Robotic Assisted En-Bloc Removal of Kidney, Ureter and Bladder Wall for Endometriosis.

STUDY OBJECTIVE: To highlight a case where a nephroureterectomy and partial bladder cystectomy needed to be done due to endometriosis. DESIGN: A video article demonstrating a case study and the surgical management. SETTING: Ureteral endometriosis is a complex form of endometriosis [1]. If left untreated, the ureter can become significantly compressed leading to hydroureter, hydronephrosis and complete loss of kidney function [2]. INTERVENTIONS: This is a case of a 29-year-old patient with pelvic pain and cyclical rectal bleeding. Further investigation showed significant left hydronephrosis and almost complete loss of left kidney function (8% on renogram). MRI revealed endometriosis involving the posterior bladder wall and distal left ureter, a large full-thickness sigmoid nodule and a large left endometrioma. The patient underwent a robotic-assisted left nephroureterectomy, partial cystectomy (bladder), excision of pelvic endometriosis and sigmoid resection. This procedure was performed jointly with the gynecologist, urologist, and colorectal surgeon and the SOSURE technique was employed [3]. The specimen (left kidney, whole length of ureter and bladder wall around ureteric orifice) was removed en-bloc through a small 3cm extension of the umbilical incision. As the distance between the sigmoid nodule and the anal verge was 35cm, which was above the limit of the transanal circular stapler, a limited resection was performed over a discoid excision. The patient made a good recovery postoperatively. CONCLUSION: Ureteral endometriosis is an indolent and aggressive condition which can lead to silent kidney loss. It is essential that hydronephrosis and hydroureter is ruled out in cases with deep endometriosis. Isolated hydronephrosis should also prompt a suspicion for endometriosis.

The Combined Robotic-assisted Laparoscopic and Thoracic Approach in the Management of Diaphragmatic, Pleural, and Pericardial Endometriosis.

STUDY OBJECTIVE: To demonstrate the advantages of a combined robotic-assisted laparoscopic and thoracic approach in the management of extensive diaphragmatic, pleural, and pericardial endometriosis. DESIGN: A video article demonstrating excision of endometriosis from pericardium, diaphragm, and pleura. SETTING: Thoracic endometriosis is the most common site of extrapelvic endometriosis [1]. Surgical treatment aims to excise all visible disease to relief symptoms and prevent recurrence [2-4]. INTERVENTIONS: A 41-year-old lady with cyclical shoulder tip and chest pain and known extensive diaphragmatic endometriosis was referred to our center. The procedure was done jointly by a gynecologist and a thoracic surgeon experienced in robotic-assisted endometriosis excision (Supplemental Video 1). Robotic-assisted laparoscopy revealed extensive full-thickness diaphragmatic endometriosis and a full-thickness pericardial nodule. Pericardial endometriosis excision was performed and a 1 cm defect was left open in the pericardium. Multiple diaphragmatic endometriotic nodules were excised and pleural cavity was entered (Image 2). On robotic-assisted thoracic surgery, further deep endometriotic lesions were detected and excised from the posterior aspect of the diaphragm. These lesions were not identified abdominally despite complete division of falciform ligament, full mobilization of the liver, and the use of a 30-degree scope. Superficial endometriotic lesions on parietal pleura were also detected (Image 3) and excised. The defects on the diaphragm were closed (Image 4). Chest and abdominal drains were left in situ. The patient was discharged on day 4. CONCLUSION: The combined robotic-assisted laparoscopic and thoracic approach is indicated in selected cases and allows full exploration of the thoracic cavity and both sides of the diaphragm, thus preventing incomplete excision of the disease. Robotic surgery also allows smooth dual-surgeon teamwork.

Gynecologic and reproductive health in patients with pathogenic germline variants in DICER1.

OBJECTIVE: Germline pathogenic variation in DICER1 underlies a tumor-predisposition disorder with increased risk for cervical embryonal rhabdomyosarcoma and ovarian sex-cord stromal tumors, particularly Sertoli-Leydig cell tumors. The gynecologic and reproductive health of these females has not yet been described. METHODS: All female subjects recruited from November 2011 to July 2018 participating in an epidemiologic study of families with pathogenic DICER1 germline variation were included in this cross-sectional analysis. Participant evaluation included obstetric-gynecologic history, physical examination, hormone testing, pelvic ultrasound and record review. RESULTS: Of 64 females aged 2-72 years, fifteen underwent treatment for pleuropulmonary blastoma as children and three were treated for cervical embryonal rhabdomyosarcoma. Of nine patients reporting a history of ovarian tumors, all presented with virilization or amenorrhea; eight occurred in adolescence. Post-pubertal females with no history of ovarian tumors experienced normal pubertal development, reported regular menstrual cycles, were fertile and underwent natural menopause at median age of 52 years. Thirty-two of 33 women who tried to conceive successfully delivered liveborn children. Of these 32, 10 experienced pregnancy-related thyroid enlargement resulting in thyroidectomy within one year of pregnancy; nine others had undergone pre-pregnancy thyroidectomy. CONCLUSION: In these DICER1-carrier females, DICER1-related gynecological tumors occurred during childhood or adolescence in some after which women generally experienced healthy reproductive lives. Individual education and screening for these tumors is warranted. The high rate of DICER1-related multinodular goiter resulting in pre- and post-pregnancy thyroidectomy underscores the importance of thyroid monitoring during pregnancy to ensure maternal and fetal wellbeing.