Delayed spinal arachnoiditis with syringomyelia following aneurysmal subarachnoid haemorrhage: a case report with patient experience.

BACKGROUND AND IMPORTANCE: Syringomyelia, or the formation of fluid-filled cysts within the spinal cord, associated with delayed spinal arachnoiditis is an uncommon complication of aneurysmal subarachnoid haemorrhage. To date, about 18 cases have been reported in medical literature, with just two reported in patients under the age of 35 years. CLINICAL PRESENTATION: A 27-year-old female patient complained of sudden, severe headaches in the occipital region, nuchal rigidity, and drowsiness when she presented at our institution. A head computed tomography scan revealed intraventricular bleeding in the lateral and fourth ventricles with more extensive haemorrhaging in the frontal horns. A left posterior inferior cerebellar artery (PICA) aneurysm was confirmed via digital subtraction angiogram, and endovascular embolization was done. Two years later, the patient reported intense pain in the lower back along with symptoms suggestive of spinal cord compression. Spinal magnetic resonance imaging (MRI) showed spinal adhesions from C1 to L4, syringomyelia with some vasogenic oedema extending from T3 to T9 level, and a cyst in the lumbar region. Consequently, a right hemilaminectomy was performed along with microsurgical release of arachnoid adhesions and placement of a subdural drain. Radiological and symptomatic improvements were observed. Since then, the patient's clinical condition has remained stable during the past three years of follow-up visits. CONCLUSIONS: Literature on optimal treatment modalities and patient prognosis is scarce and debated. The time for symptom improvement depends on the level and extent of spinal cord involvement. Rehabilitation may be required for most patients, as complete symptomatic recovery may not be attainable.

Spinal cord schistosomiasis in a 6-year-old child with complete recovery after spine surgery and medical treatment: case report and discussion.

Spinal cord schistosomiasis is a rare and severe form of schistosomiasis. The prognosis is largely conditioned by early diagnosis and treatment. The authors present a case of spinal cord schistosomiasis complicated by spinal cord compression syndrome. This is the case of a 6-year-old patient who presented with febrile gastroenteritis followed by complete paralysis of both lower limbs of sudden onset following a brief stay in a village setting with notion of multiple baths at a stream. Spinal cord MRI revealed an enlarged spinal cord spanning D10 to D12 with heterogeneous contrast enhancement and a syrinx cavity above the lesion. Biological workup revealed an inflammatory syndrome. Treatment consisted of decompressive laminectomy with biopsy of the lesion and a syringo-subarachnoid shunt. Pathological analysis revealed fragments of central nervous system tissues with an infiltrate composed of lymphocytes, plasmocytes, and macrophages producing granulomatous foci lined with areas of necrosis in addition to a large contingent of polynuclear eosinophils, agglutinating around or covering in some places elongated ovoid structures, with relatively thick eosinophilic shells and presenting a terminal spur. Adjuvant treatment consisted of praziquantel and corticotherapy for 1 month. The evolution showed marked improvement in the neurological deficits. She now walks unassisted and has good sphincter control. Spinal cord schistosomiasis is rare in our context; its diagnosis is difficult. The treatment is both medical and surgical.

[A Surgical Case of Currarino Syndrome with Syringomyelia].

Currarino syndrome is characterized by a triad of anorectal malformations, sacral defects, and presacral masses. Although it is not extremely rare, this report presents a surgical case of Currarino syndrome with syringomyelia and discusses related literature. The patient is a girl, aged 2 years and 2 months, who presented with marked constipation, chronic cystitis, and lower limb weakness. After examining the patient through magnetic resonance imaging, we diagnosed her with rectal compression due to meningocele and syringomyelia. The base of the meningocele was detached, and the spinal cord was untethered. One week after surgery, her lower limb weakness and constipation improved. Following up on symptoms and performing imaging is essential to determine a treatment plan for Currarino syndrome. (Received 28 February, 2023; Accepted 22 March, 2023; Published 1 August, 2023).

A Rare Case Report of Eight Syndrome Secondary to Syringomyelia Associated with Type I Chiari Malformation.

