Linda Baker MD Julie Strickland MD MPH

Transcription

1 Linda Baker MD Professor, Department of Urology UT Southwestern, Dallas TX Julie Strickland MD MPH Professor, Department of OB/GYN University of Missouri Kansas City, Kansas City MO Objectives Address the diagnosis, preoperative p assessment and management of challenging surgical cases Provide innovative tricks of the trade Utilize group expertise to analyze difficult clinical situations 1

2 MT A Challenging Case 15 year old referred for primary amenorrhea; possible vaginal anomaly No complaints of abdominal pain or genital bleeding. Pubertal milestones: Thelarche:2 years prior; Adrenarche?2 3 years Growth velocity was normal Primary care physician: Noted on vulvar examination that she had an angle to her vaginal canal,? Vaginal mass; otherwise physical exam/genital findings normal History MT continued: PMH: no prior illnesses PSH: none FH: Mother with menarche at age 14; No related family illness SH: High school student; normal development; lives with family unit; denies drugs alcohol or sexual activity. Swims in season and runs 1 2 miles most days. ROS: Denise headaches, weight gain or loss, increased exercise or activity; no history of constipation or urinary issues, relates good balanced diet without restrictions; dines with the family 2

3 Physical examination/laboratory BP: 102/55, P 80 WT:56.6 Height: 172: BMI:19.2 Well developed well nourished female; no pain or distress Skin: no acanthosis, acne or hirsuitism Breasts: Tanner III Genitalia: Tanner III; normal external genitalia; introitus normal; Q tip placed in vagina with normal vaginal length, some orientation to the left; cervix viewed on left; no vaginal mass palpated although vagina deviates to left Ultrasound 3

4 Other: FSH: 6.74 Prolactin: 3.6 TSH: Estradiol: 18 Renal ultrasound:absent R kidney; otherwise normal Bone age: consistent with chronologic age Diagnosis: Primary amenorrhea consistent with constitutional delay; OHVIRA (Herlyn Werner Wunderlich) Unique features of this case: Patient going to South America in 2 months for extended summer mission trip. Wants to delay surgery until she returns but is worried about the consequences if menarche begins. 4

5 Unanswered questions: With no history of vaginal bleeding and apparent right vaginal obstruction why does she have fluid in the obstructed right side? Patient desires menstrual suppression to allow timing of repair delayed. What is the best option for short term suppression? With no vaginal distention and need for suppression what are the anatomic considerations for repair? What technique should be used to identify anatomic landmarks? Clinical Course /Plan Avoidance of long term suppression in this case Short term menstrual suppression with OCP while out of country. Patient has experienced spontaneous menstrual spotting but no symptoms to date Plan to stop OCP 2 months prior to repair Use of ultrasound guidance/ inflatable catheter to delineate obstructed side with vaginal resection 5

6 Interesting literature Williams C, Nakhal R, Hall Craggs M, et al. Transverse vaginal septae: management and long term outcomes. BJOG. Published online: Santos XM, Dietrich JE. Obstructed Hemivagina with Ipsilateral Renal Anomaly. J Pediatr Adolesc Gynecol 2016 Feb;29(1):7 10. Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome:management and follow up. Fertil Steril Apr;87(4): Silveira SA, Laufer MR.Persistence of endometriosis after correction of an obstructed reproductive tract anomaly J Pediatr Adolesc Gynecol 2013 Aug;26(4):e93 4 Khong TL, Siddiqui J Mallinson P, et al. Herlyn Werner Wunderlich syndrome: uterusdidelphys didelphys, obstructed hemivagina, and ipsilateral renal agenesis role of sonographically guided minimally invasive vaginal surgery. Eur J Pediatr Surg 2012 Apr;22(2): Cooper AR, Merritt DF. Novel use of a tracheobronchial stent in a patient with uterine didelphys and obstructedhemivagina. Fertil Steril 2010 Feb;93(3):900 3 KN A challenging surgical case 46XY DSD patient first presented at age 12. History at birth was of ambiguous genitalia, had gonadectomy, hysterectomy, vaginoplasty, feminizing clitroplasty Sex of rearing: female Surgical findings at EUA: Epispadias, 9 cm torturous urethra, perineal pit 1 2 cm Placed on hormonal regimen for pubertal induction 6

