Unveiling Dandy-Walker syndrome: A surprising twist in the tale of acute hydrocephalus and Down syndrome child.

The correlation between Down syndrome and Dandy-Walker syndrome is an exceptionally uncommon occurrence. To date, only four cases have been documented. All previously reported cases involved individuals under the age of 37 months, with prenatal or birth diagnoses. Additionally, most of these cases displayed a limited life expectancy and experienced poor developmental outcomes. In this report, we present the first-ever instance of an 11-year-old male patient, previously undiagnosed with Dandy-Walker syndrome, who presented with acute intracranial hypertension. Magnetic Resonance Imaging revealed an active hydrocephalus caused by a Dandy-Walker malformation. The patient's condition was effectively managed through the implementation of a ventriculo-cysto-peritoneal shunt. This case highlights the coexistence of Dandy-Walker syndrome and Down syndrome in an asymptomatic young patient. Furthermore, it demonstrates that active hydrocephalus in such cases can be successfully addressed through either endoscopic third ventriculostomy or ventriculo-cysto-peritoneal shunt procedures.

Primary Extra-axial Glioblastoma: Case Report and Literature Review

Glioblastoma multiforme (GBM) is the most frequent and most aggressive primary brain tumor in adults,mainly located in the cerebral hemispheres. In the literature, few cases of primary GBM have been reported to have radiographic and intraoperative features of extra-axial lesions, leading to a diagnostic dilemma. Despite the advances in imaging modalities, the diagnosis of GBM can be challenging, and it is mainly based on the histopathologic confirmation of the excised tumor. We describe the case of a 76- year-old previously healthy female patient who presented to our hospital due to speech disturbances and cognitive impairment. The diagnosis of the tumor type on magnetic resonance imaging (MRI) was difficult, as the findings were suggestive of a malignant meningioma due to the heterogeneous enhancement of a dural-based mass with a dural tail sign. Moreover, the intraoperative findings revealed an extra-axial mass attached to the dura. A histological examination confirmed the diagnosis of glioblastoma with arachnoid infiltration. The patient underwent adjuvant radiotherapy and concomitant temozolomide treatment, she had clinical improvement postoperatively, and was stable during the six months of follow-up. Glioblastoma should be considered in the differential diagnosis of primary extra-axial mass with atypical and malignant features, especially in elderly patients.

Cervical Hyperextension Deformity After Sagittal Balance Correction in Patient with Congenital Limb-Girdle Myopathy: Surgical Technique and Review of Literature.

BACKGROUND: There is no gold standard surgical treatment for cervical hyperextension deformity, especially in case of muscular dystrophy. Special considerations and caution should be taken as they carry a high risk of early mortality and spinal cord injury. Only a few case reports are available in the literature. CASE DESCRIPTION: We report a case of surgical correction of an iatrogenic cervical hyperextension deformity following sagittal balance correction in a patient with congenital limb-girdle myopathy. The patient was successfully treated by posterior cervical release and fusion after verification of the range of motion, reducibility of the deformity, and absence of any positional spinal cord compression with dynamic radiographic examination and preoperative magnetic resonance imaging in the desired postoperative position. CONCLUSIONS: We suggest posterior cervical release and fusion in case of a radiologically and clinically reducible cervical hyperextension deformity under both motor and sensory spinal evoked potential monitoring. In cases of longstanding, rigid, nonreducible cervical hyperextension, laminectomy and concomitant duroplasty could be considered.

Rare Complication of Percutaneous Technique: Intradural Cement Leakage and Its Surgical Treatment.

Popularity of percutaneous vertebroplasty and vertebral augmentation to treat vertebral compression fractures is increasing. Cement leakages are relatively common, but intradural cement leakage is rare. Few cases of intradural cement leakage have been reported in the literature, and emergency surgery has been reported to be <1%. A 64-year-old man with osteolytic vertebral compression fracture at L1, caused by a malignant tumor, had undergone a vertebral augmentation by craniocaudal procedure. Neurologic examination after the surgery revealed paralysis in both legs immediately postoperatively. Computed tomographic imaging revealed a large cement leakage into the spinal canal. The patient was referred to our department for emergency management. He was rapidly scheduled for surgical decompression by laminectomy. First, stabilization by a secure posterior short fixation was done. The laminectomy showed that insertion of a needle through the pedicle had breached the dura and had caused intradural cement leakage. As shown in the Supplementary Video, the piece of intradural cement was finally removed after posterior durotomy. This case demonstrates that even if percutaneous vertebroplasty is a relatively safe technique, it should be performed by well-trained physicians and with great care to prevent disabling complications.

Mid-Term Clinical and Radiological Outcomes After Kyphoplasty in the Treatment of Thoracolumbar Traumatic Vertebral Compression Fractures.

