Successful surgical excision of the accessory urethra in a 13-year-old boy with Effmann Type IIA-2 urethral duplication. Case report and literature review.

INTRODUCTION AND CLINICAL IMPORTANCE: Urethral duplication is a congenital anomaly characterized by the partial or full development of an auxiliary urethra as a second urethral channel varying in extent and location. The course of treatment for urethral duplication should be individualized for each patient based on the type of anomaly and the existence of symptoms. In most cases, if the ventral orthotopic urethra is normal, excision of the dorsal auxiliary urethra is almost always curative. CASE PRESENTATION: We report a case of a 13-year-old boy who presented with recurrent urinary tract infection, occasionally having drops of urine at an external pin hole meatal opening on the dorsum of the penile shaft. Several investigations were performed to confirm the presence of the dorsal accessory urethra. The patient underwent complete excision of the accessory urethra, and 12 weeks post-surgery the patient was completely asymptomatic. DISCUSSION: Urethral duplications are rare anomalies. These anomalies have been classified in various ways in the literature, including sagittal and lateral duplications, dorsal and ventral duplications. The sagittal plane is where the majority of urethral duplications occur. Based on the anatomic variation, clinical presentation and severity of the anomaly, urethral duplication treatment should be customized for each patient. An asymptomatic child may require no specific treatment. Total surgical excision of the dorsal accessory urethra appears to offer the best curative solution. CONCLUSION: Excision of the dorsal accessory urethra is usually curative when the ventral orthotopic urethra is normal. Thorough investigation before surgery is mandatory to determine the type of urethral duplication. The most common diagnostic procedures for urethral duplications include cystoscopy, KUB ultrasonography, and micturating cystourethrography. These procedures can help plan surgery, prevent complications such as neurovascular bundle injuries and urine incontinence, and improve patient self-esteem and quality of life.

A rare case report of ectopic-adrenal paraganglioma with inferior vena cava infiltration. Case report and literature review.

INTRODUCTION AND IMPORTANCE: Phaeochromocytomas are neuroendocrine neoplasms arising from chromaffin cells of the adrenal medulla and are known as intra-adrenal paragangliomas. On the contrary, paragangliomas are non-epithelial neuroendocrine neoplasms that produce other peptide hormones such as norepinephrine, epinephrine, and dopamine. In a setting with limited resources, such a condition may result in inaccurate diagnosis and treatment, which may lose a patient life if left untreated. In addition, PPGLs present a surgical challenge to many surgeons who are not thoroughly prepared. CASE PRESENTATION: We describe a case of extra-adrenal paraganglioma in a 27-year-old female who presented with symptoms of heartbeat awareness for almost a year and abdominal swelling for three months. Physical examination revealed a palpable mass around the umbilicus. Abdominal pelvic CT scan revealed a well-circumscribed mass in the central retroperitoneum displacing the IVC laterally and abdominal aorta medially with no invasion of surrounding structures. Results for pheochromocytoma workup showed elevated levels of 24-h urine Vanillylmandelic acid (VMA), and 24-h metanephrine excretion. Laparotomy through extended midline incision was done with successful excision of the tumor. The blood pressure, which was initially high normalized after surgery. DISCUSSION: The diagnosis of a malignant EAP is commonly made based on recurrence and the development of metastasis to lymph nodes or to other organs. Our patient presented with similar symptoms as pheochromocytoma. Compared to the commonly stated 10 % risk for pheochromocytomas, people with EAPs have been observed to have a 20 % to 50 % incidence of malignancy. EAPs account for at least 15 % of adult and 30 % of pediatric pheochromocytomas and are frequently found in the Zuckerkandl organ. Pheochromocytoma symptoms may manifest asymptomatically in patients with EAPs, or they may occur because of compression of nearby tissues. Surgical excision of the tumor is always the best option in managing EAP. CONCLUSION: Extra-adrenal paragangliomas can be successfully managed surgically, provided there is a multidisciplinary team, and the tumor location is well recognized and can reduce the chance of tumor metastasis.

"A successful reconstruction of remnants of corporal bodies and penile urethra after Total Glans Penis amputation following surgical circumcision in a 5-year-old boy". A case report and literature review.

INTRODUCTION AND IMPORTANCE: Circumcision is the most common surgical procedure done in boys. Its complication varies from minor to severe. In most of African countries circumcision is often performed by traditional circumcisers. The management of penile glans amputation depends on the duration before presentation with auto-transplantation being the favorable management in acute phase of presentation. The amputation of the glans is a serious complication of circumcision because it can derange the urinary and sexual functions of a patient and may lead into psychological instability and low self-esteem. CASE PRESENTATION: We report a case of total glansectomy in a 5-year old boy who presented 12 months after surgical circumcision which was done by inexperienced medical personnel at his house. Patient presented with a pinhole urethra opening, missing glans, buried phallus with remnants of corporal bodies. Penile exploration was done, with successful significant phallus stump of about 5 cm, phallus reconstruction and meatoplasty was achieved. Partial Skin thickness grafting of the phallus stump was performed. At six- month follow up, the patient had both satisfactory cosmetic and functional outcomes. CLINICAL DISCUSSION: Total glansectomy is a rare surgical emergency with auto transplantation being the best option in acute management however successful outcome for a delayed glansectomy depends on proficient surgical penile exploration and comprehensive post operative care. Based on the clinical presentation, the goals of management for our patient were to achieve a largely patent urethral opening in the long term and to prevent further urethral meatal stenosis as well as achieving adequate phallus stump for sexual function. CONCLUSION: The amputation of the glans is a serious complication of circumcision because it can derange the urinary and sexual functions of a patient if neglected. Findings on this case report highlight the importance of raising awareness among traditional health care workers to prevent urinary and sexual function disabilities. Prevention of penile amputation can be prevented by providing proper training for circumcisers and providing public education for hospital circumcision.

An extremely rare neoplasm 'atypical spindle cell pleomorphic lipomatous tumor': a case report.

Spindle cell lipoma is a rare histological variant of lipoma accounting for 1.5% of adipocytes tumors. It is composed of an admixture of mature adipocytes and fibroblast-like spindle cells in a myxoid stroma. Retroperitoneal atypical spindle cell lipomatous tumor are extremely rare only a single report in the literature reported. Herein we describe a case of a giant lipomatous tumor that was causing partial bowel obstruction. Successful excision with en block resection of the tumor, distal ureter and posterior wall of the bladder was achieved by teamwork between gastrointestinal surgeons and Urologist. The bladder defect was repaired, and left ureteric reimplantation was done. The patient made a good recovery with excellent progress. He has resumed his normal activities as a farmer.