Primary Amelanotic Malignant Melanoma of the Tongue.

Background: Primary malignant melanoma rarely occurs in the oral cavity. The tongue is a particularly unusual primary site; lesions may be pigmented or amelanotic. Primary malignant melanoma is frequently mistaken for squamous cell carcinoma. Case Report: A 27-year-old male presented with a large, painless, ulceroproliferative mass on the dorsal surface of the tongue for 6 months. Squamous cell carcinoma was suspected, and the lesion was biopsied. Histopathology was compatible with primary amelanotic malignant melanoma. The patient had no cutaneous lesions consistent with malignant melanoma, and no definitive metastatic lesions were found. Ultrasound and computed tomography did not reveal any evidence of regional draining lymph node metastasis or suspicious lesions anywhere else in the body. The patient underwent composite resection of the tongue tumor and bilateral neck lymph node dissection, had an uneventful postoperative recovery, but was lost to follow-up. Conclusion: Primary oral amelanotic malignant melanoma is a highly aggressive, potentially fatal tumor and because of its rarity, presents a diagnostic challenge. The ideal treatment modality for primary malignant melanoma of the tongue is poorly defined, but surgery is regarded as the most effective course of therapy.

Anatomical correlates for the newly discovered meningeal layer in the existing literature: A systematic review.

The existence of a previously unrecognized subarachnoid lymphatic-like membrane (SLYM) was reported in a recent study. SLYM is described as an intermediate leptomeningeal layer between the arachnoid and pia mater in mouse and human brains, which divides the subarachnoid space (SAS) into two functional compartments. Being a macroscopic structure, having missed detection in previous studies is surprising. We systematically reviewed the published reports in animals and humans to explore whether prior descriptions of this meningeal layer were reported in some way. A comprehensive search was conducted in PubMed/Medline, EMBASE, Google Scholar, Science Direct, and Web of Science databases using combinations of MeSH terms and keywords with Boolean operators from inception until 31 December 2023. We found at least eight studies that provided structural evidence of an intermediate leptomeningeal layer in the brain or spinal cord. However, unequivocal descriptions for this layer all along the central nervous system were scarce. Obscure names like the epipial, intermediate meningeal, outer pial layers, or intermediate lamella were used to describe it. Its microscopic/ultrastructural details closely resemble the recently reported SLYM. We further examined the counterarguments in current literature that are skeptical of the existence of this layer. The potential physiological and clinical implications of this new meningeal layer are significant, underscoring the urgent need for further exploration of its structural and functional details.

Endovascular embolisation of posterior condylar canal dural arteriovenous fistula.

We describe a rare case of dural arteriovenous fistula (dAVF) of the posterior condylar canal in a man in his 30s who presented with recent onset headache and neck pain and subsequently acute intracranial haemorrhage. Radiological workup showed a medulla bridging vein draining dAVF of the right posterior condylar canal supplied by a meningeal branch of the right occipital artery. A dilated venous sac was seen compressing over cerebellar tonsil on the right side. There was acute haemorrhage in the posterior fossa and fourth ventricle. He was successfully managed with transarterial endovascular embolisation via a supercompliant balloon microcatheter without any complication. The balloon microcatheter effectively prevented reflux of the liquid embolic agent into the parent artery and vasa nervosa of lower cranial nerves.

Contribution of Deep Cerebral Venous Anomaly to the Emergence of Nonaneurysmal Subarachnoid Hemorrhage as Opposed to Aneurysmal Subarachnoid Hemorrhage.

BACKGROUND: The exact reason of nonaneurysmal subarachnoid hemorrhage (SAH) is an enigma. The aim of this study is to identify if type III deep cerebral venous drainage is exclusively prevalent in patients with nonaneurysmal SAH and to enumerate the predictors of poorer outcome in these patients. METHODS: All patients of age >18 years, presented at our centre with spontaneous SAH on noncontrast computed tomography head and were divided into 2 groups, aneurysmal and nonaneurysmal SAH after 4-vessel DSA. Based on the deep venous drainage pattern on both sides, basal venous drainage was found and classified into 3 types: type I, type II, and type III. The 3 groups were pitted against one another. Regression analysis were performed to predict the occurrence of nonaneurysmal-SAH with different types of basal vein. RESULTS: There were 100 nonaneurysmal SAH cases and 103 aneurysmal SAH cases. The mean age of presentation was 47.8 ± 13.55 years with slight male predominance (52%). The patients with type III venous drainage have 2 times more risk of developing nonaneurysmal SAH (95% confidence interval = 1.21-4.31) as compared to those with aneurysmal SAH. On multivariate analysis, type III basal venous drainage, worse Hunt and Hess grade at presentation, extensive bleeding were predictors of an adverse outcome. CONCLUSIONS: The presence of type III venous distribution is associated with a 2-fold increase in the probability of having nonaneurysmal SAH, as well as a 3-fold increase in the risk of developing poorer neurological sequelae.