Eight syndrome is defined as the combination of a unilateral conjugate gaze palsy and ipsilateral seventh cranial nerve palsy. It may occur as a result of demyelinating, vascular, infectious, or compressive lesions of the brainstem localized to the caudal pontine tegmentum. A 43-year-old woman was admitted to our clinic with complaints of headache, inability to look to the left, and weakness on the left side of her face. The complaints had begun abruptly about a month before her admission. Suboccipital decompression surgery for type I Chiari malformation had been performed 10 years earlier. Neuro-ophthalmological examination revealed left-sided horizontal gaze palsy and anisocoria. Cranial and cervical magnetic resonance images revealed cerebellar tonsillar herniation and syringomyelia, the latter of which was considered to be the cause of eight syndrome. No interventions were performed, and periodic follow-up was advised on neurosurgical consultation. Left gaze palsy and facial palsy recovered almost completely in three months, while the anisocoria persisted. Syringomyelia should be considered among the causes of horizontal gaze palsy plus ipsilateral seventh nerve palsy, termed as eight syndrome. Clinical suspicion and appropriate radiological examination can aid in the diagnosis.

Cerebrospinal Fluid Flow and Brain Motion in Chiari I Malformation: Past, Present, and Future.

Cranio-spinal volume and pressure changes associated with the cardiac-cycle and respiration are altered in Chiari I malformation (CMI) due to obstruction of cerebrospinal fluid (CSF) flow at the foramen magnum. With the introduction of motion-sensitive MRI sequences, it was envisioned that these could provide noninvasive information about volume-pressure dynamics at the cranio-cervical junction in CMI hitherto available only through invasive pressure measurements. Since the early 1990s, multiple studies have assessed CSF flow and brain motion in CMI. However, differences in design and varied approaches in the presentation of results and conclusions makes it difficult to fully comprehend the role of MR imaging of CSF flow and brain motion in CMI. In this review, a cohesive summary of the current status of MRI assessment of CSF flow and brain motion in CMI is presented. Simplified versions of the results and conclusions of previous studies are presented by dividing the studies in distinct topics: 1) comparing CSF flow and brain motion between healthy subjects (HS) and CMI patients (before and after surgery), 2) comparing CSF flow and brain motion to CMI severity and symptoms, and 3) comparing CSF flow and brain motion in CMI with and without syringomyelia. Finally, we will discuss our vision of the future directions of MR imaging in CMI patients. EVIDENCE LEVEL: 2. TECHNICAL EFFICACY: 5.

Natural History of Chiari 1 Deformity.

PURPOSE: Chiari type 1 deformity (Ch1) is associated with bony deformity of the skull base and herniation of cerebellar tonsils more than 5 mm below the foramen magnum. Magnetic resonance imaging (MRI) is used for diagnosis and surgery is advised for symptomatic children. We present a case series using MRI including cerebrospinal fluid (CSF) flow, in children with Ch1 to demonstrate a variety of outcomes, both surgical and spontaneous: spontaneous resolution, spontaneous worsening, postsurgical improvement, and postsurgical deterioration. CASE: A 2-week-old female newborn underwent brain MRI demonstrating an ectopic neurohypophysis, under opercularization suggesting brain immaturity and a normal craniocervical junction (CCJ). Follow-up (F/U) MRI at 6 years of age showed interval spontaneous development of Ch1 with decreased CSF spaces at CCJ. CASE: A 6-year-old girl referred for imaging with short stature and growth hormone deficiency demonstrated incidental findings of Ch1 without syringomyelia. There was 15-mm protrusion of pointed cerebellar tonsils through the foramen magnum and a reduced CSF space at the craniocervical junction. No surgery was performed, and F/U MRI at the age of 7 years demonstrated spontaneous resolution of the tonsillar ectopia (cerebellar tonsils now 3 mm right and 6 mm left) and expansion of the CSF spaces at CCJ. CASE: A 7-year-old boy with headaches and staring spells underwent an MRI demonstrating 6-mm protrusion of pointed cerebellar tonsils and CSF space reduction at CCJ. No surgery was performed, and F/U imaging at the age of 9 years demonstrated spontaneous improvement in cerebellar tonsillar position and increased bidirectional CSF flow at CCJ. CASE: A 17-month-old boy underwent brain MRI for unsteady gait and poor vestibular response, which showed Ch1 and narrow CSF spaces at the foramen magnum and with reduced CSF flow. At the age of 3 years, after posterior fossa decompression, F/U MRI showed postsurgical improvement of the position of the cerebellar tonsils and increased CSF space at CCJ. CASE: A 4-month-old male infant with a history of 34-week prematurity, prior germinal matrix hemorrhage, and neonatal subdural hemorrhage was referred for MRI of the cervical and thoracic spine for evaluation of developmental delay and hypotonia with torticollis. Magnetic resonance imaging of the spine demonstrated mild protrusion of inferiorly pointed cerebellar tonsils up to the foramen magnum, with visible CSF and without retroflexion of the dens. Follow-up MRI scans demonstrated progressive worsening of the Chiari 1 deformity, even after multiple surgeries. CONCLUSIONS: It is important to be aware of a variety of different outcomes with Ch1, including spontaneous resolution, spontaneous worsening, improvement with surgery, and even deterioration after surgery. More research is required to determine objective criteria for predicting outcome, which include both anatomic measures and physiologic measures of CSF flow, so that better surgical decisions can be made and for evaluating patients who have undergone surgery.

