Idiopathic unilateral complete oculomotor nerve palsy: a case report of diagnostic quandary.

Introduction and importance: When compared to other cranial nerve palsies idiopathic unilateral oculomotor nerve palsy with pupillary sparing is one of the least noted neurological conditions. Moreover, there lies a series of diagnostic dilemmas to come into a final diagnosis resulting in several array of clinical investigations. Hence, there is a delay in prompt management. Case summary: An elderly female without any known comorbidities presented with the complaint of headache, dizziness and dropping of left eyelid. Several arrays of diagnostic workups was done to come to a diagnosis, but even with rigorous laboratory investigations and radiological examinations, a common working diagnosis could not be made. Hence with a diagnosis of exclusion after proper neurological and neuro-ophthalmological examination, idiopathic unilateral common oculomotor nerve palsy was identified for which improvement with steroids was noted in the patient. Discussion: Idiopathic unilateral complete oculomotor nerve palsy is considered as a diagnosis of exclusion when all the diagnostic parameters fail to signify and positive results. The vague symptomatic presentation of the disease condition further compels the treating physician to carry out several panels of laboratory to radiological investigations. But if identified in time the treatment modality is straightforward. Conclusion: The diagnostic quandary in timely identification of such disease conditions needs a pertinent diagnostic guideline so as to avoid the unwanted panel of investigations.

Takayasu arteritis and interstitial lung disease: a case report and literature review.

Introduction and importance: Although pulmonary artery involvement is well recognized, the incidence of interstitial lung disease (ILD) with Takayasu arteritis is very rare. The pathophysiology of ILD in Takayasu is still incompletely understood, in contrast to several studies establishing the relationship between ANCA-associated vasculitis and ILD. The management of this patient involved a multidisciplinary approach with long-term follow-up. Case presentation: The authors present a case of HRCT-proven interstitial lung disease in a patient with Takayasu arteritis and heart failure. The patient was on long-term corticosteroids on and off for several years and recently developed progressive dyspnea with a dry cough. After reviewing her history and physical examination, pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) were performed, and interstitial lung disease was diagnosed. The patient was managed by a team of pulmonologists, rheumatologists, and cardiologists and gradually improved after adjustment of medications, including corticosteroids and mycofenolate, and via long-term oxygen therapy. Clinical discussion: Takayasu arteritis is a rare form of systemic vasculitis that can involve the pulmonary vasculature, such vasculitis with associated parenchymal involvement is rare. ILDs have been demonstrated with ANCA-associated vasculitis; however, whether the pathophysiology applies to Takayasu is unknown. Since Takayasu can be debilitating to the patient, the association of ILDs can have further prognostic implications. Given that no established guidelines exist to address this association, management is based on clinical expertise. Conclusion: The authors report a case of Takayasu arteritis and associated ILD and its pharmacological management. Takayasu arteritis is a very uncommon type of vasculitis, and pulmonary parenchymal involvement further contributes to this case's rarity. As the management of Takayasu arteritis alone is cumbersome, the addition of another significant comorbidity, such as ILD, can pose several threats to the patient. Given the rarity of this association, no established guidelines exist, making clinical expertise crucial for managing such patients. Further research is needed to explore the underlying mechanisms and develop evidence-based treatment strategies for this rare combination.

Neurotoxicity and acute renal injury secondary to Russell's viper bite in an individual: a case report from Nepal.

Introduction and importance: Owing to the high number of envenomation and fatalities, the Russell's viper holds greater medicinal significance than any other Asian serpent. South East Asia is one of the most snakebite-prone regions in the world. Dense population, extensive agricultural practices, the abundance of venomous snake species, and an overall lack of knowledge about primary treatment (first aid) are the major culprits associated with snake bite-related morbidity and mortality. The venom of vipers is known to produce vasculotoxicity and contains hemotoxins. Case presentation: The authors describe a patient who was bitten by a viperine snake and showed signs of both neurotoxicity and acute kidney injury (AKI). The 20 years male was treated in a tertiary care centre in Nepal. The patient developed respiratory failure and needed ventilator support. Further, more haemodialysis was also done to manage AKI. Later, the patient was discharged after a smooth recovery. Discussion: Numerous clinical manifestations, such as neurotoxicity and vasculotoxicity, can result from a viperine bite. The majority of viperine snakebites are hemotoxic. Dual neurotoxic symptoms are possible after a viperine bite despite their rarity. The prevention of respiratory failure depends critically on the early detection of neurotoxicity. Conclusion: Unusual neuromuscular paralysis is caused by Russell's vipers (Daboia russelii) in South East Asia. Physicians should know the exceptional presentations of snakebites to diagnose and treat patients.