7 KN A challenging case At age 18 underwent laparoscopic sigmoid vaginoplasty, vaginal flap, excision of mullerian reminant labiaplasty, cystoscopy, epispadius repair Started on vaginal dilations, did well initially with only complaints of mucous drainage 3 mo post op: Complained of acute inability to pass dilator Returned to OR with findings of normal vaginal length,stenosis 2 cm into vagina at area of perineal graft junction Had intraoperative dilation to #25 Hegar with inflatable stent placed KN A challenging case 6 month post operative Gradually had more pain/difficulty with dilation Returned to OR for dilation under anesthesia 1 year post operative Continued to have pain/difficulty with dilations with minimal compliance Returned to OR with findings of now stenosis 3 cm from introitus. Lateral excision of stenotic area with free buccal flap placed Inflatable dilator placed post operative Intermittent dilations begun 7

8 KN A challenging case 2 years post operative Compained of progressive difficulty with dilation and pain Returned to OR: Stenotic area excised laterally with mucosal mobilization over defect. Inflatable stent placed and patient resumed dilation 3 years post operative Patient able to dilate to M/L dilator but continued to complain of pain and difficulty with dilation Returned to OR: 28 Hegar dilator passed without difficulty; area with some erythema, friable Started on Estrogen cream with improvement in tolerance to dilation KN A challenging case 5 years post operative Lost to follow up In a relationship but unable to tolerate vaginal penetration Can use large dilator but is painful Using E2 cream and compliant with oral estrogens 8

9 KM Clinical considerations How can we increase vaginal compliance and function for this patient? The etiology of retraction of vaginal perineal suture line to an intravaginal place?short mesentery ;?early dilation Is dilation necessary after sigmoid vaginoplasty? 9

10 Interesting references Wright C, Hanna MK. Thirty six vaginal constructions: lessons learned. J Pediatr Urol Aug;10(4): Nowier A, Esmat M, Hamza RT. Surgical and functional outcome of sigmoid vaginoplasty among patient with variants of disorders of sex Int Braz J Urol May Jun;38(3):380 6 Quint EH, Park JM. Promising surgical innovations involving buccal mucosa for vaginal creation and reconstruction. Obstet Gynecol2014 May;123(5):921 2 Grimsby GM, Bradshaw K, Baker LA. Autologous buccal mucosa graft augmentation for foreshortenedvagina. Obstet Gynecol 2014May;123(5): EP A challenging case 7 year otherwise healthy girl with 6 month history prepubertal vaginal bleeding of uncertain etiology. On vaginoscopy, found to have an exophytic mass arising anterior to and involving the cervix. Final pathology: Clear Cell Carcinoma Workup revealed a T3B lesion with cancer extending to vagina with no metastatic disease Patient was referred to Sloan Kettering Cancer center for consideration for fertility sparing procedure 10

11 EP her clinical course Patient was pretreated with 3 cycles of Cisplatinum/Taxol chemotherapy Surgical procedure: Radical hysterectomy, repair of bladder wall, transposition of ovaries, partial vaginectomy, nephrectoureteroctomy of atretic right kidney, lymph node dissection. Received 2 additional cycles of Taxol and Cisplatinum Local brachytherapy administered after chemotherapy Post operative course Mother instructed to do post radiation dilations. Compliance with dilations was difficult, but continued for 1 year Patient followed with 6 month EUA, vaginoscopy and pap smears which have all been normal Patient with spontaneous signs of puberty age 10 2 years post operative patient complained of incontinence intermittent and primarily post voiding. She reported being wet sometimes after voiding. She denied dysuria, hematuria, but related some urgency. Placed on oxybutrin with some improvement. 11

12 3 years post operative: Patienttaken taken to the operating room for routine vaginal surveillance. At the time of vaginoscopy, lesion noted approximately 1 cm into introitus. No other lesions noted. When lesion explored closely appreared to be fistula with communication immediately inferior to bladder neck. Clinical cases How to fix this now 13 year old with previously radiated pelvis now intrapubertal with urethralvaginal fistula at the bladder neck. How long to continue surveillance? The role of vaginal dilation in children 12

13 Interesting references Tancer ML. A report of thirty four instances of urethrovaginal and bladder neck fistulas. Surg Gynecol Obstet.1993 Jul;177(1): Lester FC, Farmer DL, Rabban JT, Chen LM.Radical trachelectomy for clear cell carcinoma of thecervixin a 6 year old: a case report, review, and description of the surgical technique. J Pediatr Surg Aug;45(8):E1 5 13