Paroxysmal Neuropathic Pruritus in Patients With Chiari Malformation Type I: A Rare Phenotype.

Chiari malformation type I (CM-I) is a group of deformities in the posterior fossa and hindbrain, including the pons, cerebellum, and medulla oblongata. Paroxysmal pruritus in CM-I has been reported only once before in the literature. This study was a cross-sectional study over 12 months at a tertiary care pediatric hospital involving children aged one to 18 years with CM-I presenting with paroxysmal itching. Three patients with CM-I presented with severe episodes of paroxysmal itching. Patient 3 was started on carbamazepine therapy for seizures, and incidentally, his itching subsided. The pruritus of neuropathic etiology has been reported to be associated with syringomyelia, spongiform encephalopathies, autoimmune disorders like multiple sclerosis, patients with end-stage renal failure on dialysis, and neoplasms. Antihistamines and antiallergics are ineffective in treating these patients, reiterating a central mechanism for pruritus. At present, no drugs have been approved for the treatment of neuropathic pruritus specifically. The commonly used treatments for neuropathic itch are antiseizure medications, tricyclic antidepressants, gabapentinoids, ketamine, and oral kappa opioids, including butorphanol and difelikefalin. Better structured prospective studies are needed to analyze the prevalence and scales to assess disability caused due to neuropathic itch in CM and may enhance understanding in this area.

Complex spinal dysraphism: myelomenigocele associated with dorsal bony spur, split cord malformation type I, syringomyelia, lipoma and tethered cord.

Variations in split cord malformation (SCM) are known. However, association of SCM type I with myelomeningocele along with same level dorsal bony spur has not been described previously. We report a 1-year old male child who presented with these findings with associated syringomyelia, lipoma and tethered cord.

Syringosubarachnoid shunt: insertion technique.

BACKGROUND: Syringomyelia is a rare disorder but its impact on patients' quality of life can be devastating. The exact pathophysiology remains unknown; the syrinx can either be idiopathic or associated with conditions such as Chiari malformation, scoliosis, malignancy, infection and trauma. Several techniques have been described to decompress syringomyelia with distal drainage to the subarachnoid space, pleura or peritoneum. METHOD: We present a modification of the syringosubarachnoid shunt insertion technique with minimal myelotomy and the use of a T shaped shunt which does not require suturing to the pia matter to prevent shunt migration. CONCLUSION: This technique for syringosubarachnoid shunt insertion is likely to prevent shunt migration and scarring whilst minimizing suturing and optimising syrinx decompression.

Cervical syringomyelia with caudal thoracic epidural lipomatosis: case report and literature review.