Intractable seizures after cranioplasty, a dreadful post-operative complication managed efficiently from Nepal: A case series.

INTRODUCTION AND IMPORTANCE: Cranioplasty is an elective neurosurgical procedure following decompressive craniectomy, often associated with post-operative complications such as hemorrhage, seizures, infection, hydrocephalus, and bone resorption. While seizures post-cranioplasty is not uncommon, Intractable seizures are rare but a dreadful complication following cranioplasty. CASE SUMMARY: A 23 and 17-year-old male underwent decompression craniectomy for traumatic brain injury and subsequently underwent titanium mesh and acrylic cranioplasty respectively, During the post-operative period both patients developed intractable seizures. Initially seizures were refractory to multiple anti-epileptic drugs and benzodiazepine induced coma. Intractable seizures were controlled gradually with few anti-epileptic drugs after the removal of cranioplasty. During 2 years' follow-up, there was no new episode of seizures complained by patients. DISCUSSION: Elective cranioplasty following decompressive craniectomy after traumatic head injury is one of the commonest surgical modality carried out globally as a life saving measure. But post-operative complications following cranioplasty such as wound infection, implant displacement, bone resorption, hydrocephalus, epidural hematoma are the commonest observed complications whereas post-operative seizure is the most dreadful complication needing immediate intervention. Along with it intractable seizure is rarer during post- operative period. CONCLUSION: Intractable seizures post-cranioplasty, though rare, are serious and often linked to cerebral edema from negative pressure drainage and dysregulation of cerebral blood flow. Removing the cranioplasty can restore blood flow regulation, stabilize brain tissue, and resolve seizures. Care providers should be aware of this rare complication and to be counselled the patient and the family before the surgery.

Ventriculo peritoneal shunted patient undergoing laparoscopic procedure - A case report and review of the literature.

INTRODUCTION AND IMPORTANCE: It has been more than century since the establishment of relationship between the intracranial pressure (ICP) and intraabdominal pressure (IAP) was suspected and confirmed backed up by several studies. The studies clearly demonstrate a link and the positive correlation between the two. CASE SUMMARY: 18 years old lady with previous history of ventricular peritoneal shunt during 6 months of age was planned for laparoscopic cholecystectomy. Intraoperatively free flow of CSF was observed without any intraoperative and post-operative complications. DISCUSSION: Pneumoperitoneum created during laparoscopic procedures may lead to the rise in ICP and thus, lead to serious consequences. This has been of a growing concern in a patient with Ventriculo Peritoneal Shunt (VP Shunt) in situ. The uncertainty regarding the safety of laparoscopic procedures in VP shunted patients thus, has been a subject of debate over the years. CONCLUSION: Our aim therefore is to depict similar case we encountered in our department and based on it, we reviewed the literature in the quest of determining the safety of Laparoscopic procedure in patients with VP shunt.

Cerebral venous sinus thrombosis in patient of immune thrombocytopenic purpura managed with mechanical thrombectomy: An anecdotal endovascular experience from lower middle income country.

Cerebral venous sinus thrombosis in itself is rarely encountered clinical entity and its association with immune thrombocytopenic purpura (ITP) makes it more unusual presentation. No any as such standard guidelines exist that guides the prompt evidence based management in such concurrent cases but neuroendovascular modality can play a pivotal role.

Outcomes of stereotactic thalamotomy in patients of essential tremor: A systematic review.