BACKGROUND: The management of thoracolumbar posttraumatic compression fractures varies widely among centers, and there is no real consensus as to the optimum approach. The objective of our retrospective study was to detect the progression of vertebral kyphosis in nonosteoporotic patients treated by balloon kyphoplasty (KPB) who presented with recent compression fractures of the thoracolumbar region. METHODS: In this retrospective study, we investigated the evolution of vertebral and regional kyphosis in 77 patients treated by KPB for compression vertebral fractures (Magerl A) between 2007 and 2011. All treated patients, even those lost to follow-up, were included in our statistical analysis. RESULTS: In the 77 patients, a 2.4° deterioration of vertebral kyphosis (P = 0.0004) and a 4.5° worsening of regional kyphosis (P < 0.0001) were observed at the end of the follow-up period. No statistical correlation between the worsened kyphosis and the deterioration of long-term pain was identified. The mean visual analog scale score was 2.5, associated with very low disability on functional scores. A3-2 and A3-3 fractures are characterized by worsening vertebral and regional kyphosis. CONCLUSIONS: The paucity of studies of posttraumatic vertebral compression fractures in the scientific literature explains the lack of consensus regarding the optimum treatment approach. Postoperative results with KPB favor vertebral and regional kyphosis stability. KPB remains indicated in this situation except in cases of for burst fracture.

Improvement in Sagittal Balance After Decompression Surgery without Fusion in Patients with Degenerative Lumbar Stenosis: Clinical and Radiographic Results at 1 Year.

OBJECTIVE: To evaluate modifications in static spinal status after posterior decompression surgery without fusion in patients with symptomatic central canal stenosis. METHODS: From November 2014 to May 2016, 72 patients who underwent isolated decompression for lumbar spinal stenosis were enrolled prospectively in this single-center study. All of the patients had lateral full-body x-ray scans with the EOS system (EOS Imaging, Paris, France) before surgery and after 12 months of follow-up. Patients were classified into 3 groups according to their preoperative sagittal vertical axis (<50 mm, ≥50 mm, and <100 mm, ≥100 mm). RESULTS: SVA decreased significantly (SVA preoperative: 72.3 ± 43.1; SVA postoperative: 48.3 ± 46.8. P < 0.001). Lumbar lordosis increased significantly from 41.9 ± 13.4 in the preoperative period to 46.5 ± 14.8 at the last follow-up (P < 0.001). In the imbalance groups, the mean postoperative SVA decreased significantly compared with preoperative SVA (P = 0.004). Surgery led to a significant increase in lumbar lordosis in the 3 groups (P < 0.05). Nonetheless, a certain degree of residual imbalance persisted in the major imbalance group. In all of the groups, decompression surgery led to a significant improvement in clinical scores (P < 0.05). CONCLUSIONS: Our study showed an improvement in sagittal balance and lumbar lordosis after decompression surgery without fusion, even in patients with a preoperative SVA >100 mm. However, a certain degree of sagittal imbalance may persist after surgery in patients with major initial imbalance (SVA >100 mm). Nonetheless, after surgery, these patients experienced a clinical benefit comparable with that in the other groups.

Antithrombotic Medication Use and Misuse Among Patients with Intracranial Hemorrhage: A 16-Year, Lebanese, Single-Center Experience.

OBJECTIVE/BACKGROUND: The use of antithrombotic medication (ATM) frequently is reported in patients with intracranial hemorrhage (ICH) and is associated with increased mortality. Unfortunately, ATMs sometimes are prescribed and/or used inappropriately. We sought to determine the rate of ATM misprescription/misuse among patients with ICH in a single-center retrospective study. METHODS: All patients admitted with ATM-related ICH in 1998-2014 were included. Charts were reviewed and demographic, clinical, and radiologic variables were recorded. The type of ATM, dose, and duration of treatment were analyzed critically. The adequacy of ATM prescription/use was assessed in light of the recommendations and guidelines of the American Heart Association, American Stroke Association, and French National Authority for Health, in effect at the time of admission. RESULTS: A total of 106 patients with mean age 68 years were identified. Aspirin (53.8%) was the most commonly used drug, followed by oral anticoagulants (31.1%) and clopidogrel (22.6%). In only 80 patients (75.5%), the use of ATM was in line with contemporary guidelines. In the remaining 26 (24.5%), the use of ATMs was inappropriate, including bad drug combination, wrong dose, poor indication, wrong drug class, and/or incorrect treatment duration. CONCLUSIONS: In this Lebanese cohort of patients with ICH, the 24.5% rate of ATM misprescription and/or misuse is highly alarming and the origin of this problem is likely multifactorial. Immediate measures should be undertaken, and efforts should be focused on regaining tight control of ATM prescription and fulfillment, ensuring good patient education, and offering more vigilant oversight on physician licensure.

Small cell bladder cancer: biology and management.

Small cell bladder cancer (SCBC) is a rare and aggressive form of bladder cancer. It exhibits similar biological behavior to small cell lung carcinoma. Untreated, it is associated with a very poor prognosis. Appropriate oncologic surgery remains the mainstay of treatment of this disease but is not curative alone in the majority of the cases. Adding systemic therapy to the treatment regimen has been shown to improve survival. The most common chemotherapy regimens used in published series include a platinum complex plus etoposide, although doxorubicin-based regimens and standard urothelial cancer regimens also have been associated with response. Despite robust chemotherapy responses, metastatic disease is associated with relapse and a median overall survival of 18 months or less. Better understanding of the molecular alterations driving SCBC may facilitate the development of new therapeutic strategies and improved outcomes.