Pure 46, XY gonadal dysgenesis and 46, XY complete androgen insensitivity syndrome: A case report.

BACKGROUND: Disorders of sex development (DSD) are congenital conditions characterized by atypical development of chromosomal, gonadal, and phenotypic sex. 46, XY DSD can result from disorders of testicular development or androgen synthesis. METHODS: We present 2 rare cases of 46, XY DSD, specifically XY pure gonadal dysgenesis and complete androgen insensitivity syndrome. RESULTS: Both cases underwent prophylactic gonadectomy due to the elevated risk of gonadal malignancy. Bilateral gonadoblastoma and dysgerminoma were diagnosed on one side, while Leydig cell hyperplasia and only Sertoli cells were diagnosed in the seminiferous tubules on both sides. The normal menstruation for the pure gonadal dysgenesis patient only as CAIS patients never menstruate. Estrogen replacement therapy was administered periodically to promote the development of secondary sexual characteristics and menstruation in pure gonadal dysgenesis case, as well as to prevent osteoporosis. Follow-up examinations revealed no tumor recurrence, and the patient with Swyer syndrome had regular menstrual cycles. CONCLUSION: Laparoscopic bilateral prophylactic gonadectomy and long-term hormone therapy with patient counseling and support are recommended.

Minimally invasive treatment of uterine necrosis with favorable outcomes: an uncommon case presentation and literature review.

BACKGROUND: Uterine necrosis is a rare condition and is considered a life-threatening complication. However, cases of uterine necrosis were rarely reported, particularly those caused by infection. In terms of treatment, no minimally invasive treatment for uterine necrosis has been reported, and total hysterectomy is mostly considered as the treatment option. OBJECTIVE: The article specifically focuses on minimally invasive treatments and provides a summary of recent cases of uterine necrosis. CASE PRESENTATION: We report the case of a 28-year-old patient gravid 1, para 0 underwent a cesarean section after unsuccessful induction due to fetal death. She presented with recurrent fever and vaginal discharge. The blood inflammation markers were elevated, and a CT scan revealed irregular lumps with low signal intensity in the uterine cavity. The gynecological examination revealed the presence of gray and white soft tissue, approximately 5 cm in length, exuding from the cervix. The secretions were found to contain Fusobacterium necrophorum, Escherichia coli, and Proteus upon culturing. Given the patient's sepsis and uterine necrosis caused by infection, laparoscopic exploration uncovered white pus and necrotic tissue openings in the anterior wall of the uterus. The necrotic tissue was removed during the operation, and the uterus was repaired. Postoperative pathological findings revealed complete degeneration and necrosis of fusiform cell-like tissue. Severe uterine necrosis caused by a multi-drug resistant bacterial infection was considered after the operation. She was treated with antibiotics for three weeks and was discharged after the infection was brought under control. The patient expressed satisfaction with the treatment plan, which preserved her uterus, maintained reproductive function, and minimized the extent of surgery. CONCLUSION: Based on the literature review of uterine necrosis, we found that it presents a potential risk of death, emphasizing the importance of managing the progression of the condition. Most treatment options involve a total hysterectomy. A partial hysterectomy reduces the extent of the operation, preserves fertility function, and can also yield positive outcomes in the treatment of uterine necrosis, serving as a complement to the overall treatment of this condition.