BACKGROUND: Essential tremor is a neurological condition associated with movement disorder with more prevalence among adult group of population. The burden of essential tremor is peaking globally but with the advancement in the area of functional neurosurgery such as stereotactic thalamotomy, the quality of life of such patients can be improved drastically. METHODS: This systemic review was conducted in accordance to the guidance of preferred Reporting items for Systematic Review and Meta-Analysis(PRISMA). Databases of "PubMed", "Embase", "Web of Science", "Cinhal Plus", and "Scopus" from inception till 2023 was undertaken. A combination of keywords, Medical Subject Headings (MeSH), and search terms such as Search strategy for PubMed search was as follows: "stereotactic thalamotomy" AND "essential tremor". RESULTS: This systematic review analyzed 9 studies with a total of 274 patients of essential tremor patients. Unilateral thalamotomy was carried out among 268 patients and bilateral thalamotomy in rest of the patients. Vim and Vom nucleus were the site of thalamotmy with ventral intermedius nucleus being the major one. Ten different types of clinical tremor rating scales were used to assess pre operative and post operative improvement in the tremor scales of the individual patients. Dysarthria and limb weakness was noted post operative complication in majority of the cases. CONCLUSION: Our study revealed that stereotactic thalamotomy provided good functional outcome in patients of essential tremor who underwent unilateral thalamotomy compared to bilateral thalamotomy. The positive outcome outweighs the complications in such functional surgery.

Anti-coagulants-induced intracranial hemorrhage managed with decompressive craniectomy: a case report of lesson learned.

Introduction and importance: Intracerebral haemorrhage (ICH) secondary to use of anti-coagulants is one of the fearsome complications. Haemorrhage within the intracranial space due to such anti-coagulants results in expansion of the intracranial bleeding despite the reversal of anti-coagulants. Hence, timely surgical intervention can be lifesaving. Case summary: An elderly female who was undergoing management for her deranged coagulation parameters was found to be in a state of features suggestive of stroke. The patient was on regular anti-coagulants medication for her cardiology issues. Computed tomography (CT) scan showed intracranial haemorrhage, which underwent expansion on same day; hence decompressive craniectomy was done. During further stay in the ICU patient's Glasgow coma scale fluctuated but symptomatic improvement was noted. Anti-coagulants adjustment was made by a cardiologist and further, there was no expansion of intracranial bleeding within normal coagulation parameters. Discussion: Anti-coagulants are rampantly used in several cases. Despite the several complications, there is a desperate need for such medications for the betterment of the patient's condition. Pharmacological management is a major modality in the reversal of oral anti-coagulants (OAC)-induced ICH, but in rare cases in the background of OACs-induced ICH, there occurs expansion of haemorrhage. Hence there is a need for neurosurgical intervention, whether it be minimally invasive surgery or decompressive craniectomy. Conclusion: In the background of the low prevalence of OACs-induced ICH, there is an absence of a robust guiding treatment protocol. Furthermore, there exist minimal reported cases which underwent surgical intervention and resulted in a good prognosis.

Contiguous diastematomyelia with tethered cord, intradural extramedullary dermoid tumor, and lipomyelomeningocele: A unique case of spinal dysraphism.

Diastematomyelia, tethered cord, intradural extramedullary dermoid tumor and lipomyelomeningocele such disease entities themselves are rare in their own form and concurrent presentation of all those pathological states in a single individual can be considered one of the rarest forms of spinal dysraphism globally. Moreover for prompt management with optimal prognosis needs refined neurosurgical intervention guided by intraoperative neuromonitoring so as to bring about the best quality of life in the patient.

Alstrom syndrome with classical findings: a rare case report of monogenic ciliopathy co-occurrence in twins.

