Pediatric spinal arachnoid cysts with compressive myelopathy: single-center experience and update on surgical management.

PURPOSE: This study describes the surgical outcome of pediatric primary spinal arachnoid cysts (SACs) presenting with compressive myelopathy and gives an update on the classification and management of these rare lesions. METHODS: We performed a single-center retrospective analysis of pediatric patients operated for primary spinal arachnoid cysts. The clinical and radiologic profiles and surgical outcomes of these children were analyzed. Subgroup analysis was done in the laminoplasty vs laminectomy groups to see for the development of spinal deformity. RESULTS: There were 10 males and seven females with a mean age of 10.4 years (range:6-14 years). The cysts extended to an average of 5.2 levels (range:2-8). They were extradural in seven (41%) and intradural in 10 (59%). Six intradural and four extradural cysts underwent laminectomy (n = 10) while four intradural and three extradural cysts underwent laminoplasty (n = 7). Although three out of 10 cases in the laminectomy group and none in the laminoplasty group had post-operative spinal deformity, this result was not statistically significant (p = 0.110). There was a moderate negative correlation between post-operative cord occupancy ratio (COR) and post-operative McCormick grade (Pearson correlation coefficient = -0.453, p = 0.068), suggesting that higher CORs are associated with lower McCormick grades. CONCLUSION: Symptomatic pediatric primary spinal arachnoid cysts are safely and effectively managed by marsupialization or microsurgical excision. Considering the growing age group, laminoplasty rather than laminectomy should be the standard surgical procedure to prevent late postoperative spinal deformity. Clinically significant recurrences are rare in the setting of adequate cord expansion and restored subarachnoid CSF flow following surgery.

Inter-dural spinal cyst with acute thoracic compressive myelopathy: anatomical aspects of spinal dura, case report and literature review.

INTRODUCTION: Inter-dural juxta-facet spinal cysts occur rarely. They form as part of the degenerative spinal disease process and can be misdiagnosed as synovial cysts or ganglion cysts. We report the case of a thoracic inter-dural juxta-facet spinal cyst causing acute compressive thoracic myelopathy. METHODS: The data was collected retrospectively from patient records. The literature review was performed in PubMed. RESULTS: We report a case of symptomatic inter-dural juxta-facet thoracic spinal cyst. The literature review showed a variety of different spinal cysts including arachnoid cyst, discal cyst, ganglion cyst, epidermoid cyst and synovial cysts. Micro-instability and repeated microtrauma associated with degenerative changes are most likely contributors to its formation. Asymptomatic cysts can show spontaneous resolution. When symptomatic, they can be managed with surgical excision with good patient outcome. CONCLUSION: Inter-dural spinal cysts can be diagnosed and surgically excised to produce excellent post-operative outcome. High pre-operative index of suspicion of this diagnosis together with good understanding of the intraoperative anatomy are essential to avoid inadvertent dural breach.

An extradural cyst in a French Bulldog.

CASE HISTORY: A 7-year-old, male neutered French Bulldog was referred to a specialist veterinary hospital for evaluation of progressive paraparesis of 6-months' duration. The owners reported both faecal and urinary incontinence at home. CLINICAL FINDINGS: The dog presented with ambulatory paraparesis and pelvic limb ataxia that was more pronounced in the right pelvic limb. The pelvic limb withdrawal response and sciatic myotatic response were reduced bilaterally. Postural reaction responses were delayed in both pelvic limbs, and this was more obvious in the right pelvic limb. The anal tone and perineal sensation were normal at the time of examination.An L4-S3 myelopathy was suspected. CT of the spine revealed a compressive, bilobed, extramedullary, cyst-like structure within the vertebral canal, between L7 and S3. Surgical removal of the cyst via a L7-S1 dorsal laminectomy was performed. Histopathological examination and additional immunohistochemistry of the excised structure indicated a probable ependymal cyst with a ciliated lining. The dog recovered well post-operatively, and at follow-up 3 weeks later had some improvement of his neurological signs. The paraparesis and pelvic limb ataxia had improved; however, the remaining neurological examination was similar to the pre-surgical examination. DIAGNOSIS: Extradural cyst. CLINICAL RELEVANCE: Spinal cysts can contribute to clinical signs that resemble other common chronic spinal cord diseases, such as intervertebral disc disease. Therefore, this disease should be considered as a differential when dealing with cases of progressive paraparesis and pelvic limb ataxia. This case report may potentially provide opportunities in the future for further understanding of the pathogenesis, behaviour, outcomes and subclassification of spinal cysts in dogs.