Management of scalp arteriovenous malformations: a rising trend towards percutaneous direct puncture embolization technique-our experience.

BACKGROUND: Scalp arteriovenous malformations (AVMs), or cirsoid aneurysms of the scalp, usually present with troublesome symptoms and cosmetic disfigurement. Endovascular/percutaneous embolization has evolved as a sole treatment method or adjunct to surgical excision in the management of scalp AVMs with an excellent outcome. PURPOSE: To discuss minimally invasive techniques for treating scalp AVMs as well as to highlight the role of embolization before surgery. MATERIAL AND METHODS: This is a retrospective study of 50 patients with scalp AVM who underwent embolization (percutaneous/endovascular) during 2010-2019 at a tertiary care center. n-butyl cyanoacrylate (n-BCA) was used as an embolizing agent in all the cases and the patients were followed up at three- and six-month intervals with Doppler evaluation. RESULTS: A total of 50 patients were included in the study. The occipital region was the most common location; 82% were Schobinger class II lesions and 18% were class III lesions. Thirteen patients had small-sized AVMs and 37 patients had large-sized AVMs. Post-embolization surgery was performed in 36 patients. Of the patients, 28 underwent percutaneous embolization, 20 underwent endovascular embolization, and two underwent both to achieve complete embolization of the lesion. The number of percutaneous procedures increased in the latter half of the study period as the safety and efficacy of the technique were established. No major complications were seen in this study. CONCLUSION: Embolization of scalp AVMs is a safe and effective technique and can be used in isolation for small lesions and as an adjunct procedure to surgery for large-sized lesions.

Endovascular management of pulmonary arteriovenous malformations presenting as multiple brain abscesses.

Pulmonary arteriovenous malformations (PAVMs) are rare vascular lesions characterised by abnormal connections between the pulmonary artery and vein bypassing the pulmonary capillary bed and causing right-to-left shunt. Paradoxical embolism is known to occur in these cases, leading to inoculation of septic focus in the systemic circulation. We report a case of multiple PAVMs who presented clinically with seizures and altered sensorium. On radiological work-up, multiple brain abscesses and large PAVMs were evident. The patient was successfully treated by endovascular embolisation of the PAVMs using a vascular plug and multiple coils. The patient showed complete clinical recovery and resolution of brain abscesses on follow-up.

Non-aneurysmal subarachnoid hemorrhage: Is the deep venous system the hidden culprit?

AIM: The exact cause of bleeding in non-aneurysmal sub-arachnoid hemorrhage (SAH) is yet to be established. The present study intends to evaluate the morphological variants of deep cerebral venous drainage, especially basal veins of Rosenthal (BVR), and to correlate if such a venous anomaly is associated with increased incidence of non-aneurysmal SAH. METHODS: A prospective analysis of all the patients of age more than 12 years with spontaneous non-aneurysmal SAH and undergone 4-vessel DSA for the diagnosis of the source of bleeding was included in the study (n = 59). The anatomy of the basal venous distribution was evaluated and was divided into 3 different types, namely normal (Type A), normal variant (Type B), and primitive (Type C), based on DSA findings. The follow-up of these cases was noted. The three groups were compared with one another. RESULTS: The median age of presentation was 51 years with slight male predominance (52%). Primitive venous drainage was associated with a poorer grade at presentation (p = 0.002), more severe bleed (p = 0.001), vasospasm (p = 0.045), and a poorer outcome at 6 months (p = 0.019). Hydrocephalous and vasospasm were seen in patients with primitive venous drainage. On multivariate regression analysis for poorer outcome, it was observed that a worse grade at presentation, extensive bleed, primitive venous drainage are independent predictors of an adverse outcome. CONCLUSION: The presence of primitive venous drainage has a linear relationship with the development of non-aneurysmal SAH with multi-cisternal hemorrhage, worse grade at presentation, and unfavorable outcome.