Introduction and importance: Alstrom syndrome is one of the rarest monogenic ciliopathy belonging to autosomal recessive disorder. The pathophysiology of Alstrom syndrome is not well understood but based upon the available medical literature its mechanism can be linked with recessive mutation in Alstrom syndrome 1(ALSM1) gene resulting in various multiple organ involvement and poor prognosis. Moreover the co-occurrence of such syndrome simultaneously in twins in same period of time is considered rare. Case presentation: Monochorionic diamniotic twins male born to healthy parents with significant antenatal and natal history along with decreased vision in both eyes in both twins since neonatal period. Throughout the childhood the disease progressed without any confirmatory diagnosis during which the twins underwent simultaneous multiple systemic involvement such as legal blindness in both twins at the age of 11 years, insulin resistance and features of diabetes mellitus, sensorineural hearing loss, subclinical hypothyroidism and various deranged metabolic panels. Certain diagnosis of Alstrom syndrome was made at the age of 16 years in both twins after whole-exome sequencing. Clinical discussion: Based on genetic profile alstrom syndrome is a unique diagnosis. Along with its multi-organ involvement features, its progression and prognosis should also be looked upon while diagnosis and management in such syndromic patients. The diagnostic delay in such cases is also a matter of concern which can result in further delay in halting adverse effects of the disease itself. The multidisciplinary approach with involvement of endocrionologist, ophthalmologist and audiologist can bring upon improvement in quality of life of the patients. Conclusion: With the prevalence of 1 in million cases Alstrom Hallgren syndrome is one of the rare genetic disorder with poor prognosis. In our case we present classical findings in twins who were diagnosed as Alstrom syndrome concurrently and further diseases progressed simultaneously.

A rare case report of low-grade endometrial sarcoma: A surgical tale from Himalayas.

INTRODUCTION: In the context of female genital tract malignancy, uterine sarcoma is considered the rarest form of the disease. Despite the inert nature of low-grade endometrial sarcoma, they must be meticulously diagnosed on time, with an exact grading of the severity and staging of the disease, which further guides the treatment modality and prognosis. CASE SUMMARY: A married Asian female without any significant past medical and surgical history complained of abdominal distension and discomfort, which was progressive in nature, for which a radiological assessment was made that showed features suggestive of endometrial sarcoma. Total abdominal hysterectomy with sapingoopherectomy was done without any perioperative complications. Histology further confirmed the diagnosis. Post-operatively, the patient had an unremarkable hospital stay and was discharged home. DISCUSSION: Endometrial stromal sarcoma is one of the rare malignant entities presenting usually in late adult females, but sometimes it can present at an earlier age as well. Abdominal masses in females, although usually overlooked as benign, can sometimes be associated with a malignant picture. Low-grade endometrial sarcomas have been seen to masquerade other minor benign cases, such as leiomyoma. Despite the rarity of such malignant conditions, diagnosis and management are rather straightforward, and post-operative patient prognosis has been found to be rewarding. CONCLUSION: Among the uterine sarcoma cases, endometrial sarcoma comes under the malignant disease of the least occurrence. Compared to other malignant conditions, these patients present with minor symptoms like discomfort, which may go unchecked. The major factor that should be noted is the on-time diagnosis and appropriate choice of treatment modality. Overall, despite a minute prevalence and difficult diagnosis, the prognosis of the patient is rather good.

A rare case of morbidly adherent placenta in a young Primigravida with RH negative pregnancy managed with peripartum subtotal hysterectomy.

INTRODUCTION AND IMPORTANCE: Postpartum hemorrhage (PPH) can be defined as excessive bleeding (>500 ml) from the genital tract after the delivery of baby upto 6 weeks. PPH accounts for major cause of maternal mortality rate. Prevention and early intervention can prevent this complication of delivery. However condition like placenta accreta leads to retention of placenta and makes PPH inevitable. CASE SUMMARY: We present the case of massive postpartum hemorrhage secondary to Placenta accreta in young primigravida with RH negative pregnancy. Clinical findings and investigations were not significant during her admission. She delivered the baby via vaginal route but placenta was not expelled till 30 min. Due to failed manual removal of placenta patient was shifted to OT.Manual vacuum aspiration was done in OT setting and chunks of placenta along with blood clots were obtained.Uterine balloon tamponade was inserted. Due to persistent PV bleeding subtotal hysterectomy was carried out in line for placenta accreta. DISCUSSION: Placenta accreta being one of the life threatening obstetric condition, it should be diagnosed as early as possible and need prompt management so as to prevent maternal mortality. Due to increasing number of cesarean delivery the cases of placenta accreta has been rising but rarely in some cases can it present in young primigravida with Rh negative pregnancy. CONCLUSION: In the cases of morbidly adherent placenta it is necessary for obstetrician to early identify such conditions and timely intervene to save the mother's life. Moreover Rh negative could be a hidden risk factor.

