Sigmoid neovagina by combined laparoscopic-perineal route for Rokitansky syndrome

OBJECTIVE: To evaluate the feasibility of a combined laparoscopic-perineal procedure to create a neovagina. PATIENTS AND METHODS: We reported four cases of patients with a Mayer-Rokitansky-Küster-Hauser syndrome. The surgical procedure was the same for all patients. The results have been evaluated on the operative time, the intra and postoperative complications, the first day haemoglobin drop, the antalgic drugs consumption, the transit recovery, the hospital stay, and the neovagina length. RESULTS: The mean operative time was 311 minutes. The mean haemoglobin drop was 2.3 g/dl. The mean paracetamol, nonsteroidal anti-inflammatory (NSAID), and morphine consumption were: 16 g, 216.7 mg, and 12.6 mg, respectively. The mean transit recovery, and hospital stay were: 2.3 days, 6.5 days, respectively. No intra or postoperative complication occurred. The mean neovagina length evaluated at one month follow-up visit was 12 cm. DISCUSSION AND CONCLUSION: This surgical technique appeared feasible and reproducible for teams having an adequate experience in laparoscopic gynaecologic and digestive surgery. This technique allowed to obtain a neovagina with enough length and without any shrinkage. Laparoscopy reduces the psychological and aesthetic consequences of surgery, especially for these young patients already distressed by their malformation.     

Squamous Papilloma With Hyperpigmentation in the Skin Graft of the Neovagina in Rokitansky Syndrome: Literature Review of Benign and Malignant Lesions of the Neovagina

BackgroundIt is rare for a benign or malignant neoplasm to develop in a neovagina.CaseThis is the first report of a squamous papilloma with hyperpigmentation which developed in the neovagina 12 years after a McIndoe procedure was done with a split-thickness skin graft from the patient's buttock. The patient had congenital absence of the vagina and uterus (Rokitansky syndrome, Mayer-Rokitansky-Küster-Hauser syndrome, MRKH syndrome). It presented as post-coital blood spotting for 3 months.ConclusionAs a routine, all cases of vaginal construction regardless of the reason for the surgery or the lining of the neovagina should have a continuous annual examination and careful inspection of the entire vagina. Biopsy of any unusual finding should be done. Despite the dark color of the vaginal lesion, bleeding and rapid appearance our patient had a benign tumor. Radiation therapy to the neovagina and prolonged treatment with podophyllin and cauterization should be avoided.     

Late postoperative urinary stress incontinence after a laparoscopic-assisted Vecchietti procedure for neovagina creation in a patient with Rokitansky syndrome: successful treatment with the TVT-O technique

A case report of urinary stress incontinence after surgery in a 20-year-old patient with Mayer-Rokitansky-Küster-Hauser syndrome is presented. A neovagina was successfully created with a laparoscopic-assisted Vecchietti procedure. Fifteen months later, after normal and satisfactory sexual intercourse, the patient was seen for stress urinary incontinence. A second surgery consistent with the transobturator tension-free vaginal tape (TVT) system with nonelastic polypropylene suburethral TVT allowed full correction of the urinary symptoms.     

Laparoscopic creation of a neovagina in patients with Rokitansky syndrome: analysis of 52 cases

OBJECTIVE: To evaluate the surgical feasibility and the long-term anatomic and functional results of a new procedure that uses the endoscopic approach to treat uterovaginal agenesis, known as Rokintansky syndrome. DESIGN: Evaluation of surgical feasibility and recording of clinical data over a 12-month follow-up. SETTING: Tertiary referral center for the treatment of female genital malformations. PATIENT(S): Fifty-two patients with vaginal agenesis. Intervention(s): The laparoscopic version of the Vecchietti method was used to create a neovagina. MAIN OUTCOME MEASURE(S): Anatomic success was defined as a neovagina >/=6 cm long, allowing easy introduction of two fingers, within 6 months after corrective surgery. Functional success was considered achieved if the patient reported satisfactory sexual intercourse starting from 6 months after surgery. RESULT(S): The surgical procedure was performed with no major complications and with 100% anatomic success; functional success was obtained in 98.1% of the study population. CONCLUSION(S): In patients with Rokitansky syndrome, the laparoscopic approach for creating a neovagina by the Vecchietti method is simple, safe, and effective.     

A patient with combined polycystic ovary syndrome and autoimmune polyglandular syndrome type 2

We report a patient with combined polycystic ovary syndrome (PCOS) and autoimmune polyglandular syndrome (APS) type 2. A 26-year-old female presented with polyuria, polydipsia and acute weight loss. She was diagnosed with: (1) type 1 diabetes, with hyperglycemia, impaired insulin secretion, and positive autoantibodies for GAD-65 and IA-2; (2) autoimmune thyroiditis, with hypothyroidism, positive anti-microsomal and antithyroglobulin antibodies; and (3) PCOS, with hyperandrogenic signs that had developed 5 years earlier, amenorrhea for the previous 6 months, and characteristic multiple microcystic appearance of both ovaries on ultrasonography. She is being treated with multiple subcutaneous insulin injections, thyroxine replacement, and cyclic medroxyprogesterone for the aforementioned diseases, respectively. Although several investigations have reported a relationship between PCOS and the individual components of APS, this is the first report of both syndromes occurring simultaneously. Potential mechanisms for their interrelation and the possibility that PCOS is an autoimmune disease are discussed.     

Endometriosis in a patient with Rokitansky-Kuster-Hauser syndrome

This case with Rokitansky-Kuster-Hauser syndrome and right ovarian endometriosis was previously presented as a proof against the transplant theory of Sampson. New operative findings showed a functioning endometrium in a right occult rudimentary horn and retrograde menstruation through the Fallopian tube with hematosalpinx, tubal endometriosis, right ovarian and appendicular endometriosis.     

Pregnancy in a patient with Turner syndrome

Fertility in patients with a diagnosis of Turner's syndrome confirmed by karyotype examination is a very rare phenomenon. Only in 2% of all cases the pregnancy is a result of a spontaneous ovulation and intrauterine fertilization. Due to high proportion of complications occurring in pregnancy, in labour and in puerperium, only minority of patients can expect delivering a healthy infant. Literature data indicate that 40% of patients with Turner's syndrome give birth to a healthy child. Other pregnancies are terminated either by delivering a child with congenital defect or by a spontaneous abortion. We report a case of a 26-year-old patient with Turner's syndrome (46XX/45XO) who menstruated regularly after hormonal replacement therapy. She had regular menstrual bleeding through period of two years, after withdrawal of hormones administration. In march diagnosis of pregnancy was made, as a result of a spontaneous ovulation. The healthy, full-term fetus was born in 40th week of pregnancy.     

Davydov's procedure for the treatment of neovaginal prolapse in Rokitansky syndrome

A 19-year-old woman with Rokitansky syndrome with neovaginal prolapse following self-dilation showed vaginal vault eversion of a 4- × 3-cm neovagina. A modified Davydov procedure was performed. No complications occurred. Vaginoscopy after 6 months showed an iodine-positive 8- × 3-cm neovagina. Functional results were assessed with the Female Sexual Function Index.