Repeat Single-Session Stereotactic Radiosurgery for Cerebral Arteriovenous Malformations: A Systematic Review, Meta-Analysis, and International Stereotactic Radiosurgery Society Practice Guidelines.

BACKGROUND AND OBJECTIVES: Repeat stereotactic radiosurgery (SRS) for residual arteriovenous malformations (AVMs) can be considered as a salvage approach after failure of initial SRS. There are no published guidelines regarding patient selection, timing, or SRS parameters to guide clinical practice. This systematic review aimed to review outcomes and complications from the published literature to inform practice recommendations provided on behalf of the International Stereotactic Radiosurgery Society. METHODS: We performed a systematic review and meta-analysis following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. A comprehensive search of MEDLINE, Scopus, Web of Science, and Embase was conducted. Fourteen studies with 925 patients met the inclusion criteria. Patients were treated between 1985 and 2022. All studies were retrospective, except for one prospective cohort. RESULTS: The median patient age at repeat SRS ranged from 32 to 60 years. Four studies (630 patients) reported detailed information on Spetzler-Martin grade at the time of repeat SRS; 12.54% of patients had Spetzler-Martin grade I AVMs (79/630 patients), 46.51% had grade II (293/630), 34.92% had grade III (220/630), 5.08% had grade IV (32/630), and 0.95% had grade V (6/630). The median prescription doses varied between 15 and 25 Gy (mean, 13.06-22.8 Gy). The pooled overall obliteration rate at the last follow-up after repeat SRS was 59% (95% CI 51%-67%) with a median follow-up between 21 and 50 months. The pooled hemorrhage incidence at the last follow-up was 5% (95% CI 4%-7%), and the pooled overall radiation-induced change incidence was 12% (95% CI 7%-20%). CONCLUSION: For an incompletely obliterated AVM, repeat radiosurgery after 3 to 5 years of follow-up from the first SRS provides a reasonable benefit to the risk profile. After repeat SRS, obliteration is achieved in the majority of patients. The risk of hemorrhage or radiation-induced change appears low, and International Stereotactic Radiosurgery Society recommendations are presented.

Stereotactic radiosurgery for intracranial cavernous malformations of the deep-seated locations: systematic review and meta-analysis.

OBJECTIVE: To determine the outcomes of stereotactic radiosurgery (SRS) for deep-seated (brainstem, basal ganglia, thalamus, cerebellar peduncle) intracranial cavernous malformations (ICMs). METHODS: A systematic review and meta-analysis was performed according to PRISMA and MOOSE guidelines. The main outcomes were comparing pre- and post-SRS hemorrhage rates, using the pooled risk ratios (RR) as the measure of effect. Additionally, the study assessed lesion volume changes and radiation-injury incidence. RESULTS: Data of 850 patients across 14 studies were included in the meta-analysis. The pooled RR of all deep-seated ICMs show a decrease in hemorrhage rate after SRS compared to pre-SRS over the total follow-up period (RR =0.13), initial 2 years (RR =0.22), and after 2 years (RR =0.07). For 9 studies that reported hemorrhage rate of the brainstem only, the pooled RR shows a decrease of hemorrhage rate after SRS compared to pre-SRS over the total follow-up period (RR =0.13), initial 2 years (RR =0.19), and after 2 years (RR =0.07). Volumetric regression was achieved in 44.25% and stability in 56.1%. The pooled incidence of symptomatic and permanent radiation injury was 9% (95% CI, 7-11) and 3% (95% CI, 0-1.9%), respectively. CONCLUSION: SRS appears effective in reducing hemorrhage rates for deep-seated ICMs. The risk of symptomatic radiation injury is low. Given the high risk of surgical morbidity, SRS is a reasonable treatment option for patients with deep-seated ICMs with at least one prior hemorrhage.

Bullous pemphigoid in a previously healthy adolescent: a case report and literature review.

Introduction: Bullous pemphigoid (BP) is considered the most common bullous autoimmune disorder, characterized by autoantibodies directed against hemidesmosomes in the skin and mucous membranes. It usually affects elderly individuals in the sixth through eighth decades of life, with an average age at onset of 65 years. Only a few cases have been reported in children and teenagers. Case presentation: Herein, we report a 17-year-old boy who presented with a pruritic vesicular rash on his arms and legs accompanied by erythema. He was treated at the beginning with topical lotion and acyclovir, but the rash kept deteriorating and eventually bullae appeared, involving also his mouth. A dermatologist was consulted and diagnosed him with BP, and he was treated accordingly. Discussion: BP is the most prevalent autoimmune bullous illness, caused by autoantibodies against hemidesmosomes in the basement membrane of skin and mucosal surfaces, which in turn attract immune cells, including T-cells and neutrophils, and activate them, which causes damage to and separation of keratinocytes, resulting in the bullous formation. Diagnosis can be accomplished by recognizing clinical symptoms supported by histopathological and immunofluorescence testing. Steroids, whether topical or systemic, are the cornerstone treatment; depending on the extent of the disease, other immunosuppressant drugs can be used as a second line. Conclusion: BP manifestations are polymorphic; physicians should keep in mind that they may present with non-bullous, pruritic lesions, which may persist for some days to several months before bullae appear. Although this disease is rare in the young population, it should be considered in the differential diagnosis of bullous lesions.

