Surgical treatment and outcome of haematomyelia with a traumatic cause in a dog and a cat.

Two surgically treated haematomyelia cases were documented. One dog and one cat were presented for acute progressive paraplegia following a fall from height incident. Neurological examinations suggested a L4-S3 myelopathy in both cases. Radiography and magnetic resonance imaging in both cases revealed no fracture or subluxation of the spine, but well-defined intramedullary mass lesions in lower lumbar regions compatible with haemorrhage and haematoma formation. Exploratory surgeries were performed over the lesions. Dark-red friable masses were removed via myelotomy. Histopathological examinations revealed organizing haematomas at the acute to subacute stage. Postoperatively, both cases improved and regained ambulation. The dog walked normally but remained urinary and faecal incontinent 9 months after the surgery. The cat was continent and ambulatory with a paraparetic gait 5 months after the surgery. In both cases, the outcomes and the patients' quality of life were considered satisfactory by the owners.

Spontaneous Hematomyelia Associated with the Use of Non-vitamin K Antagonist.

Vitamin K antagonists have been frequently prescribed as anticoagulants with the potential side effect of spontaneous hematomyelia with a poor prognosis. However, to our knowledge, there has been no report of spontaneous hematomyelia combined with the use of a non-vitamin K antagonist. A 63-year-old man presented with left leg weakness, impaired sensation, and urinary retention while taking rivaroxaban (non-vitamin K antagonist) for 4 months for atrial fibrillation. Anticoagulant agents were discontinued. Methylprednisolone pulse therapy was administered without surgical hematoma evacuation. Three months after the initial development of the hematomyelia, the symptoms improved to grade 5 for both lower extremities, and there was complete recovery in sensory and urinary functions. This might be the first description of a complete recovery of neurologic deficits without hematoma evacuation in spontaneous hematomyelia patients caused by non-vitamin K antagonist therapy.

Primary spinal atypical teratoid/rhabdoid tumour presenting with hematomyelia and subarachnoid haemorrhage-a case report.

Atypical teratoid/rhabdoid tumours (AT/RTs) are highly aggressive and uncommon malignant tumours of the central nervous system (CNS) affecting children younger than 3 years of age. Primary spinal cord involvement is an extremely rare presentation. AT/RTs show necrosis and haemorrhages on histopathology frequently. However, spinal atypical teratoid/rhabdoid tumour (AT/RT) with hematomyelia and spinal subarachnoid haemorrhage (SAH), as seen in our case, has never been reported in the literature in the paediatric age group. We report a case of primary spinal AT/RT in a 3-year-old male child presenting acutely with hematomyelia and spinal SAH and try to elucidate its pathophysiological basis.

Syringomyelia: a practical, clinical concept for classification.

BACKGROUND: The term syringomyelia describes many pathogenetically different disorders, and a variety of attempts to group these based on different criteria have been proposed in the literature. As a consequence a lack of consensus regarding classification and terminology exists. This inconsistency extends to the ICD-10 classification of diseases in regards to syringomyelia (G95.0) and hydromyelia (Q06.4). We propose a new unifying concept for classification that also incorporates diagnostics and treatment. METHODS: The PubMed online database was used to gain a general overview of the existing pathogenetic theories in relation to syringomyelia. Illustrative cases at our department were included and similar cases of the literature were found using the PubMed database. All material was reviewed with main focus on the classification and terminology used. RESULTS: Despite syringomyelia (G95.0) and hydromyelia (Q06.4) existing as independent ICD-10 entities, we have shown that the use of classifying terminology for fluid-filled cavities in the spinal cord is indiscriminate and inconsistent. Even though a general agreement on the believed pathogenetic mechanism exists, and the general treatment methods are used in accordance with this mechanism, the terminology fails to function as a simple and universal link between theory and treatment. CONCLUSIONS: We propose a new causal concept for an ICD classification with syringomyelia (G95.0) as the only describing terminology, thus abandoning the use of hydromyelia (Q06.4). Syringomyelia is divided into five subgroups according to the associated pathologies. The classification is based on applied diagnostics and serves as a clinical guidance for treatment.

