findings. The definition and treatment of OTCS have been controversial since first proposal. The purpose of this study was to evaluate the existence, prevalence, histological characteristics, and surgical outcomes of OTCS. METHODS: We retrospectively analyzed patients who underwent untethering surgery for OTCS from January 2010 to December 2019. Inclusion criteria were (1) clinical manifestation of TCS; (2) supported by urodynamic study (UDS) or electromyography/nerve conduction study; (3) no structural lesions in the urological tract or spinal cord, and no developmental delay; and (4) postoperative follow-up for > 6 months. Sectioned fila from OTCS patients were histologically compared with those from cases of thickened filum or low-lying conus. RESULTS: Five (four female, one male) of 439 patients (1.1%) who underwent untethering surgeries for occult spinal dysraphism corresponded to OTCS. Mean age at the time of surgery was 16 years (7-22 years). Mean postoperative follow-up duration was 45 months (15-114 months). The main symptom was urinary dysfunction in four patients and leg pain in one. All patients had detrusor-sphincter dyssynergia. Fila from OTCS patients revealed increased fibrous tissue as in TCS patients. Four patients showed postoperative improvement and one with preoperative static course had no improvement. CONCLUSIONS: This study suggests that OTCS is a definitely existing entity although rare. OTCS is curable when timely treatment is given. Sudden onset with rapid progression of symptom seems the best indication for surgery.

Myelomeningocele as an anomaly of secondary neurulation.

PURPOSE: Myelomeningocele (MMC) is the representative entity of open neural tube defects resulting from an error during primary neurulation. However, cases of MMC in the region of the secondary neural tube (below the junction of S1 and S2 vertebrae) are sometimes encountered. We aimed to analyze the clinical features of atypical "low-lying" MMC in comparison to the typical MMC and suggest possible pathoembryogenesis. METHODS: From 1986 to 2020, 95 MMC patients were treated in our institute. A retrospective review of the radiological and clinical information was performed. We defined "low-lying" MMCs as those with fascia or lamina defects below the S1-2 interspinous ligament. RESULTS: Thirty-one out of the 95 MMC patients were identified as having low-lying MMC. The percentage of low-lying MMC within the entire MMC group increased dramatically (19% from 1990 to 1999 and 48% from 2000 to 2020). Thirty-nine percent of the low-lying MMCs were associated with hydrocephalus, and 36% showed the Chiari malformation. Clean intermittent catheterization was being performed by 52% of the patients and 46% had a motor weakness. The proportions of hydrocephalus, neurological symptoms, and the number of related procedures in the low-lying MMC were substantially lower than the typical MMC in our cohort and the literature. CONCLUSIONS: We present cases of atypical MMC occurring in the region of secondary neurulation. These cases provide clues that secondary neurulation may lead to open neural defects. Future experiments with animal models supporting what we have seen in the clinics will greatly enhance the understanding of the developmental process of neurulation and the corresponding anomalies.

Significant Therapeutic Effects of Adult Human Neural Stem Cells for Spinal Cord Injury Are Mediated by Monocyte Chemoattractant Protein-1 (MCP-1).

The limited capability of regeneration in the human central nervous system leads to severe and permanent disabilities following spinal cord injury (SCI) while patients suffer from no viable treatment option. Adult human neural stem cells (ahNSCs) are unique cells derived from the adult human brain, which have the essential characteristics of NSCs. The objective of this study was to characterize the therapeutic effects of ahNSCs isolated from the temporal lobes of focal cortical dysplasia type IIIa for SCI and to elucidate their treatment mechanisms. Results showed that the recovery of motor functions was significantly improved in groups transplanted with ahNSCs, where, in damaged regions of spinal cords, the numbers of both spread and regenerated nerve fibers were observed to be higher than the vehicle group. In addition, the distance between neuronal nuclei in damaged spinal cord tissue was significantly closer in treatment groups than the vehicle group. Based on an immunohistochemistry analysis, those neuroprotective effects of ahNSCs in SCI were found to be mediated by inhibiting apoptosis of spinal cord neurons. Moreover, the analysis of the conditioned medium (CM) of ahNSCs revealed that such neuroprotective effects were mediated by paracrine effects with various types of cytokines released from ahNSCs, where monocyte chemoattractant protein-1 (MCP-1, also known as CCL2) was identified as a key paracrine mediator. These results of ahNSCs could be utilized further in the preclinical and clinical development of effective and safe cell therapeutics for SCI, with no available therapeutic options at present.

Caudal duplication syndrome: a literature review and reappraisal of its pathoembryogenesis.