Combined endovascular and surgical management of a case of Cobb syndrome.

Cobb syndrome is a rare neurocutaneous disorder characterised by spinal and cutaneous vascular malformations in a metameric distribution. Managing such cases is a challenge for the interventionists and neurosurgeons as the results are often suboptimal. We describe a case of Cobb syndrome in a young male child who presented clinically with acute paraparesis and lower backache. On radiological workup, an intradural perimedullary complex spinal arteriovenous fistula was seen with presence of subarachnoid haemorrhage in the spinal canal and compressive myelopathy. There was a haemangioma in paraspinal muscles and a maculopapular cutaneous lesion in the lower back. He was treated with combined endovascular embolisation and surgical intervention and showed significant clinical improvement on follow-up.

Repeated aspiration and sclerotherapy to manage recurrent spinal epidermoid cyst.

Spinal epidermoid cysts are rare lesions and epidermoid cyst in intramedullary location is even rarer. Surgical excision is the mainstay of treatment; however, in cases of recurrence, repeat surgery becomes quite difficult. Treatment of recurrent intramedullary epidermoid cyst by surgery alone is a challenge. We managed one such rare case with repeated aspiration and sclerotherapy. Here, we have highlighted hypertonic saline sclerotherapy as a promising tool to treat recurrent spinal epidermoid cysts.

Traumatic dissecting pathology of posterior cerebral artery: a report of two cases-aneurysm and pial arteriovenous fistula.

We present two cases of 17-year-old man and 10-year-old boy presenting with subarachnoid haemorrhage and a history of road traffic accident. One patient had dissecting aneurysm of the posterior cerebral artery (PCA), and the other patient had partially thrombosed aneurysm on CT angiography. On digital subtraction angiography of the second patient, there was formation of PCA pontomesencephalic vein pial arteriovenous fistula (PAVF). Both the patients underwent endovascular treatment: stent-assisted coiling for aneurysm and coiling with parent vessel occlusion for PAVF. There were no procedural complications. Follow-up angiography showed no residual aneurysm or fistula. Trauma is one of the recognised causes of dissection, and intracranial dissections can present as stenotic lesions, aneurysms or fistulas, depending on the pathology. Traumatic dissecting PCA aneurysm has been reported in only two case reports previously, and post-traumatic PAVF in PCA has not been reported.

Complete resolution of long pedunculated thrombus of the proximal ascending aorta following conservative management: an interesting case.

We report a case of a 60-year-old woman who presented clinically with symptoms of acute embolic stroke. On workup with MRI, carotid Doppler and subsequent CT angiography, a long pedunculated mobile thrombus was seen with the base of the thrombus attached to the ascending aorta and the tip protruding into the left common carotid artery. She was advised urgent cardiovascular surgery consultation; however, she preferred medical management over surgery. She was put on dual antiplatelet therapy. On follow-up after 6 months, there was complete resolution of the thrombus.

Spinal Epidural Fistulas-A Separate Entity to Dural Fistulas with Different Angioarchitecture and Treatment Approach.

OBJECTIVE: Spinal epidural arteriovenous fistulas (SEAVFs) are the rarest variety of spinal vascular malformation and are often misdiagnosed as type 1 spinal dural fistula. This retrospective study highlights the salient anatomic differentiating points of these entities and also highlights the importance of a planned endovascular treatment approach using different routes of access. Efficacy of the endovascular treatment at 3 months follow-up was also studied. METHODS: We retrospectively reviewed 11 treated patients with SEAVF. Existence of epidural arteriovenous fistula in all these patients was confirmed by spinal angiography. The Aminoff-Logue Scale score was assigned both before and after the procedure. The statistical results were expressed as percentages, and the preprocedure scale was compared with the postprocedure scale at 3 months by using a nonparametric Wilcoxon signed-rank test. RESULTS: The patients ranged in age from 7 to 53 years, with male predominance. Paraparesis was the commonest symptom, and 1 patient had congestive cardiac failure caused by a large fistula. Location was mostly dorsolumbar with intradural venous reflux into the perimedullary venous system (Castilla type A) noted in 3/11(27%) patients, and the remaining 73% patients had Castilla type B1 with an enlarged epidural venous sac. Therapeutic embolization was performed from arterial, venous, or combined routes. The 3-month postprocedure clinical assessment showed statistically significant (P < 0.004) improvement in the Aminoff-Logue Scale score. CONCLUSIONS: The differentiating points between SEAVF compared with type 1 spinal dural fistula are emphasized. The study also highlights the importance of a good angiographic assessment to best access the fistula by arterial, venous, percutaneous, or combined routes. Endovascular treatment resulted in statistically significant clinical improvement at 3 months follow-up.