Testicular adrenal rest tumor in a pediatric patient with congenital adrenal hyperplasia: A case report.

Testicular adrenal rest tumors (TARTs) are benign intratesticular tumors that occur mostly in male patients with congenital adrenal hyperplasia (CAH), their prevalence in these populations can reach up to 94%. We hereby report a male child with known CAH, presented with bilateral irregular testicular masses which were diagnosed as TARTs. TARTs were first reported in 1940, They were named due to their resemblance to adrenal tissue, they are almost always benign but can blunt spermatogenesis and endocrine function of the testis leading to infertility, they are diagnosed by a combination of clinical history, physical exam, and imaging studies, MRI and U/S are equally good for diagnosis and follow-up, treatment includes surgical resection or observation depending on tumor size, symptoms, and fertility goals. TARTs are benign testicular tumors that are strongly associated with CAH, they can be completely asymptomatic or can cause pain and infertility, diagnosis can be done by imaging modalities like MRI or U/S, and treatment options include observation or surgical removal.

Nerve sheath myxoma masqueraded as intramuscular myxoma: an extremely rare tumor with unusual location - a case report and literature review.

Nerve sheath myxoma (NSM) is a very rare benign nerve sheath tumor that mostly affects young adults, with a peak incidence in the 30s. Patients usually present with an asymptomatic swelling commonly affecting the dermis and subcutaneous tissues of the head, neck, and upper extremities. Lower extremities are a much rarer location, and when this occurs, the knee/pretibial region is the most common location. NSM has not been reported within the anterior tibialis muscle. Case Presentation: The authors present the first reported case of NSM in the anterior tibialis muscle in a 39-year-old man presented as deep swelling in the left leg. Discussion: NSM is difficult to diagnose clinically or using standard imaging techniques; MRI and ultrasonography cannot differentiate it from other anomalies. Histopathology alone is not enough to distinguish NSM from neurothekeomas, which were considered synonymous in the past. They can be distinguished by using immunohistochemical markers. Conclusion: Soft tissue tumors in lower extremity muscles are somewhat difficult to diagnose. Excision of the tumor by a specialized orthopedic surgeon and histopathological/immunohistochemical analysis were the only avenues to correctly diagnose the NSM.

Massive hemoptysis as a sole presentation of left atrial myxoma.

Diagnosis of myxoma can be difficult given its variable presentation and while adopting common sense in diagnosing this condition, physicians should also be aware of atypical presentations. Herein we present a 47-year-old heavy smoker presented with massive blood-stained expectoration. He was later diagnosed with cardiac myxoma and managed accordingly.

Ewing sarcoma in calcenous presented as chronic osteomyelitis: a case report and literature review.

Ewing's sarcoma (ES) is an uncommon primary malignant tumor of the bone, mostly affecting 5-20 years of age with male predominance. It commonly affects the diaphysial region of long bones, while the small bones of hands and feet are extremely rare (3-5%) to be affected. The authors report a case of calcaneal ES which was misdiagnosed as bacterial chronic osteomyelitis and treated inappropriately. A high index of suspicion is required in diagnosing tumors at rare sites to avoid undue delay in initiating appropriate therapy. As the overall prognosis of ES of the calcaneum is lower compared to other sites, these tumors should have an extensive radiological evaluation and histological confirmation, as misdiagnosis and treatment delays will have detrimental outcomes.

A report of presacral epidermoid cyst in perimenopausal women: An extremely rare site and an unusual cause of chronic constipation.

BACKGROUND: Epidermoid cyst is rare congenital lesion of ectodermal origin that arises from the remnants of the embryonic tissues. Although epidermal cysts are frequently observed throughout the body, they are rarely found in the presacral regions. It more commonly occurs in women of reproductive age as an asymptomatic, incidental finding during routine physical examination or imaging studies, or during obstetric and gynecologic events. CASE PRESENTATION: A 48-year-old female patient presented with intermittent constipation, lower abdominal and pelvic pain that had developed progressively during the previous six months, which was temporarily relieved with regular laxatives. Magnetic resonance imaging showed a presacral cystic tumor with a high signal intensity on T1-weighted images and, low signal on T2-weighted images with no significant enhancement post-contrast administration. The mass pushed the rectum laterally by external compression. This tumor was diagnosed as a developmental cyst, and total mass resection with negative margins was performed. After that, tumor was histopathologically diagnosed as an epidermoid cyst. DISCUSSION AND CONCLUSION: Epidermoid cyst is a common entity but is rare at presacral space. Also, an epidermoid cyst is an uncommon entity among cystic pathologies found in the presacral region, which includes benign and malignant pathologies. Due to the potential of subsequent infection or cancer, a meticulous clinical examination with correlated radiographic imaging, followed by total mass excision and histopathological evaluation are crucial.