INTRODUCTION: The term caudal duplication syndrome (CDS) was first introduced for complex anomalies of the distal caudal end of the trunk. The pathoembryogenesis of CDS is yet unknown, although a few theories have been proposed. We reviewed the previously proposed pathoembryogenetic theories and suggested a new perspective through the common clinical characteristics shown in CDS cases reported in the literature. METHODS: We conducted a systematic literature search of the online database PubMed from October 1993 to October 2020, using the search term "caudal duplication syndrome", according to the first mention of this entity. A total of 17 articles with 23 patients were reviewed. RESULTS: The most common manifestations were the duplication of the distal colon, genitourinary organs, and lower spine. Specifically, the duplicated bladders or uteri contacted their counterpart through a septum, and the duplicated bowels ran parallel. More caudal structures, such as the urethra or anus, were formed separately. The duplication seems to be a result of division by an intervening septum or structure in each part. In addition, duplication was not limited to the structures formed from the caudal cell mass (CCM), such as the distal spine and spinal cord, but also included hindgut structures. Moreover, anomalies involving caudal mesenchymal defects were also present. Considering clinical manifestations that are related to all three germ layers and seemingly the overseptation of these germ layers in CDS patients, with supporting data from animal experiments, events such as late-stage errors involving Hensen's node/the primitive streak and the duplication of the CCM with the hyperplasia of the abnormally located central caudal mesenchyme are probable pathoembryogenetic mechanisms for CDS. The "leakage" of the normal growth power of the caudal mesenchyme into the intervening midline space between the two CCMs and consequent weak lateral and caudal pushes of the caudal mesenchyme may explain the association of caudal agenesis or its related anomalies with CDS. CONCLUSION: We propose a theory that by a molecular interaction, an insult causes late gastrulation phase problems, resulting in ectopic primitive streak formation, and therefore, a duplication of the CCM is induced. Subsequently, the overactivity of abnormally positioned midline mesenchyme between the two CCMs may divide the hindgut derivatives by a central septum. Underactive lateral and caudal pushes of the caudal mesenchyme may lead to an association of features shown in caudal agenesis.

The stability of multifocal ventriculoperitoneal shunts with Y-connections.

PURPOSE: Multifocal ventriculoperitoneal shunts with Y-connections (MVPS with Ys) are widely used in many centers when neuroendoscopic procedures on entrapped ventricles are not feasible; however, their use is not frequent. This study aimed to confirm the stability of an MVPS with Y and, for the first time, identify the factors that influence stability. METHODS: We studied 33 consecutive patients who underwent initial conversion to MVPS with Ys. The one-year overall shunt survival rate was calculated and compared with the historical outcome of single ventriculoperitoneal shunts (VPSs). The factors influencing the one-year overall shunt survival rate were also investigated. The one-year survival rate for proximal catheters in each location was further investigated, and the rates were compared among locations. The factors affecting proximal catheter survival were determined. RESULTS: The one-year overall shunt survival rate of MVPS with Y was 70%, which was not much different from that of previously reported single VPSs, including our institution. We found no significant factor influencing overall shunt survival, but when an additional catheter was inserted into the fourth ventricle, the survival rate was exceptionally low at 40% (p = 0.21). When we investigated the one-year survival rate of each proximal catheter, we found that the location of the proximal catheter showed a certain trend toward significance (p = 0.07), especially in the case of the fourth ventricle, which had the lowest survival rate at 57% and an odds ratio of 15.64 (p = 0.013) in multivariate analysis. However, when the catheter was sufficiently inserted parallel to the brain stem using navigation, the survival was relatively well maintained (1,995 to 2,547 days). CONCLUSIONS: The stability of MVPS with Y was similar to that of single VPSs. However, the malfunction rate of the proximal catheter inserted at the fourth ventricle in the Y-connection was higher than that at other locations. The transcerebellar vertical approach or transtentorial approach parallel to the brain stem may decrease the malfunction rate.

Combined unilateral coronal-lambdoid suture synostosis: surgical outcome of suturectomy and postoperative helmet therapy.

INTRODUCTION: Nonsyndromic, multi-suture craniosynostosis is not common, especially those involving unilateral coronal and lambdoid sutures. Based on the experience on 6 cases, we analyzed the skull morphology of combined unilateral coronal-lambdoid suture synostosis and evaluated the surgical outcome of suturectomy. METHODS: Patients who underwent an operation for craniosynostosis in Seoul National University Children's Hospital from 2010 to 2018 were reviewed. For qualitative analysis of the surgical outcome, five typical morphologic characteristics (ipsilateral superior orbital rim deviation, deviation of the nasal ridge, ipsilateral frontal flattening, contralateral parietal bulging, ipsilateral occipitomastoid bulging) in unilateral coronal-lambdoid suture synostosis were evaluated based on medical photos, plain skull radiographs, and CT scans. For quantitative analysis, three parameters (nasal root deviation, orbital asymmetry, posterior skull base deviation) were measured. RESULTS: Among 316 patients with craniosynostosis, 41 patients had nonsyndromic, multi-suture synostosis. There were 6 unilateral coronal-lambdoid suture synostosis patients who were all treated with suturectomy. Qualitative evaluation of the postoperative outcome revealed that the nasal root and orbital rim deviations and the contralateral occipitomastoid bulging showed satisfactory improvements. However, ipsilateral frontal flattening and contralateral parietal bulging were improved but still present in most cases. Quantitative, craniometric analysis supported the qualitative outcomes. The nasal ridge deviation angle was corrected from 7.04 to 1.79 degrees. The posterior skull base deviation angle improved from 6.29 to 3.55 degrees. CONCLUSION: Unilateral coronal-lambdoid suture synostosis suturectomy followed by helmet therapy resulted in favorable outcomes, although the correction of frontal flattening was less satisfactory than the other measures. Considering the minimal invasiveness of the treatment, suturectomy may be a viable option for these patients.