Secondary spinal cord changes and spinal deformity following traumatic spinal cord injury.

Secondary spinal cord changes can follow spinal cord injuries (SCIs). This retrospective study was to uncover the chronic secondary changes that affect the spinal cord following severe injuries and to evaluate the influence of residual spinal deformity in the development of posttraumatic spinal cord changes. Fifty-eight patients (39 male, 19 female) with complete traumatic SCI and recent Magnetic resonance imaging (MRI) follow-up were reviewed retrospectively. A minimum of 2 years duration between trauma and MRI study was required (mean 2.9 years [2.1-4.7]). Two groups of patients were formed: with spinal deformity (and or spinal canal compromise) and without spinal deformity (and or spinal canal compromise). MRI of the injured spine demonstrated four major types of spinal cord changes; these are spinal cord atrophy, myelomalacia, syrinx, and focal cyst formation. The correlation of these changes to the presence of spinal deformity and or spinal canal compromise was also studied. Twenty-three patients (40%) of the studied population had more than 30° kyphosis and or 50% compromise of the spinal canal. Chronic spinal cord changes occurred in 25 patients (43%), 17 of these changes occurred in patients with spinal deformity and the remaining 8 occurred in patients without spinal deformity or canal compromise (p ≤ .05). The prevalence of spinal cord atrophy and focal cysts was significantly higher in patients with residual deformity and or spinal canal compromise (p ≤ .05). The authors recommend proper spinal cord decompression and fixation for patients with complete SCI to reduce the chance of secondary SCI.

Histopathology of synovial cysts of the spine.

AIMS: Cystic lesions derived from the synovial and ligamentous structures of the spine have varied histological appearances. Not uncommonly, there is discrepancy between the clinicoradiological diagnosis and histology. Therefore, we sought to characterise the histological features of tissue submitted as 'synovial cysts' of the spine. METHODS AND RESULTS: Resected specimens of the spine labelled 'synovial cysts' and 'lumbar cysts' were histologically evaluated and classified on the basis of histopathological features. Seventy-five histological samples of spinal cysts were identified. Thirty-one were classified as synovial cysts (definite synovial lining), 28 showed pseudocystic degeneration of the ligamentum flavum, seven showed pseudocyst formation without evidence of a synovial lining or degeneration of the ligamentum flavum, and eight showed cyst contents only or no histological evidence of cyst wall for evaluation. Twenty-five cases (33%), especially those showing pseudocystic degeneration of the ligamentum flavum, were associated with very characteristic tumour calcinosis-like calcium deposition with a surrounding foreign-body giant-cell reaction. CONCLUSION: Histology of 'synovial cysts' of the spine shows varied types of cyst; a large proportion are not synovial-lined cysts, but rather show pseudocystic degenerative changes of the ligamentum flavum, often associated with very characteristic finely granular calcifications and a foreign-body giant-cell reaction. This may have implications not only for understanding the pathogenesis of these lesions, but also for their varied responses to non-surgical interventions.

Pediatric intraspinal arachnoid cyst: successful endoscopic fenestration. Illustrative case.

BACKGROUND: Intradural spinal arachnoid cysts (SACs) are a rare cause of spinal cord compression. Treatment is centered on decompression of the spinal cord via laminectomy or laminoplasty followed by resection or fenestration of the cyst. Although laminectomy or laminoplasty access may be needed to achieve the desired result, either procedure can be associated with more extensive surgical dissections and long-term spinal stability concerns, including postsurgical kyphosis. OBSERVATIONS: The authors present a case of a cervical intradural SAC in a 4-month-old girl presenting with symptomatic compression. The patient was treated by laminotomy and endoscopic fenestration of the SAC with resolution of symptoms and no disease progression 10 months postoperatively, when the patient was 14 months old. LESSONS: Microsurgical endoscopic fenestration of an intradural SAC can provide a less invasive means of treatment while avoiding the risks associated with more invasive approaches.

Iatrogenic Spinal Deformity Following Spinal Intradural Arachnoid Cyst Fenestration Despite Minimal Access With Laminoplasty and Endoscopy in a Pediatric Patient.