Pituitary macroadenoma with panhypopituitarism masquerading as schizophrenia: a diagnostic dilemma managed conservatively.

Introduction and importance: Secretory pituitary macroadenoma also known as prolactinoma are benign neoplasm comprising very minimal cases of intracranial masses. Among the various presentation suggestive of panhypopituitarism, psychosis, and features of schizophrenia is very rarely seen. In the majority of cases, neurosurgical intervention for the excision of tumor is considered a standard treatment modality but conservative management with dopamine agonist and steroids have also been shown to provide an optimal level of care also improving the quality of level of patient. Case presentation: A 42-year-old Asian male presented with a history of talking to self, delusion of persecution, over talkativeness, hallucination, increased suspiciousness, and history of lost and found in the streets where he was working as a migrant worker. The patient was initially managed in line of schizophrenia with the antipsychotics drug of choice. On further assessment there was no improvement of psychiatric symptoms but they further deteriorated with additional neuropsychiatric symptoms; hence, MRI brain was carried out. Following which, the diagnosis of pituitary macroadenoma was confirmed and further more hormonal analysis was done, which showed findings suggestive of panhypopituitarism. The patient was then managed conservatively with dopamine agonist and steroids, which showed rapid improvement of psychiatric symptoms with a massive reduction in the size of the pituitary macroadenoma. Clinical discussion: With the incidence of 100 per million cases pituitary adenomas are considered locally invading with the characteristic compression of the surrounding structure, presenting as visual hallucinations, olfactory hallucinations, episodes of losing time, apathy, and features suggestive of adrenal insufficiency, hypogonadotrophic hypogonadism, and symptoms secondary to hormonal imbalance such as hypothyroidism. Psychiatric symptomatic presentations are considered a very rare presentation in cases of pituitary macroadenoma. Also, psychiatric features and symptoms of psychosis are associated with prolcatinomas through idiopathic mechanism and the basic casualty has not been established. Surgical intervention such as trans-sphenoidal resection of the mass can be undertaken in case where mass effects is present but long-term remission and prognosis is found not to be fruitful. Conservative treatment with dopamine agonist such as cabergoline and steroids also plays a meaningful role in abrupt management in such cases. Conclusion: Pituitary macroadenoma presenting as a patient of schizophrenia is noted very rarely in medical literature; hence, investigations in view of neurosurgical diagnosis in cases presenting as psychosis should be considered for ideal holistic management. Conservative management can also be a breakthrough treatment modality in complete recovery of pituitary macroadenoma.

Endoscopic evacuation of chronic subdural hematoma with rigid and flexible endoscope: case report.

Introduction: Subdural haematoma (SDH) is a common neurosurgical condition after head trauma requiring evacuation to prevent secondary brain injury. The first choice of management in these patients is a large craniotomy or burr-hole evacuation. However, sometimes due to lack vision during drain tube insertion or irrigation the authors might land up in a complication like cortical bridging vein rupture, haemorrhage etc. Also, the management of septate chronic SDH (CSDH) with multiple neo-membranes does not have a well-defined surgical approach. Recently, endoscopic evacuation has been reported to a be a feasible method for evacuation in acute, subacute and chronic SDH patients. Presentation of case: A 65-years-old male patient presented with a history of recent head injury and symptoms of headache and urinary incontinence of 7 days (Glasgow Coma Scale Score 15/15). Computed tomography scan revealed CSDH at both fronto-parietal convexity more on right side. Discussion: The authors reported our initial experience on a typical case of an older patient with chronic subdural haematoma and its evacuation with the assistance of both rigid and flexible endoscope. The authors could visualize cortical bridging veins and neo-membranes intraoperatively and guided our drainage tube accordingly to avoid inadvertent haemorrhage. There was no recurrence of symptoms postoperatively. Thus we achieved apparent successful evacuation of the CSDH in this patient in a 6-month follow-up. Conclusion: Endoscopic evacuation of CSDH proves to be an effective minimally invasive modality and more studies are required on larger patient groups with long-term follow-up imaging to confirm its superiority.