Syringomyelia associated with epidural lipomatosis is a rare finding. Only three published cases of epidural lipomatosis associated with syringomyelia exist in the literature.We report the case of a 46-year-old woman who presented with progressive myelopathy over an 18-month period. Imaging revealed significant thoracic spinal cord compression secondary to epidural lipomatosis from T3 to T8 with cephalad cervical syringomyelia extending from C7 to T1. Imaging was unremarkable for Chiari malformation or a craniospinal space-occupying lesion. A T2 to T8 laminoplasty was performed, removing excessive epidural adipose tissue to decompress the thoracic spinal cord. Postoperatively, the patient reported symptom improvement with complete symptom resolution at 3 months. Follow-up imaging at 3-months demonstrated thoracic spinal cord decompression with mild syrinx reduction. At two-year follow-up the patient remained asymptomatic with unchanged imaging.Syringomyelia in the setting epidural lipomatosis is a rare finding.

Fibrofatty filum terminale: long-term outcomes from a Singapore children's hospital.

OBJECTIVE: The role of prophylactic detethering a fibrofatty filum terminale (FFT) remains equivocal. Furthermore, long-term studies focusing on urological outcomes are sparse. The aims of this study were to present an institutional experience on the perioperative and long-term outcomes of FFT surgery and to assess for factors that contribute to postoperative clean intermittent catheterization (CIC). METHODS: This was a single-institution, retrospective study conducted over a 20-year period. Patients younger than 19 years of age who underwent surgery for FFT were included. Variables of interest included patient demographics, clinical presentation, radiological findings, postoperative complications, and long-term need for CIC. Outcomes were measured using the Necker functional score and modified Hoffer Functional Ambulation scale score at 3, 6, and 12 months postdischarge. RESULTS: A total of 164 surgeries were performed for FFT from 2000 to 2020. The median age at surgery was 1.1 years, and the mean follow-up duration was 8.3 years. There were 115 patients (70.1%) who underwent prophylactic-intent surgery and 49 patients (29.9%) who underwent therapeutic-intent surgery. The proportion of therapeutic-intent surgeries increased significantly with age percentiles (0-20th, 21.9%; 20th-40th, 9.1%; 40th-60th, 18.2%; 60th-80th, 36.4%; and 80th-100th, 63.6% [p < 0.001]). Thirty patients (18.3%) had an associated syndrome, the most common (n = 19, 11.6%) being VACTERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities). Forty-eight patients (29.3%) had an associated malformation (anorectal anomaly = 37, urogenital anomaly = 16, and sacral anomaly = 3). Cutaneous manifestation was the most common presentation (n = 96, 58.5%), followed by lower-limb neurological deficits (n = 21, 12.8%). A low-lying conus was present in 36.0% of patients (n = 59), and 16.5% had an associated syrinx (n = 27). There were 26 patients (18.8%) with an abnormal preoperative urodynamic study. Three patients (1.8%) had postoperative complications that required repeat surgery. There were no cases of CSF leakage. One patient (0.6%) developed retethering requiring another surgery. Postoperative CIC was required in 11 patients (6.7%). Multivariable analyses showed that an abnormal preoperative urodynamic study (adjusted OR 5.5 [95% CI 1.27-23.9], p = 0.023) and having an intraspinal syrinx (adjusted OR 5.29 [95% CI 1.06-26.4], p = 0.042) were associated with the need for CIC. CONCLUSIONS: The authors' results demonstrate that detethering surgery for FFT is a relatively safe procedure and can be performed prophylactically. Nonetheless, the risks of postoperative CIC should be emphasized during the preoperative counseling process.

Role of Chiari Decompression in Managing Spinal Deformity Associated with Chiari I Malformation and Syringomyelia.

CM-I-associated syringomyelia is a risk factor for scoliosis where a larger syrinx size is more likely to be associated with scoliosis. Therefore, the effect of syrinx on scoliosis progression may be alleviated by PFD. There is no difference in the need for fusion surgery between patients undergoing PFD with duraplasty vs. those undergoing extradural decompression; however, PFD with duraplasty is associated with an improvement in curve magnitude compared to extradural decompression alone. Further study on the comparison of PFD techniques for this cohort of patients is needed. PFD is a durable surgical option for patients with CM-I, syrinx, and scoliosis. Early decompression of CM-I in younger patients and those with smaller scoliosis curves at presentation is recommended as there is a higher likelihood of halting curve progression.