Spinal intradural arachnoid cysts (SAC) are non-neoplastic lesions that can cause spinal cord compression and present with myelopathy, radiculopathy, and/or back pain. Because these cysts typically span multiple levels, endoscopy could be a useful tool to avoid wide exposure. We present an 8-year-old patient with a history of gait imbalance and urinary incontinence who was found to have a SAC spanning C7 to T6 causing spinal cord compression. An osteoplastic laminoplasty was performed from T4 to T7 followed by ultrasonic verification of intracystic septations, dural opening, and cyst fenestration. A flexible endoscope was then introduced into the cystic cavity to guide complete rostral and caudal decompression of the arachnoid cyst. At six months follow-up, the patient was able to ambulate independently, but his urinary incontinence remained unchanged. Despite the combination of ultrasound and neuroendoscopy to minimize exposure, our patient suffered from worsening kyphosis from 36 degrees preoperative to 55 degrees postoperative and worsening scoliosis from 17 to 39 degrees which required treatment with a thoracolumbar sacral orthosis. Preoperative imaging demonstrated a reverse S-shaped scoliosis with the apex at T6 and T7 which were the levels included in the laminoplasty. This illustrates the need for careful preoperative risk stratification to avoid this postoperative complication.

Radiologic Characteristics of Spinal Hemangioblastomas in von Hippel Lindau Disease as Guidance in Clinical Interventions.

OBJECTIVE: Hemangioblastomas in the central nervous system are the most common manifestation of von Hippel-Lindau (VHL) disease. Because the growth rate of hemangioblastomas is unpredictable, regular follow-up is mandatory, focusing on clinical symptoms and imaging of the central nervous system. However, clinical symptoms may be subtle and nonspecific, and data about the relationship between the radiologic findings and clinical symptoms are sparse. This study aims to evaluate if and how findings of magnetic resonance imaging (MRI) regarding spinal hemangioblastomas are associated with symptoms of VHL disease, with special attention to peritumoral edema and spinal cysts. METHODS: Serial spinal MRI scans of 43 genetically or clinically established VHL patients with at least 2 years of follow-up were reevaluated to examine the volume, growth rate, and location of spinal hemangioblastomas and the presence, size, and growth rate of peritumoral edema and cysts. Findings were compared with clinical symptoms using the Fisher exact test. RESULTS: We observed a total of 77 spinal hemangioblastomas in 28 patients. Eight of the 28 patients showed peritumoral edema and spinal cysts, and 1 patient showed peritumoral edema without cyst formation; 6 of these 9 patients showed clinical symptoms. Both peritumoral edema and spinal cysts were associated with clinical symptoms (P = 0.023 and P = 0.011, respectively). CONCLUSIONS: The presence of peritumoral edema and/or spinal cysts shown on MRI in VHL patients with spinal hemangioblastomas is associated with symptoms in more than half of the patients and may alert the clinician to intensify clinical and radiologic surveillance.

Missed anterior sacral meningomyelocele presenting with obstructive uropathy.

A 2-month-old full-term female presented with a large anterior sacral meningomyelocele resulting in transient obstructive uropathy with bilateral hydronephrosis and acute kidney injury. After initial bladder decompression and surgical resection of the meningomyelocele, there was spontaneous resolution of bladder function confirmed with urodynamics. Anterior spinal meningomyelocele (ASM) is a rare neural tube defect that may present with urinary dysfunction secondary to compression of the bladder and sacral nerve roots or congenital defects to the bladder nervous supply. Obstructive uropathy due to ASM may spontaneously resolve after surgical resection.

Isolated lumbar intradural tailgut cyst: A case report and review of the literature.

Tailgut cysts are rare developmental cysts arising from remnants of the embryological postnatal gut. Despite being frequently located in the presacral space, isolated cases of aberrant locations have been reported, including, perirenal, perianal, and subcutaneous sites, with only two cases of subdural tailgut cysts reported to date. The clinical course is often marked by linear growth, causing compression of the adjacent structures, however malignant transformation with carcinomatous features has been previously described. Hereby the authors describe a case of an intradural extramedullary tailgut cyst in a 33-year-old man presenting with progressive low back pain and signs of autonomic dysfunction, including urinary retention and bowel incontinence. Whole-spine MRI revealed an intrathecal cystic lesion located at L2-L3 level exhibiting hyperintensity on T2-weighted images not enhancing when contrast was administered. Laminectomy followed by tumor excision was performed and pathological analysis confirmed the diagnosis of tailgut cyst.