Median nerve schwannoma with a diagnostic delay of 8 years: a case report.

Introduction: Schwannomas are benign tumors of the peripheral nerve sheath, and the median nerve is the most commonly involved nerve. These benign tumors of the peripheral nerve sheath are very rare; they are clinically and radiologically similar to most other benign swellings of the hand; thus, they are often misdiagnosed. Case Presentation: A 41-year-old lady presented with an 8-year-long history of swelling over the distal forearm. The tumor measured 3.5×3.5×3.5 mm and was located over the flexor aspect of the distal part of her right forearm. Schwannoma was suspected from the clinical presentation and imaging, but the final diagnosis was established only after the surgery and histopathological analysis. At the follow-up after 1 year, the patient is doing well and her symptoms have not recurred. Conclusion: Imaging characteristics of schwannoma can be misinterpreted as some other condition, making the preoperative diagnosis very difficult and important. Thus, clinicians should be aware of such swellings, especially those that have been unnoticed or misdiagnosed, and provide optimal diagnoses to confer good outcomes.

Primary repair of the severe "spaghetti wrist" with good functional outcome measured using quick dash scoring system: Management from LMIC perspective.

INTRODUCTION AND IMPORTANCE: Spaghetti wrist as a part of occupational injury is common among the young adults. Involvement of the multiple neurovascular structures and tendons at wrist may severely impact the quality of life. But if timely intervention can be undertaken the prognosis can be modified with good functional outcome. Moreover the need for common consensus for the appropriate management protocols of such severe traumatic injuries is utmost. CASE PRESENTATION: A 29 years old working male sustained severe cut injury at volar aspect of wrist from glass involving multiple tendons and neurovascular structure as a part of occupational injury. Patient was rushed within 1 h to the nearest tertiary care center and further within 2 h emergency reconstructive surgery was carried out with primary repair of ten tendons, one nerve and one artery. At 9 months follow up patient had satisfactory sensory and motor functional outcome and further at 10 month follow up Qucik Dash scoring system for measuring functional outcome showed significant score suggestive of good functional outcome. CLINICAL DISCUSSION: Spaghetti wrist being one of the grievous hand injuries requiring immediate surgical intervention. The involvement of multiple neurovascular structures and tendons might result in poor functional outcome if there is delay in proper management. Despite absence of adequate guidelines in treatment of such occupational injuries immediate primary repair can be also a significant modality of management. CONCLUSION: Spaghetti wrist being severe traumatic injury without any common consensus for proper management, despite this timely intervention with only primary repair have shown good functional outcome with enhanced quality of life.

Non-adherence to anti-hypertensive medications in a low-resource country Nepal: a systematic review and meta-analysis.

Background: Nepal is a low resource country with cardiovascular diseases being the number one cause of mortality. Despite hypertension being the single most important risk factor for cardiovascular diseases, non-adherence to anti-hypertensive medications has not been assessed systematically. So, this systematic review and meta-analysis aims to analyze the prevalence of non-adherence to anti-hypertensive medications in Nepal. Methodology: This systematic review and meta-analysis was piloted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Electronic databases of Embase, PubMed, Scopus, Web of Science, Cochrane Library, Cinhal Plus, and Google Scholar were searched from inception till 1 February 2023. The random-effects model with 95% confidence interval (CI) was used to calculate the non-adherence rate. Results: Altogether, 14 studies with a total of 3276 hypertensive patients were included in the meta-analysis. The pooled prevalence of non-adherence to anti-hypertensive medications was 49% (95% CI: 0.37-0.62, I2=98.41%, P<0.001). The non-adherence rate using Morisky Medication Adherence Scale (MMAS) was 55% (95% CI: 0.34-0.76, I2=99.14%, P<0.001), and using Hill-Bone Compliance Scale, the non-adherence rate was 45% (95% CI: 0.37-0.54, I2=84.36%, P<0.001). In subgroup analysis, the non-adherence was higher in rural areas 56% (95% CI: 0.51-0.61, I2=0.0%, P=0.46) compared to urban areas 42% (95% CI: 0.31-0.54, I2=96.90%, P<0.001). The trend of non-adherence was increasing after 2020. Additionally, forgetfulness, carelessness, cost of medications, number of comorbidities, and using an alternate form of medication were common factors associated with non-adherence. Conclusions: This meta-analysis showed that half of the hypertensive population of Nepal are non-adherent to their anti-hypertensive medications, thereby posing a significant long-term cardiovascular consequence among Nepali population.

