脊髄空洞症

PURPOSES: To evaluate medium-long term bowel function, health-related quality of life (HRQoL), and related prognostic factors in patients with complete Currarino syndrome (CS). METHODS: A retrospective cohort study of patients with complete CS treated at Beijing Children's Hospital between January 2008 and December 2023 was conducted. Bowel function and HRQoL were assessed cross-sectionally in December 2025 using the Rintala Bowel Function Score and the Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0), respectively. Multivariable linear regression analysis was performed to identify independent predictors of bowel function. RESULTS: Thirty-two patients with a median follow-up age of 9.0 (6.3, 9.8) years were included. Total Rintala score of these patients was 16.5 (12.0, 19.0), including 78.1% (25/32) with soiling and 50.0% (16/32) with constipation, and total PedsQL 4.0 score was 96.8 (92.7, 98.9). Multivariable analysis identified total sacral dysplasia (β = -3.86, p = 0.026) and perioperative complications related to presacral mass (β = -6.24, p = 0.002) as independent predictors of poorer bowel function. Surgical resection for megarectosigmoid did not improve overall bowel function and was associated with a higher rate of soiling compared with conservative management (100.0% vs. 44.4%, p = 0.034). CONCLUSIONS: Patients with complete CS had significant medium-long term bowel dysfunction, primarily determined by sacral dysplasia and perioperative complications related to presacral mass. In complete CS patients with megarectosigmoid, surgery did not improve overall bowel function.

INTRODUCTION: Subacute post-traumatic ascending myelopathy (SPAM) is defined as a neurological deterioration extending several levels above the initial spinal cord injury (SCI) within the first few weeks post-injury, without associated mechanical instability or syringomyelia. This report details three cases of SPAM to highlight this condition. CASES PRESENTATION: We present three case reports who sustained SPAM over the last 7 years at the Hospital Universitari Vall d'Hebron in Barcelona (Spain). The first patient was a 41-year-old male with a traumatic C5 SCI (AIS A), diagnosed of SPAM 35 days post-injury with a C3 SCI (AIS A) and treated with descending taper of dexamethasone (Dx). The second patient was a 45-year-old female with a traumatic C7 SCI (AIS A), diagnosed of SPAM 39 days post-injury with a C4 SCI (AIS A) and treated with a 3-day methylprednisolone (MP) bolus. The third patient was a 40-year-old male with a traumatic T7 SCI (AIS B), diagnosed of SPAM 11 days post-injury with a T5 SCI (AIS A) and treated with a 7-day MP bolus. The third patient showed improvement in AIS classification. A literature review of identification and treatment of SPAM was conducted. DISCUSSION: SPAM is a severe and rare complication that should be considered within weeks following an acute traumatic SCI. There is no consensus in the literature regarding the treatment of corticosteroids related to SPAM. In the three cases presented, methylprednisolone was associated with better outcomes than dexamethasone; however, given the small sample size, this observation remains preliminary and hypothesis-generating.

Spinal arachnoid webs are rare intradural extramedullary lesions that most commonly occur in the thoracic spine and may cause focal dorsal spinal cord compression, cerebrospinal fluid flow obstruction, syringomyelia, and progressive myelopathy. Diagnosis can be delayed because symptoms are often nonspecific and the arachnoid web itself may be difficult to directly visualize on standard magnetic resonance imaging. The characteristic "scalpel sign," defined by focal dorsal indentation of the spinal cord, is a key imaging feature. We present a case of a 47-year-old male patient with progressive mid-thoracic pain, left thoracic sensory disturbance, and bilateral foot numbness. Magnetic resonance imaging demonstrated a thoracic syrinx and focal dorsal indentation of the spinal cord at T8-T9 consistent with the scalpel sign. High-resolution constructive interference in steady-state imaging demonstrated a dorsal intradural arachnoid band, supporting the diagnosis of thoracic spinal arachnoid web. The patient underwent T8-T9 laminectomy, durotomy, and microsurgical excision of the arachnoid web. Postoperatively, he experienced resolution of sensory symptoms within two weeks, and follow-up imaging demonstrated interval reduction of syringomyelia. This case highlights the importance of recognizing thoracic arachnoid webs as an underdiagnosed cause of myelopathy and syringomyelia. Early recognition of characteristic imaging findings and definitive microsurgical treatment can result in favorable clinical and radiographic outcomes.

BACKGROUND: Spontaneous intracranial hypotension (SIH) is an underrecognized yet increasingly diagnosed cause of orthostatic headaches. Reduction of intracranial CSF volume and pressure produces characteristic imaging findings such as diffuse pachymeningeal enhancement and cerebellar tonsillar descent, mimicking a Chiari malformation type I. Distinguishing SIH from other causes of hindbrain herniation is critical for prompt recognition and targeted treatment to resolve symptoms and radiological abnormalities. OBSERVATIONS: A 55-year-old male patient presented with a history of persisting postural headaches, back pain, and sensory loss. MRI of the spine demonstrated a subtle ventral epidural fluid collection extending along the cervical and thoracic spine. Intraoperative digital subtraction myelography confirmed a CSF leak site at the T3-4 level due to a dural defect. Under the same anesthetic, a posterior T3-4 laminectomy and intradural repair of the ventral dural defect were performed. The patient recovered with complete resolution of symptoms and no neurological deficits or weakness. Postoperative MRI showed resolution. LESSONS: Identifying a pathological defect can be challenging in SIH, especially in chronic cases that mimic Chiari malformation type I due to tonsillar decent. The authors illustrate the clinical, radiological, and surgical framework utilized at their institution for managing a chronic SIH case due to a ventral dural defect. https://thejns.org/doi/10.3171/CASE26369.

BACKGROUND Charcot neuroarthropathy (CN) is a rare and easily misdiagnosed neuropathic arthropathy, often secondary to conditions such as syringomyelia and diabetes mellitus. Its clinical course is insidious, making early diagnosis challenging, and the presence of infection further complicates management. CASE REPORT This article reports a case of a 48-year-old male patient with elbow CN and secondary infection due to syringomyelia. The patient presented with swelling, erythema, and elevated skin temperature of the left elbow, but without significant pain. Imaging studies revealed elbow dislocation accompanied by extensive bony destruction. Laboratory test results showed a markedly elevated high-sensitivity C-reactive protein level (62.10 mg/L), and bacterial culture identified Staphylococcus aureus infection. After thorough debridement, the patient was treated with vacuum sealing drainage (VSD) combined with intravenous vancomycin for infection control. Following 2 sessions of VSD therapy, the infection was effectively controlled, with good granulation tissue formation, significant reduction in joint swelling, and negative bacterial culture results. At the 6-month follow-up, the elbow remained stable without signs of recurrent infection; the patient reported mild pain and limited range of motion, and definitive reconstructive surgery was deferred due to ongoing risk of infection and patient preference. CONCLUSIONS This case suggests that early and thorough debridement combined with VSD is an effective local treatment strategy for CN patients with infection. This approach aids in infection control and promotes wound healing, thereby creating favorable conditions for subsequent functional recovery. VSD, through continuous negative pressure drainage and irrigation, effectively removes necrotic tissue and exudate, improves local microcirculation, and demonstrates good application value in managing CN-related infections.