Waste not, want not: Report of a completely calcified C1-C2 juxtafacet cyst and literature review.

BACKGROUND: Calcified juxtafacet cysts in the cervical spine are extremely rate. Such symptomatic cysts commonly cause neck pain, radiculopathy, or even myelopathy. MR and CT studies typically document cord/ root compression. On occasion, some of these cysts will spontaneously regress, while many others may warrant surgical removal. CASE DESCRIPTION: A 70-year-old male presented with a 2-year history of a progressive tetraparesis. The preoperative MR/CT studies showed a C1-C2 left extradural mass occupying more than half of the spinal canal. On MR, it was homogeneously hypointense on both T1- and T2-weighted images, while the CT showed a calcified cyst. Intraoperative and histopathological findings documented a calcified cervical juxtafacet cyst (i.e. ganglion subtype) that was fully excised without sequelae. CONCLUSION: C1-C2 juxtafacet cervical cyst should be considered when a patient presents with myelopathy due to a calcified MR/CT documented paraspinal lesion contributing to significant cervical cord/root compression.

Diagnostic Dilemma in Discriminating Between Spinal Neurenteric Cysts and Simple Arachnoid Cysts Based on Embryogenesis and Surgical Correlation.

BACKGROUND: Neurenteric cyst (NEC) is a rare intradural spinal tumor, but a correct preoperative diagnosis remains challenging. A misdiagnosis of arachnoid cyst (AC) often leads to conflicting surgical management and significantly higher recurrence. CASE DESCRIPTION: We report the case of a 26-year-old woman who presented with progressive spastic quadriparesis with myelopathy below the C4 level, which was caused by a ventral intradural extramedullary cystic tumor at the C3-4 level. Magnetic resonance images showed the cystic content as identical to cerebrospinal fluid, which prompted the tentative diagnosis of spinal AC. Surgical fenestration was scheduled. However, intraoperative findings of a thick-walled cyst and severe adhesion to the neural structure without a history of trauma and inflammation were more compatible with the pathogenesis of an NEC. Because of the high recurrence rate after an incomplete resection of an NEC, we did a complete resection of the cyst with adhesive rootlets instead. Pathology analysis and immunohistochemical staining confirmed the diagnosis of an endodermal-derived NEC. CONCLUSIONS: NECs must be differentiated from ACs because they are different diseases and require different surgical management. In cases with clear cystic content, however, the diagnosis is likely to be AC, but a thick cystic wall and structural adhesions should suggest the differential diagnosis of NEC. Gross total removal of NECs should be attempted to reduce NEC recurrence.

Short- and long-term outcome and magnetic resonance imaging findings after surgical treatment of thoracolumbar spinal arachnoid diverticula in 25 Pugs.

BACKGROUND: There is a successful outcome after surgical management of spinal arachnoid diverticula (SAD) in up to 82% of cases. HYPOTHESIS/OBJECTIVES: We hypothesized that Pugs have favorable short-term and poor long-term prognosis after surgical treatment of thoracolumbar SAD. The aim of the present investigation was to describe clinical findings, short- and long-term outcomes, and follow-up magnetic resonance imaging (MRI) findings in Pugs with thoracolumbar SAD. ANIMALS: Twenty-five client owned Pugs with 12-month follow-up information after surgical treatment of thoracolumbar SAD. METHODS: Multicenter retrospective case series. All medical records were searched for Pugs diagnosed with SAD. Data regarding signalment, history, surgical procedure, outcome, histopathology, and follow-up MRI results were extracted. RESULTS: Mean age at presentation was 7.32 (range 2-11) years, 80% were males. Short-term outcome was available in 25 dogs, and improvement was confirmed in 80% of dogs. Long-term outcome was available in 21 dogs, and deterioration was confirmed in 86% of cases, with late-onset recurrence of clinical signs after initial postsurgical improvement affecting 85% of Pugs. A moderate correlation (r = 0.50) was found between duration of clinical signs and outcome. In 8 dogs with deteriorating clinical signs, follow-up MRI revealed regrowth of the SAD in 2 cases, new SAD formation in 2 cases, and intramedullary T2W hyperintensity/syringomyelia in 6 cases. CONCLUSIONS AND CLINICAL IMPORTANCE: This study suggests that Pugs with thoracolumbar SAD do not have a favorable long-term prognosis after surgical treatment for reasons yet to be determined.