Getting More Out of Follow-up Three-Dimensional Time-of-Flight Magnetic Resonance Angiography in Endovascularly Treated Intracranial Aneurysms.

BACKGROUND: We retrospectively re-evaluated follow-up three-dimensional (3D) time-of-flight (TOF) magnetic resonance angiography (MRA) in patients with aneurysms treated with coiling at our Institute. AIMS: To document the type and frequency of postcoiling residue patterns as seen on follow-up MRA and to document their evolution with time where a further follow-up MRA was available. To assess the implications of the location of the aneurysm on residue and recurrence. SUBJECTS AND METHODS: 3D TOF MRA for 104 aneurysms were evaluated for residue size and residue pattern. Mainly, three residue patterns were identified. The aneurysms were allocated to different groups depending on the location. Multiple MRA studies were available in subgroup 1* and subgroup 2* where the residue growth or reduction and pattern change was noted and residue growth rates were calculated. RESULTS: Collectively 54 (51.92%) aneurysms showed occlusion (pattern 1 and 1A), 31 (29.81%) showed neck residue (pattern 2A, 2B and 2C) and 19 (18.27%) showed recurrence (pattern 3A, 3B and 3C, residue size >3 mm) at the last follow-up MRA. Type 2A/3A patterns were more common. In terms of residue and recurrence, the distally located aneurysms (Group 3) appeared to do well. For those showing growing residue/recurrence, the average growth rate was calculated at 0.094 mm/month and 0.15 mm/month, respectively, for subgroup 1* and subgroup 2*, although the difference was not statistically significant. With longer follow-up the persisting and growing residues from both the subgroups, not warranting early re-treatment, showed a low growth rate at approximately 0.05 mm/month. CONCLUSIONS: TOF MRA helps in identifying different residue patterns in coiled aneurysms. Serial follow-up MRA appears useful in showing the pattern and size changes in the residual aneurysm. Although more work is required in this regard, calculation of aneurysm/residue growth rate may be useful in prognostication and in scheduling further follow-up or retreatment. The risk factor related to the location of the aneurysm warrants further study.

Mucinous adenocarcinoma arising from chronic perianal fistula mimicking horseshoe abscess.

Perianal fistulae are commonly seen clinical entity. Development of malignancy within a perianal fistula is rare. Even rarer is the development of mucinous adenocarcinoma in a chronic fistula-in-ano. Only a handful of such cases have been reported in the past. A case of mucinous adenocarcinoma arising in chronic perianal fistula in a 34-year-old woman is being described. She presented with complaints of perineal fullness, pain and recurrent pus discharge from perianal fistula for 4 years. On radiological workup, a large solid-cystic pelvic mass was seen in relation to the fistula. On MRI, the lesion was mimicking a large horseshoe abscess. Transrectal ultrasound-guided biopsy and subsequent histopathological examination confirmed the presence of mucinous adenocarcinoma with tumour cells immunopositive for CK7 and CK20.

Abscess inside craniopharyngioma: diagnostic and management implications.

Abscess inside the pituitary fossa is very rare. Such abscess can be primary, occurring in an otherwise healthy pituitary gland, or secondary, developing inside a diseased gland (ie, harbouring craniopharyngioma, Rathke's cleft cyst, etc). Secondary pituitary abscess inside a craniopharyngioma remains an extremely rare occurrence. Our literature search revealed only six such cases reported so far. In this report, we present the seventh case of craniopharyngioma with abscess in a 38-year-old woman. We describe the uniqueness of the clinical presentation of our case, the radiological pointers to the possible diagnosis and the management issues in our patient. A review of literature is also included to provide a comprehensive picture of this rare condition to the readers.