Adult Chiari Malformation Type I: Surgical Anatomy, Microsurgical Technique, and Patient Outcomes.

In this study, the authors summarize the current knowledge on epidemiology, demographics, risk factors, and prognostic factors that influence outcomes in patients with adult Chiari malformation type I (CM-I) who underwent posterior fossa decompression surgery with duraplasty. Furthermore, they describe the contribution of their research group to the field of adult CM-I treatment, including association of increased body mass index with severity of CM-I and syringomyelia, relevant surgical anatomy, and surgical technique of 270° microsurgical decompression of foramen magnum. The authors also report on common complications in the literature and describe techniques for prevention of complications.

Neuropathic arthropathy of the shoulder secondary to operated syringomyelia: a case-based review.

Neuropathic arthropathy (NA) is a long-term progressive disorder that causes joint destruction in the existence of a neurologic deficit. Minor injuries and fractures are commonly overlooked until the visible joint deterioration becomes persistent. Syringomyelia is one of the important causes of NA. The appearance of clinical signs in syringomyelia is caused by longitudinal cysts formed in the cervical and cervicothoracic regions of the spinal cord. Depending on the existence of the underlying disorder, the number and localization of the syrinxes, a range of symptoms, involving pain, sensation deficit, loss of motor function, and deep tendon reflex abnormality, emerge. The case is here described of a 68-year-old female patient with shoulder NA following syringomyelia, who partially responded to the rehabilitation program. Furthermore, the available case reports were comprehensively reviewed on Web of Science, Scopus, and PubMed/Medline. Furthermore, the available case reports were comprehensively evaluated on Web of Science, Scopus, and PubMed/Medline. Thus, we aimed to present the demographic characteristics, symptoms, physical examination signs, treatment, and follow-up parameters of syringomyelia-related shoulder NA cases.

Outcome predictors and clinical presentation of syringomyelia.

BACKGROUND: The prognosis of syringomyelia is not yet established. Syringomyelia derived from different etiologies contributes to similar symptoms. OBJECTIVE: Assess the syringomyelia in our medical institutes and describe the etiologies and clinical appearance of the disorder. And identify the predictors of a good outcome and to find the most suitable timing of surgical intervention according to our results. METHODS: This retrospective cohort study used databases in our hospitals to analyze 70 cases of syringomyelia between 1997 and 2014. All available information was obtained from medical records and radiological reports. We used American Spinal Injuries Association disability scores (ASIA scores), the modified Nurick classification system, and recorded the number of days the patient was hospitalized, for neurological and functional assessment. Univariate and multivariate analyses were used to evaluate the relationship between clinical factors and outcomes. RESULTS: Non-communicating syringomyelia was the most common type of syringomyelia. In univariate analysis, autonomic dysfunction and motor impairment were strong predictors of poor neurological and functional outcomes. In addition to the above factors, syrinxes at the cervical level predicted better functional outcomes than at any spinal level in multivariate analysis. CONCLUSIONS: Motor impairment, which is commonly seen in patients with syringomyelia in Taiwan, is a strong predictor to poor neurological and functional outcomes. Our study indicates that patients without autonomic dysfunction or motor impairment should receive timely surgical intervention to prevent symptomatic deterioration. We also found that cervical syringomyelia in particular has the potential for good functional recovery after adequate intervention.

A case report of spinal toxocariasis with extensive tumor-like involvement.