Spinal extradural arteriovenous fistulas: clinical article.

OBJECT: Our understanding of spinal extradural arteriovenous fistulas (eAVFs) is relatively limited. In this study the authors aimed to provide the demographics, natural history, and treatment results of these rare lesions. METHODS: The authors performed a pooled analysis of data in the PubMed database through December 2012. Individualized patient data were extracted to elucidate demographic, clinical, and angioarchitectural features of spinal eAVFs as well as outcomes following different treatment strategies. RESULTS: Information on 101 patients was extracted from 63 eligible studies. The mean patient age was 45.9 years, and there was no significant overall sex predilection. Only 3% of the lesions were incidental, whereas 10% occurred in patients who had presented with hemorrhage. None of the 64 patients with at least 1 month of untreated follow-up sustained a hemorrhage over a total of 83.8 patient-years. Patients with lumbosacral eAVFs were significantly older (mean age 58.7 years, p < 0.0001), were significantly more often male (70% male, p = 0.02), had significantly worse presenting Aminoff-Logue motor and bladder scores (p = 0.0008 and < 0.0001, respectively), and had the greatest prevalence of lesions with intradural venous drainage (62% of cases, p < 0.0001). Neurofibromatosis Type 1 (30% of cases, p < 0.0001) and subarachnoid hemorrhage (9% of cases, p = 0.06) were associated with and exclusively found in patients with cervical eAVFs. The overall complete obliteration rate was 91%. After a mean follow-up of 1.7 years, the clinical condition was improved in 89% of patients, the same in 9%, and worse in 2%. Obliteration rates and outcome at follow-up did not significantly differ between surgical and endovascular treatment modalities. CONCLUSIONS: Spinal eAVFs are rare lesions with a low risk of hemorrhage; they cause neurological morbidity as a result of mass effect and/or venous hypertension. Their treatment is associated with a high rate of complete obliteration and improvement in preoperative symptoms.

Surgical treatment of spinal vascular malformations performed using intraoperative indocyanine green videoangiography.

This study aims to evaluate the benefits of intraoperative indocyanine green (ICG) videoangiography and associated surgical outcomes of patients with spinal vascular malformations. ICG videoangiography was used during 24 surgical interventions to treat spinal vascular malformations at the Beijing Tiantan Hospital from August 2009 to May 2011. The vascular malformations were removed or the fistulae were occluded with the assistance of ICG videoangiography. The completeness of fistula clipping or nidus extirpation and each patient's neurological status were evaluated. Among these 24 patients, there were seven with spinal dural arteriovenous fistulae, five glomus arteriovenous malformations, one juvenile arteriovenous malformation, nine perimedullary arteriovenous fistulae, and two perimedullary arteriovenous fistulae in combination with perimedullary arteriovenous malformations. Intraoperative ICG videoangiography confirmed the definite clipping of the fistulous points and complete removal of intramedullary arteriovenous malformations in all but one patient. All patients had satisfactory preservation of spinal cord blood supply and venous return. No adverse effects or complications related to ICG videoangiography occurred. Three patients were lost to follow up; 21 patients were followed clinically with a mean follow up of 7.5 months. The neurological deficits completely resolved in six patients, improved significantly in 10, remained stable in two, and were aggravated in three patients. Our experience shows that intraoperative ICG videoangiography offers useful information on the pathological and physiological vascular anatomy encountered during surgery for spinal vascular malformations.

Cavernous malformations of the central nervous system combined with cutaneous vascular lesions due to KRIT1 mutation: a case report.

Cavernous malformations (CMs) of the central nervous system can occur in a sporadic condition or as a familial form with an autosomal-dominant inherited pattern. Apart from a family history, some clinical features may help to identify familial CMs. We demonstrate clinical, neuroradiological, pathological, and genetic data of a patient with cerebral and spinal CMs. The presence of multiple cerebral CMs and distinct cutaneous vascular lesions, including hyperkeratotic cutaneous capillary-venous malformations, in this patient suggested familial CMs. A genetic study confirmed a nonsense mutation (c.1708A>T) in the KRIT1 gene.

