Urethral reconstruction using amniotic membrane allograft in hereditary androgen insensitivity syndrome: a case series.

Partial androgen insensitivity syndrome is a rare X-linked disorder. While most cases are sporadic, familial cases are less frequent. The management of this syndrome follows a multidisciplinary approach involving hormone substitution, psychological counseling, and surgical procedures. We present a case series of three young siblings with familial partial androgen insensitivity syndrome who presented with a female phenotype. All of them were managed with hormonal treatment for 6 months followed by surgical reconstruction. The operative procedure involved phalloplasty and urethroplasty using amniotic membrane transplant, which is considered a novel technique in this group of patients. No intraoperative or postoperative complications were observed and good results were achieved within 2 years of follow-up.

Successful management of ectopic kidney stones in a patient with situs inversus totalis: a rare case report.

BACKGROUND: Situs inversus totalis is a very rare congenital anatomical variation, in which all thoracic and abdominal organs are right-left inverted. This condition is associated with an increased risk of organ malformations including ectopic kidney, which is a very rare combination. CASE PRESENTATION: A 56-year-old male presented with colicky left iliac pain associated with nausea, vomiting, and irritative lower urinary symptoms. The patient has a medical history of recurrent lower urinary infections and a family history of situs inversus totalis. Radiological images demonstrated dextrocardia, situs inversus totalis of all the abdominal organs, and an ectopic pelvic kidney on the left side, with 4 stones inside it. Left nephrectomy was performed due to extensive renal damage. At discharge and during follow-up, the patient's condition was satisfactory and stable. CONCLUSIONS: The ectopic kidney may present diagnostic and therapeutic challenges when associated with situs inversus.

Multiple vas deferens with polyorchidism and many congenital malformations in a symptomatic 11-year-old male patient: a rare case report.

BACKGROUND: Ductus deferens may manifest in a variety of anomalies such as its absence, duplication, ectopy, or diverticulum. Ectopic seminal tract opening has two main types, ectopic ejaculatory duct opening, and ectopic vas deferens opening. Generally, ductus deferens anomalies affect approximately 0.05% of the population. Patients may be asymptomatic or complaining of urinary tract infections and/or epididymitis. Most of these cases are associated with renal dysplasia. To confirm the diagnosis Cystourethroscopy catheterization and retrograde urethrogram should be performed, but the definitive diagnosis is done by vasography. The definitive treatment is complete surgical resection of the pathological urogenital connection. This case is commonly discovered while exploring other findings such as testicular torsion and inguinal hernia. CASE PRESENTATION: We report a rare case of an 11-year-old male who presented with gross hematuria and numerous congenital malformations including a left polydactyly clubfoot, polyorchidism, with several surgical procedures, and left kidney dysgenesis. Surgery was performed for a left inguinal hernia, during which a third undescended testicle was discovered incidentally and was eradicated. A retrograde urethrogram was performed to establish the diagnosis. A fistula- that is connected with the left ureter- was resected. The histopathologic findings confirmed the diagnosis of true duplication of the Vas deferens, with communication between the ureter and the vas deferens. By follow-up, the kidney function tests were within normal limits. CONCLUSIONS: This case report aims to highlight the early diagnosis and management of the duplicated vas deferens and the associated congenital malformations to improve the prognosis and kidney function and to avoid long-term complications.