Pediatric Symptomatic Sacral Extradural Arachnoid Cyst: Surgical Management Review.

Spinal arachnoid cyst (SAC) constitutes about less than 1% of all spinal tumors. It commonly occurs in third to fifth decades of life and mostly located in the thoracic region. Sacral extradural arachnoid cyst in the pediatric age group is an extremely rare location of occurrence. A such rare case of extradural arvwachnoid cyst of the sacral region, who presented with urinary incontinence is reported. The patient was managed surgically with laminectomy at S1-S3 vertebral level and complete excision of the extradural sacral arachnoid cyst with closure of transdural communication. The patient tolerated surgical procedure very well with regained bladder sphincter control at 6 months of follow-up. Early diagnosis and surgery are recommended for the treatment of symptomatic pediatric sacral arachnoid cysts.

Isolated Dorsal Thoracic Neuroenteric Cyst with Spinal Cord Compression: Case Reports in Pediatrics.

BACKGROUND: Neuroenteric cysts are rare developmental anomalies of the central nervous system that account for approximately 0.7%-1.3% of all spinal cord lesions. The majority of spinal neuroenteric cysts are located ventral to the spinal cord. Dorsally located neuroenteric cysts are rare and are almost always associated with other spinal anomalies. Isolated dorsal spinal neuroenteric cysts, without other associated spinal anomalies, are extremely rare. These lesions can be mistaken for the more commonly encountered spinal cysts (e.g., arachnoid cysts, ependymal cysts, cystic teratomas). CASE DESCRIPTION: We present the first pediatric case of a 9-month-old boy with an isolated dorsal thoracic neuroenteric cyst and cord compression that underwent surgical resection. The patient tolerated the procedure well and remained neurologically stable after surgery. Pathology was consistent with a neuroenteric cyst. Complete resection was not achieved at the time of surgery. In retrospect, knowing the lesion is a neuroenteric cyst, a more aggressive approach aiming for a complete surgical resection should have been undertaken. To our knowledge, only 2 adult cases of isolated dorsal spinal neuroenteric cysts have been reported in the literature. CONCLUSIONS: Neuroenteric cysts can occur dorsal to the spinal cord without any associated spinal anomalies. The gold standard treatment for neuroenteric cysts is surgical resection, and the goal of surgery is complete resection.

Nuances in Localization and Surgical Treatment of Syringomyelia Associated with Fenestrated and Webbed Intradural Spinal Arachnoid Cyst: A Retrospective Analysis.

INTRODUCTION: Intradural spinal arachnoid cysts (SACs) are among many etiologies for syringomyelia. Consequentially, neurologic symptoms arise such as pain, gait disturbance, and bladder dysfunction. Identification of SAC on magnetic resonance imaging (MRI) can be challenging, as SACs can be fenestrated or in the form of fine webs. METHODS: Imaging and clinical data for 7 patients who underwent surgical treatment for SAC associated with syringomyelia were reviewed. All previous publications of this pathology were reviewed via MEDLINE search. RESULTS: Seven patients with a mean age 59 years were found to have a SAC causing syringomyelia. Intraoperative exploration confirmed SAC appearances of fine webs or a fluid-filled loculation impinging on the spinal cord. Common presentations were back pain, gait disturbance, and bladder incontinence. Diagnosis was made by MRI, although in 3 cases, the SAC was not identified on the initial review. Computed tomography myelogram was performed in one case due to the enlarged syringomyelia and lack of obvious spinal cord compression. Thoracic laminectomy/laminoplasty was performed for all patients, centered at the level of a subtle indentation of the cord; the syringomyelia proper was not directly addressed. Postoperatively, all patients had complete resolution of their symptoms with MRI demonstrating resolution of the syringomyelia. CONCLUSIONS: Careful evaluation of the MRI can demonstrate subtle indentation of the cord at the caudal or cephalad end of the syringomyelia and may obviate the need for additional imaging. Meticulous arachnoid dissection and establishment of good CSF flow is sufficient for resolution of the syringomyelia, averting the need for more aggressive procedures.

Successful percutaneous management of symptomatic central posterior epidural Baastrup cyst: a potential surgical sparing option?