BACKGROUND: Toxocariasis is a common parasitic infection worldwide. Although it can present as several clinical syndromes, neurological manifestation is rare. Only a few reports are available on spinal cord involvement of toxocariasis. We report a case that presented with gait disturbance due to progressive lower limb spasticity. The patient had had visceral toxocariasis infection 8 years before. A spine magnetic resonance image (MRI) showed syringomyelia along the entire thoracic cord with small nodular enhancing lesions in the mid-portion of the syrinx, which led to the suspicion of ependymoma. Surgical mass removal was performed. However, histopathological examination of the mass did not show any malignant cells; instead, there were numerous axonal retraction balls with an eosinophilic granular body-like appearance. The serum antibody titer against toxocariasis was borderline high. Taken together, these observations led to a diagnosis of Toxocara infection, and the patient was treated with albendazole. CONCLUSION: To the best of our knowledge, this is the first case report of tumor-like spinal toxocariasis involving extensive lesions. A solid enhancing mass with accompanied syrinx and hemorrhage might be a Toxocara infection. It can easily be diagnosed with serologic tests and simply be treated with oral albendazole if suspected.

Pediatric Chiari I malformation: novel and traditional measurements associated with syrinx and surgery.

PURPOSE: Multiple imaging parameters have been examined to estimate the presence of syrinx and the need for surgery in Chiari I patients (CM1); however, no consistent or definitive criteria have been proposed. The objective of this study was to review existing and identify novel radiological and clinical characteristics of CM1 patients that associate syrinx development and surgical intervention. METHODS: Patients with Chiari I malformation diagnosed on imaging between 0 and 18 years were retrospectively reviewed from January 1, 2007 to February 12, 2020. Participants were included if they had a baseline MRI of the head and spine prior to surgical intervention if required. Forty age-matched controls with cranial imaging were identified for comparison. Imaging parameters and clinical symptoms were recorded. RESULTS: A total of 122 CM1 patients were included in this study. Of the 122 patients, 28 (23%) had syrinx, and 27 (22%) had surgery. The following imaging parameters associated with syrinx and surgical intervention were identified: midbrain length (P < 0.001; P = 0.032), the obex position (P = 0.002; P < 0.001) and medullary kinking (P = 0.041; P < 0.001). Among the clinical features, the presence of overall pain (P = 0.017; P = 0.042), neck pain (P = 0.005; P = 0.027), and sensory dysfunction (P < 0.001) were found to be strongly associated with syrinx and surgery. CONCLUSION: While further investigation is needed, these specific radiological and clinical parameters should be considered when evaluating CM1 patients and may be used to guide further management.

Various manifestation of Chiari I malformation in children and improvement after surgery.

INTRODUCTION: Chiari I malformation is defined as caudal displacement of the cerebellar tonsils into the foramen magnum. The most commonly associated finding is cervical syringomyelia. The most common presenting symptom is occipital and upper cervical pain. The incidence of syringomyelia is between 30% and 70%. Surgery is recommended for symptomatic patients. The main purpose of the study is to present the data of children with Chiari type I malformation that were treated surgically in low-volume center and to describe some atypical presentations in children. MATERIALS AND METHODS: At University Medical Centre Maribor, Slovenia, we performed a retrospective study of children with Chiari type I malformation that were treated surgically in the period from 2012 until 2021. The indication for surgery was symptomatic Chiari type I malformation. Suboccipital decompression with laminectomy of at least C1 (in one case also C2) with splitting of dura or duraplasty was performed. In a few cases coagulation of both tonsillar tips was necessary to achieve sufficient decompression and restoration of adequate cerebrospinal fluid (CSF) flow. RESULTS: Ten children (under 18 years of age) underwent surgery for Chiari I malformation. Four patients had atypical presentation. In nine patients there was improvement after surgery. Complication rate was zero with no revision surgery. In one case suboccipital headache persisted. In all cases with preoperative syringomyelia it improved after surgery. Scoliosis improved in two out of three cases. There was no mortality after the procedure. DISCUSSION: When patients with Chiari I malformation become symptomatic, it is recommended to perform surgery as soon as possible. Comparing the techniques; dura-splitting technique has advantages of lesser operation duration, lesser intraoperative bleeding and lower complication rates than duraplasty. CONCLUSIONS: The majority of patients with Chiari I malformation improve after surgery. Surgical procedure is safe with very low morbidity and mortality. Surgical technique must be an individualized patient tailored choice.