Primary Spinal Intradural Ewing's Sarcoma: Hemorrhagic Presentation with Acute Neurological Deterioration in Two Pediatric Patients.

Extraosseous primary spinal intradural Ewing's sarcoma (ES) is an unusual entity. Bleed within such tumors with acute neurological worsening is extremely rare, especially in the pediatric age. In this article, we present two children with intradural-extramedullary ES who had sudden decline in their neurological status consequent to an intratumoral hemorrhage. We discuss their clinical course and also briefly review the pertinent literature. Spinal intradural Ewing's sarcomas possibly have a tendency to bleed. A short clinical symptomatology along with an acute neurological deterioration and radiologic evidence of intratumoral hemorrhage in a spinal intradural tumor should raise the suspicion of an ES.

Changing Perception but Unaltered Reality: How Effective Is C1-C2 Fixation for Chiari Malformations without Instability?

OBJECTIVE: The recently described C1-C2 fixation without foramen magnum decompression (FMD) for the management of Chiari malformations (CMs) has sparked a controversy. C1-C2 fixation has been reported to be more effective than traditional FMD. However, the results after such a procedure have not been as well validated. We assessed the efficacy of C1-C2 distraction and fusion without FMD in patients with CM and without demonstrable atlantoaxial instability. METHODS: A total of 40 patients with CM had undergone C1-C2 distraction and fusion without FMD. The preoperative and follow-up clinicoradiological data were prospectively compared using the Klekamp neurologic score, visual analog scale, pBC2 index (ventral brainstem compression), and Vaquero index (syringomyelia). RESULTS: Of the 40 patients, 28 (70%) showed improvement in their neurological score and visual analog scale, 8 remained in same status, 3 showed deterioration, and 1 died during the follow-up period. The clinical improvement did not correlate with the severity of ventral cervicomedullary compression or the presence of osseous deformities (assimilated C1 arch, platybasia and basilar invagination) despite the reduction in the mean pBC2 index (7.9 vs. 5.9). The syringomyelia had decreased in 51.7%, with a reduction in the Vaquero index at follow-up (0.48 vs. 0.38). CONCLUSIONS: The overall results after C1-C2 distraction and fusion for CM without instability were not exceptionally better and appeared to be similar to the outcomes reported with FMD. The neurological improvement seen in some patients had possibly resulted from indirect ventral decompression (due to distraction of C1-C2) rather than the stabilization itself. The presence of bony anomalies such as an assimilated C1 arch, platybasia, basilar invagination, and ventral brainstem compression did not significantly influence the outcomes.

Relying too much on upfront radiosurgery: Indolent course misinterpreted as effectiveness of radiosurgery in a case of skull base chondrosarcoma.

Background: Skull base lesions are still considered surgically challenging and primary gamma knife radio surgery (GKRS) is gaining popularity. However the effectiveness of GKRS may be overrated especially in lesions with indolent course. Case Description: We report a case of chondrosarcoma, mimicking a trigeminal schwannoma treated with upfront radio surgery. Relatively lower dose was administered in view of proximity to the brainstem. The patient was asymptomatic and the size of the lesion remained static for over a decade. This was misinterpreted as effectiveness of GKRS. The lesion grew after a decade necessitating surgery. Conclusion: With popularity of upfront GKRS, suboptimal but maximal safe radiation dose is usually prescribed for lesions close to critical structures like brainstem. In these cases the long indolent natural course of the pathology, as in the case of chondrosarcoma may be misconstrued as success of radiosurgery. An extended follow up beyond this static period is necessary before concluding its effectiveness.

Foramen magnum decompression without bone removal: C1-C2 posterior fixation for Chiari with congenital atlantoaxial dislocation/basilar invagination.

BACKGROUND: Atlantoaxial dislocation (AAD) and basilar invagination (BI) may coexist with Chiari malformations (CM) and a small posterior fossa volume. These are typically treated with craniovertebral junction fusion and foramen magnum decompression (FMD). Here, we evaluated whether C1-C2 posterior reduction and fixation (which possibly opens up the ventral foramen magnum) would effectively treat AAD and CM without additionally performing FMD. METHODS: This is a retrospective analysis of 38 patients with BI, AAD, and CM who underwent C1-C2 posterior reduction and fusion without FMD. Baseline and follow-up clinical, demographic, and radiological data were evaluated. RESULTS: The vast majority of patients (91.9%) improved both clinically and radiographically following C1-C2 fixation alone; none later required direct FMD. Notably, AAD was irreducible in 25 (65.8%) patients. Preoperatively, syringomyelia was present in 28 (73.7%) patients and showed resolution. In 3 (8.1%) patients, resolution of syrinxes did not translate into clinical improvement. Of interest, 5 patients who sustained inadvertent dural lacerations exhibited transient postoperative neurological worsening. CONCLUSIONS: Posterior C1-C2 distraction and fusion alone effectively treated AAD, BI, accompanied by CM. The procedure sufficiently distracted the dens, reversed dural tenting, and restored the posterior fossa volume while relieving ventral brainstem compression making FMD unnecessary. Surgeons should, however, be aware that inadvertent dural lacerations might contribute to unwanted neurological deterioration.