Osteoid osteoma of the rib masquerading as pain due to trauma: Removed by rib resection using preoperative CT-scan guidance.

Osteoid osteomas (OO) are benign bone tumors that are prevalent in young adults. The typical clinical picture of the disease is pain that worsens at night, which may be alleviated by Non-Steroidal Anti Inflammatory Drugs (NSAIDs). The most common imaging finding of OO is a lytic lesion, known as a nidus, with variable intralesional mineralization, accompanied by bone sclerosis, cortical thickening and surrounding bone marrow edema, as well as marked post-contrast enhancement. The most commonly affected sites are the long bones of the lower limbs, but the ribs are rarely reported sites. The present study describes a case of osteoid osteomas located in the rib which was removed by rib resection using CT-scan guidance.

Rapidly enlarging axillary cystic hygroma in a 6-year-old male patient: A case report and literature review.

INTRODUCTION: Cystic hygromas are relatively uncommon tumors of lymphatic origin, it appears most of the time, approximately 90 % before age of 2 years, and it is caused by abnormal development of lymphatic vessels. CASE PRESENTATION: Here we report a case of axillary cystic hygroma in a 6-year-old healthy boy, which presented with the rapid development of a right axillary mass during 3 days, without any predisposing factor. DISCUSSION: Cystic hygromas occur due to complete or partial obstruction of lymphatic vessels, which leads to lack of communication with the venous system, this results in the accumulation of lymphatic fluid and swelling, it occurs in the cervicofacial region most of the time 75 %, but it can arise anywhere in the body, it classically presents as painless, soft mass, diagnosis can be done using ultrasound, CT, MRI, each of which has its advantages, surgical treatment is routinely favored, but other options are also available. CONCLUSION: Axillary cystic hygromas are quite rare, few cases have been reported in fetuses and adults, but only one case in the pediatric age group, thus, in light of these cases, cystic hygromas should be considered in the differential diagnosis of any cystic axillary swelling.

A Case of Systemic Lupus Erythematosus (SLE)-Induced Immune Thrombocytopenia Presented With a Subdural Hematoma.

Systemic lupus erythematosus (SLE) is an autoimmune disease that can cause various health problems, including issues with the blood. One common blood-related symptom in SLE is immune thrombocytopenia (ITP), which leads to low platelet counts. In some cases, SLE patients with ITP may develop a rare but serious complication called subdural hematoma (SDH), which is a type of bleeding in the brain. This combination of conditions can be challenging to manage and has a high mortality rate. In a specific case, a 14-year-old girl with chronic ITP developed a sudden headache and was diagnosed with childhood-onset SLE, leading to the development of SDH. The treatment plan had to be adjusted, and a splenectomy was considered. It's important to be aware of the association between SLE, ITP, and SDH, especially in pediatric patients, and to conduct appropriate investigations in cases of severe headaches, to rule out life-threatening causes.

Extreme Oncoplastic Breast Surgery: A case series of three patients from a lower-middle income country.

Introduction: Oncoplastic breast surgery has become a major player in modern breast surgery.It broadens the indications for breast-conserving surgery. More challenging cases are being treated more with what so-called "extreme oncoplastic breast surgery" which is defined as a breast-conserving operation, using oncoplastic techniques, in a patient who, in most physicians' opinions, requires a mastectomy. Methods: Replacement and/or displacement oncoplastic techniques with contralateral symmetrization to three female patients with breast cancer were done by an oncoplastic breast surgeon. Outcomes: The three patients had smooth recoveries with good aesthetic,oncologic and psychological outcomes. Conclusion: Oncoplastic breast surgery can be a better option than mastectomy with good oncologic, Psychological and aesthetic outcomes, even with extreme cases, yet long-term studies are needed.

Emergency repair of traumatic avulsion of the right main stem bronchus using biliary stent in a 6-year-Old girl: A case report.