BACKGROUND CONTEXT: Neurogenic claudication from posterior epidural extension of a Baastrup interspinous bursal cyst is rare. Surgical decompression is the gold standard of treatment. This case report describes successful percutaneous treatment with good early clinicoradiological outcome. PURPOSE: This study aimed to describe the successful percutaneous treatment of a central posterior epidural Baastrup cyst causing neurogenic claudication. STUDY DESIGN: This is a case report study. METHODS: A 62-year-old man presented with neurogenic claudication on a background of previous lymphoma treated with chemotherapy, chronic obstructive pulmonary disease, chronic venous insufficiency, and obesity. Conservative therapy with narcotic analgesia had failed, with new requirement of a walking aid and marked reduction in walking distance. Magnetic resonance imaging confirmed severe L3-L4 canal stenosis from central posterior epidural extension of a Baastrup interspinous bursal cyst. Under conscious sedation, initial percutaneous computed tomography (CT)-guided interspinous bursography indirectly opacified the cyst and facilitated trans-laminar direct needle access to the epidural cyst. Aspiration was performed before needle fenestration and epidural steroid injection. RESULTS: Six-week review revealed significant improvement in pain and mobility, with no analgesic or walking aid requirement, and restoration of the patient's baseline walking distance. At 3 months, repeat magnetic resonance imaging (MRI) confirmed significant reduction in cyst size as the mediator of the treatment effect. Improvement in back and leg symptoms was durable at 24-month follow-up. CONCLUSIONS: In selected cases, percutaneous treatment of symptomatic central posterior epidural cysts as part of Baastrup phenomena may be feasible. This treatment approach avoided general anesthesia, avoided the procedural risks of surgical decompression, and was performed in the outpatient setting, with good early clinicoradiological outcome. This may emerge to be a surgical sparing option or an alternate to continuing conservative therapy in patients who are poor surgical candidates.

Spinal Neurenteric Cyst in Association with Klippel-Feil Syndrome: Case Report and Literature Review.

BACKGROUND: Spinal neurenteric cysts, also known as endodermal or enterogenous cysts, are rare epithelium-lined structures of presumed endodermal origin. Congenital vertebral anomalies are frequently seen in patients with neurenteric cysts, most typically anterior spina bifida, butterfly vertebrae, and hemivertebrae. However, few series of intraspinal neurenteric cysts accompanied by Klippel-Feil syndrome have been reported previously. CASE DESCRIPTION: Our purpose is to present the clinical, radiological, and histological results of a 29-year-old patient with a spinal neurenteric cyst associated with Klippel-Feil syndrome and to review previous reported cases of neurenteric cysts associated with Klippel-Feil syndrome. In our patient, cervical radiography demonstrated C5-T1 vertebral fusion and magnetic resonance imaging revealed a large intradural cystic mass. The cystic lesion was removed successfully, and it was histopathologically diagnosed as a neurenteric cyst. CONCLUSION: Neurenteric cysts should always be considered in the differential diagnosis of an intraspinal cystic mass seen in the setting of vertebral anomalies.

Dural dissection cyst: a more accurate term for extradural meningeal cyst.

AIMS: Spinal extradural meningeal cyst (EMC) aetiology remains unclear. Based on our in-depth analysis of EMC clinical characters, we propose the alternative term 'dural dissection cyst' (DDC), which is more consistent with its aetiology and pathological manifestations. METHODS: We examined the clinical, imaging and operative findings of four patients with spinal EMC (type I A) and analysed the aetiology and treatment of the cyst. RESULTS: Spinal DDC was observed between T10 and L3 in our series. Patients presented with low back pain, lower extremity numbness and weakness, and segmental muscle atrophy. Small clefts were found on the inner wall of all cysts. Microscopic suture of the cleft successfully improved patient's symptoms and neurological deficits. CONCLUSIONS: Spinal EMC (type I A) is characterized by dural dissection, so the term DDC can best reflect its aetiology. Because it is a dissection cyst, the most reasonable treatment is to suture the fistula.

Bibrachial amyotrophy and ventral spinal cyst associated with myelomalacia and intracranial hypertension.

It has been recently recognised that patients with ventral intraspinal fluid collections secondary to cerebrospinal fluid leaks can present with bibrachial amyotrophy or mimic Hirayama disease. Here we present two further patients that expand the clinical spectrum of this disorder to include association with myelomalacia and intracranial hypertension.