Bilateral late remote cerebellar hemorrhage as a complication of a lumbo-peritoneal shunt applied after spinal arteriovenous malformation surgery.

BACKGROUND/OBJECTIVE: Cerebellar hemorrhage is a very infrequent and unpredictable complication of spinal surgery. To the best of our knowledge, cerebellar hemorrhage resulting from the insertion of a lumbo-peritoneal shunt through which cerebrospinal fluid (CSF) is slowly drained has not been documented to date. METHODS: Case report. RESULTS: A 47-year-old woman presented with lower extremity weakness. Spinal arteriovenous malformation was diagnosed, and she underwent surgery. Her neurologic status improved; however, CSF collected subcutaneously as a cyst and leaked 21 days after surgery. The patient underwent urgent surgery during which the dural defect was repaired and a lumbo-peritoneal catheter was put in place to treat the CSF leakage. The lumbo-peritoneal drainage system was removed when bilateral cerebellar hemorrhage was seen 12 days later. Physical therapy was stopped, and conservative treatment was initiated consisting of bed rest, analgesics, sedatives, and careful monitoring of blood pressure. The patient's headache gradually resolved; physical therapy was restarted to rehabilitate this patient with paraparesis. CONCLUSIONS: Remote cerebellar hemorrhage seems to be life threatening and entails significant morbidity. Cerebellar symptoms, and even a late sudden headache after spinal surgery, may be signs of remote cerebellar hemorrhage, which is a rare complication.

Intramedullary cavernoma presenting with hematomyelia: report of two girls.

INTRODUCTION: Less than 20 children with intramedullary cavernoma (ImC) have been reported in the English literature; however, cases with an unfavorable outcome may be underreported. Whereas these are predominantly boys, we report two girls who presented with hematomyelia (one cervical, one thoracic) and an acute, severe neurological deficit. CASE MATERIAL: A 10-year-old girl complaining about lower thoracic pain for several days suddenly developed lower body dysesthesias and paraparesis. Magnetic resonance (MR) demonstrated hematomyelia (T8-T11), intramedullary edema (T6-L1), and an ImC at T9-T10. Within an hour, she progressed to paraplegia and was therefore operated immediately. She slowly recovered regaining independent ambulation and continence. MR after 2 years shows no recurrence. A 7-year-old girl suddenly developed cervicalgia and paresis of her left arm and leg. MR demonstrated hematomyelia and an ImC at C4-C6. She gradually recovered with minimal residual deficit at 3 months and was subsequently operated uneventfully. Multiple cerebral cavernomas and a familial autosomal cavernous malformation syndrome were diagnosed. The following 1.5 years, she complained of intermittent cervicalgia and left brachial dysesthesias, with MR suggesting active residual cavernoma. Interestingly, her complaints gradually disappeared, and she is currently asymptomatic. MR after 3.5 years shows minimal cord swelling no longer suggesting active residual cavernoma. CONCLUSION: With adequate surgical treatment either in the acute phase in case of dramatic deterioration or after clinical recuperation, prognosis of symptomatic ImC may be surprisingly good. However, subtotally resected lesions and/or syndromal cases may recur, requiring further treatment. Definitive answers await more cases with longer follow-up.

Endoscopically assisted closure of an anteriorly based spinal pial arteriovenous fistula.

The intraoperative use of an endoscope to localize an anteriorly based spinal pial arteriovenous fistula with minimal cord retraction and vessel manipulation is described.

Infarct presenting with a combination of Wallenberg and posterior spinal artery syndromes.

This is the first report of a patient presenting with a combination of Wallenberg and posterior spinal artery syndromes. The patient developed right hemiplegia and sensory disturbances on the right side of the face and over the whole body. MRI showed infarcts of the cerebellum, medulla oblongata, and upper cervical cord. These lesions were in the territory of the right posterior inferior cerebellar artery (PICA) and the right posterior spinal artery (PSA). The combination was due to severe stenosis of the right vertebral artery.