Introduction: Pediatric thoracic trauma is a rare condition, but results in high levels of morbidity and mortality. These injuries are often more devastating in the pediatric population due to differences in children's anatomy and physiology relative to adult patients. Tracheobronchial injuries secondary to blunt trauma are difficult to diagnose compared to penetrating trauma. So, a high index of suspicion is of utmost importance. Case presentation: We report a case of a 6-year-old girl who experienced complete avulsion of the right main bronchus. The patient presented with extensive emphysema and severe respiratory distress with bilateral tension pneumothorax. Endotracheal intubation and two thoracostomy tubes were applied. Pneumothorax and a massive air leak persisted on the right side. After thoracotomy, right main bronchus avulsion was present and then repaired by end-to-end anastomosis and muscle flap. minimal air leaks from right chest tubes and partially expanding right lung on chest X-ray are seen after that. So, reinforcement by biliary (instead of bronchial) stent was performed because unavailability of bronchial stent with the desired size at that time. Discussion: Traumatic tracheobronchial damage occurs in just 0.05-3% of all pediatric thoracic traumas. It's fatal condition especially in first hour. With proper recognition and management of these injuries, there is an associated improved long-term outcome. This article reviews the current literature and discusses the initial evaluation, current management practices, and future directions in pediatric thoracic trauma. Conclusion: Biliary stent placement could be a reasonable treatment option for tracheobronchial damage.

Significant response to Pembrolizumab for metastatic cutaneous squamous cell carcinoma in patient with Netherton syndrome.

Background: Netherton syndrome is a rare autosomal recessive disease that presents with a triad of symptoms which include atopic diathesis, ichthyosis linearis circumflexa, and hair shaft abnormality termed "Bamboo Hair". Netherton syndrome patients can develop cutaneous squamous cell carcinoma (cSCC) in unusually young age. Pembrolizumab is the first line treatment for locally advanced and recurrent/metastatic cSCC. Case presentation: A 44-year-old man with a history of Netherton syndrome and multiple skin squamous cell carcinoma was diagnosed with locally advanced and recurrent/metastatic cSCC two years ago. He was started on Pembrolizumab as a treatment for his cSCC. The immunotherapy course was well tolerated with no significant side effects including the expected immune related adverse events seen in patients treated with this medication. PET/CT scan showed significant regression of his disease consistent with partial response according to the response evaluation criteria in solid tumors. Discussion: Incurable and recurrent cSCC tends to metastasize, leading to an extremely poor long-term prognosis, and the treatment options for locally advanced or metastatic disease are few. Pembrolizumab, an immune checkpoint inhibitors (ICIs) showed a benefit in patients with various tumors including squamous cell carcinoma, but using this drug which is working by enhancing the immunity against tumor in patient with altered immunity like Netherton syndrome was a bit of a challenge, in terms of both effectiveness and safety. Conclusion: Pembrolizumab had a effective and safe treatment profile when it was used as a monotherapy for treating a Netherton syndrome patient diagnosed with locally advanced and recurrent/metastatic cSCC.

Surgical management of bilateral scapular winging in a previously healthy 10-year-old boy: A case report.

Introduction: Scapular winging is a pathological condition that occurs due to dysfunction of any scapulothoracic muscles, most commonly the serratus anterior which is innervated by long thoracic nerve. Case presentation: Herein, we report a 10-year-old boy presented with bilateral scapular winging for a few months, associated with vague discomfort upon shoulder movements. After taking history and performing physical examination and tests as electromyography, he was diagnosed with idiopathic bilateral scapular winging as there was no obvious cause or risk factor for his bilateral scapular winging. Discussion: Scapular winging causes decrease strength and range of motion of the shoulder, the usual complain of patients is discomfort or the unpleasant cosmetic appearance of scapular prominence, this condition, if left untreated, can cause various negative consequences on the shoulder joint, detailed history and physical exam reinforced by electromyography are crucial in determining the cause of winging, as it has many causes, initial management can be conservative, but various surgical approaches have been described for more severe cases which compromise shoulder function, choice of the surgical approach depends on the underlying cause and overall clinical picture of the patient. Conclusion: bilateral scapular winging is rare condition, only 26th cases were reported in literature, it occurs due to multiple causes, treatment can be conservative at early stages, but surgical option should be considered when shoulder function and strength become compromised.

A child diagnosed with midaortic syndrome and inherited thrombophilia after presenting with a stroke: A case report.

A 5-year-old female patient presented to the emergency room with right upper limb weakness, inability to speak and left side deviation of the mouth. Computed tomography (CT) scan was done and she was diagnosed with ischemic stroke. During her medical evaluation she was found to have high blood pressure (186/104 mm Hg). Investigations done to evaluate for secondary causes of hypertension and stroke revealed that she has inherited thrombophilia and abdominal aortic coarctation. Balloon angioplasty was done that lowered her elevated blood pressure, which was not responding well to antihypertensive medications, to near normal value for her age.