Limb conservation surgery in biphasic synovial sarcoma of thigh with vascular involvement: A race against time.

INTRODUCTION: Synovial sarcomas are malignant soft tissue neoplasm representing 5 to 10 % of all Soft tissue sarcoma with incidence of 2.75 per 100,000. It is presented in particular along with extra articular location with no as such relation to synovial structures. Among various histological pattern biphasic synovial sarcoma (SS) is considered classical type. Involvement of neurovascular structures in synovial sarcoma is least noted and such invasion by malignant cells is considered an indication for amputation. However vessel reconstructive surgeries have also resulted in conservation of limb hence providing good quality of life. CASE PRESENTATION: 13 years old Asian female presented with complaint of exposed synthetic vessel graft from her previous surgical site where femoral vessel reconstruction was carried out for SS of thigh with femoral vessel involvement. Following her (wide local excision) WLE and femoral vessel reconstruction in another center she suffered thrombosis of her femoral vessel following which emergency thrombectomy was also carried out. Later in our center vertical rectus abdominis myocutaneous flap (VRAM) flap was used to manage her exposed synthetic graft. At subsequent follow up patient was ambulating well with no signs of radiological metastasis. CLINICAL DISCUSSION: Surgical resections of mass along with synthetic graft placement of the concomitant vascular bundle have also shown significant improvement in reducing the burden of the severe diseases such as synovial sarcoma. CONCLUSION: SS with its malignant pathophysiology have impacted severely the quality of life of even among the pediatrics group of population. It is utmost need to set up proper and definitive muscular oncological care to reduce the morbidity and mortality associated with such malignancies.

Complete small bowel obstruction due to ileal volvulus with autosomal dominant polycystic kidney disease: A rare case report.

INTRODUCTION AND IMPORTANCE: Volvulus is the twisting of the mesentery of the bowel along its axis. Ileal volvulus is a rare cause of small bowel obstruction. Ileal volvulus coexisting with autosomal dominant polycystic kidney disease has not been reported in the literature previously. CASE PRESENTATION: 65-year male with known history of autosomal dominant polycystic kidney disease (ADPKD) presented with pain abdomen for 5 days, obstipation for 3 days, and multiple episodes of bilious vomiting in the emergency department. Being a suspect of bowel obstruction, X-ray abdomen was done which showed features of small intestine obstruction. Further, to find the etiology of obstruction, contrast enhanced computed tomography (CECT) abdomen was done which showed swirling of the ileal loop and the ileal mesenteric vessels along with transition point in the ileal loop suggestive of ileal volvulus. Exploratory laparotomy with detorsion of the volvulus was done for management. CLINICAL DISCUSSION: Small bowel volvulus, more specifically ileal volvulus, is a rare cause of intestinal obstruction. Patients present with the cardinal features of bowel obstruction, i.e., abdominal pain, distension, vomiting, and constipation/obstipation. Our patient had coexisting ADPKD which further aggravated the clinical presentation. Definitive management of the volvulus includes exploratory laparotomy and detorsion along with resection of the bowel if found ischemic. In our case the bowel was healthy so only detorsion was done. CONCLUSION: Early diagnosis and meticulous exploratory laparotomy is utmost for the management of ileal volvulus. Besides, the secondary etiology (ADPKD in our case) should be managed to prevent future recurrences.