Intracranial Low-Grade Fibromyxoid Sarcoma with Atypical Radiology.

BACKGROUND: Low-grade fibromyxoid sarcoma (LGFMS), an uncommon soft tissue tumor, generally occurs in extremities and is characterized by an aggressive clinical course with frequent recurrences. Primary intracranial occurrence of LGFMS is rare. The few existing descriptions present the clinicopathologic aspects of LGFMS and lack information from a radiologic perspective. In these reports, the lesions appeared solid on imaging. CASE DESCRIPTION: In a 45-year-old woman, a falx-based lesion with solid-cystic radiologic appearance that turned out to be LGFMS on histopathology. The patient's clinical follow-up (2 years) is presented along with a literature review of other primary central nervous system cases. CONCLUSIONS: This report emphasizes the radiologic aspects of LGFMS and describes an atypical solid-cystic appearance of this uncommon central nervous system tumor. This adds another facet to the imaging of LGFMS. Although rare, LGFMS can form a differential diagnosis of dural-based lesions.

Anomalous V3 Segment Aneurysm Associated with Congenital Atlantoaxial Dislocation: Case Report and Discussing the Challenges in Management.

BACKGROUND: An anomalous vertebral artery is not a deterrent for posterior C1-C2 joint manipulation and reduction of atlantoaxial dislocation (AAD). However, presence of an incidental aneurysm in the aberrant segment of artery with concurrent AAD adds to the surgical challenge. CASE DESCRIPTION: A 30-year-old woman presented with neck pain and spastic quadriparesis. Her imaging revealed atlantoaxial dislocation and bony segmentation defects. Three-dimensional computed tomography angiography showed bilateral anomalous vertebral arteries (V3 segment) and an incidental aneurysm on the arterial segment that crossed the right C1-C2 joint posteriorly. Because the artery bearing the aneurysm was nondominant, it was ligated and successful C1-C2 posterior reduction and fusion could be performed. CONCLUSION: The association of an incidental aneurysm with an anomalous vertebral artery in congenital AAD is unusual. The etiology could be an underlying collagen defect or repeated shearing trauma to the vessel wall due to C1-C2 instability. It would be less risky to proceed with endovascular embolization followed by occipitocervical fusion without opening the joints in case the aneurysm is present on the dominant aberrant V3 segment. Ventral decompression can be supplemented for irreducible AAD. On the contrary, if the aneurysm is present on the nondominant aberrant V3 segment, the C1-2 joint can be opened and manipulated following an initial endovascular treatment of the aneurysm. If the circumstances demand, the nondominant artery can be ligated and sacrificed, although there is a small risk of formation of stump aneurysm.

Jejuno-jejunal intussusception: An unusual presentation of malignant melanoma.

Jejuno-jejunal intussusception is a rare mode of metastatic melanoma presentation, with only six cases being reported in the English medical literature to date. We present a case of a 55-year-old female who presented to us with features of obstruction. On exploration, it was discovered that she had jejuno-jejunal intussusception, with enlarged blackened mesenteric lymph nodes. Resection and anastomosis were performed. On further histopathological examination, a lesion was found to be a metastatic malignant melanoma.

Scrotal enterocutaneous fistula: a rare initial presentation of inguinal hernia.

We describe the case of a 70-year-old man with a long history of inguinal hernia who finally presented to the emergency department with a scrotal enterocutaneous fistula. This is a rare complication of an incarcerated/obstructed direct inguinal hernia. We performed exploratory laparotomy with resection of the perforated small bowel and end-to-end anastamosis of the ileal segment using the purse-string suture at the deep inguinal ring.

Splenic artery pseudoaneurysm presenting as massive hematemesis: a diagnostic dilemma.

Introduction. Splenic artery Pseudoaneurysm, a complication of chronic pancreatitis, presenting as massive hematemesis is a rare presentation. Case Report. We present a case of 38-year-old male admitted with chief complaints of pain in the upper abdomen and massive hematemesis for the last 15 days. On examination there was severe pallor. On investigating the patient, Hb was 4.0 gm/dL, upper GI endoscopy revealed a leiomyoma in fundus of stomach, and EUS Doppler also supported the UGI findings. On further investigation of the patient, CECT of the abdomen revealed a possibility of distal pancreatic carcinoma encasing splenic vessels and infiltrating the adjacent structure. FNA taken at the time of EUS was consistent with inflammatory pathology. Triple phase CT of the abdomen revealed a splenic artery pseudoaneurysm with multiple splenic infarcts. After resuscitation we planned an emergency laparotomy; splenic artery pseudoaneurysm densely adherent to adjacent structures and associated with distal pancreatic necrosis was found. We performed splenectomy with repair of the defect in the stomach wall and necrosectomy. Postoperative course was uneventful and patient was discharged on day 8. Conclusion. Pseudoaneurysm can be at times a very difficult situation to manage; options available are either catheter embolisation if patient is vitally stable, or otherwise, exploration.