Gynecological Considerations in Patients with Cloacal Malformations: From Antenatal Diagnosis through Evaluation to Final Reconstruction Geri Hewitt, MD and Richard J. Wood, MD Center for Colorectal and Pelvic Reconstruction Nationwide Children s Hospital The Ohio State University College of Medicine.... Neither Dr. Geri Hewitt nor Dr. Richard Wood have any disclosures. 1
Cloaca Objectives for this session Discuss antenatal diagnosis and planning Describe the neonatal management of hydrocolpos Describe the anatomical assessment Describe operative procedures for PSARVUP, TUM & Vaginal replacement Discuss long term follow-up: Gynecology, Urology and Bowel Management 2
Antenatal Diagnosis No standard criteria Soft markers on antenatal ultrasound: abdominal or pelvic cystic mass, hydrocolpos, absent kidney, two vessel cord, dilated bladder, abnormal spine Antenatal Diagnosis ARMs are diagnosed antenatally 0-15% of cases; no data of incidence of antenatal diagnosis of cloaca Suggested counseling for families: consider delivery at tertiary care, TEF or cardiac anamlies 3
Make the Diagnosis Remain calm Logical approach Rectum Colostomy 4
Single opening on perineum Cloaca Single opening on perineum Cloaca Renal and Pelvic USS (Day 1) 5
Single opening on perineum Cloaca Renal and Pelvic USS (Day 1) Hydrocolpos +/- Hydronephrosis No Hydrocolpos Drainage of hyrdocolpos What is your practice currently? A. Intermittent perineal catherization B. Tube vaginostomy with IR C. Formal surgical vaginostomy with or without tube 6
Single opening on perineum Cloaca Renal and Pelvic USS (Day 1) Hydrocolpos +/- Hydronephrosis No Hydrocolpos Vaginostomy + Colostomy Colostomy Cloacagram and Cystoscopy Initial management Make the diagnosis: Perineal Examination VACTERL screening Associated life threatening malformations Patient requires a colostomy Exclude hydrocolpos or hydronephosis (or both) Definitively drain hydrocolpos at the time of the colostomy formation Ensure ongoing drainage in the post operative period Vesicostomy if required 7
Definitive assessment of the anatomy: Examination under anesthesia Cystoscopy and vaginoscopy Cloacagram 2D/3D (depending on availability) Multidisciplinary Team: Colorectal, Urology, and Gynecology 3 and 6 months of age Short common channel Long common channel 8
Hydrocolpos Hemivagina Definitive Anatomic Diagnosis 9
Cloacagram and Cystoscopy 1. What is the length of the common channel? 2. What is the length of the urethra (takeoff to bladder neck)? 3. How many vaginas/cervices? 4. Can I reach the rectum? Short Common Channel Long Common Channel 10
Short Common Channel (1-3cm) Urethra, vagina and rectum require reconstruction Very few exceptions in this group Anatomy is predictable PSARVUP is a reproducible operation: Total Urogenital Mobilization Very rarely- short urethra (<1.5 cm) Complex problem Needs to be recognized pre-operatively 3-5cm Common Channel 11
Transabdominal TUM vs. Urogenital Separation 3-5cm Common Channel If the urethra: from CC to bladder neck is less than 1.5-2 cm Be very cautious of doing a TUM as this will likely lead to a short dysfunctional urethra 12
3-5cm Common Channel Transabdominal TUM: by definition - dissection of the anterior wall of the urethra If it doesn t reach!!!! Urogenital separation: by definition - dissection of the posterior wall of the urethra RISK URETHRAL LOSS Long common channel cloaca (>5cm) Complex problem Complex urologic abnormalities Unpredictable anatomical variations Need for vaginal replacement Combined posterior sagittal and abdominal approach Should be referred to a Center with specialized expertise 13
Long common channel cloaca (>5cm) Posterior sagittal approach to start Urethra: Common channel becomes neourethra Vagina: Separation of vagina from urinary tract Rectum: Mobilization of a high rectum 14
Maneuvers to get the vagina to reach the perineum 1. Mobilization of native vagina 2. Vaginal Switch Procedure 3. Vaginal Replacement Colon Small bowel Rectum Vaginal switch 15
Vaginal switch Vaginal Replacement (colon) 16
Vaginal replacement (small bowel) Vaginal replacement (rectum) - Longitudinal transection 17
Vaginal replacement (rectum) - Transverse transection Common pitfalls High volume center: 25 cloaca per year Pattern recognition UG sinus not addressed Inadequate introitus, perineal body Inadequate intra-operative planning: leading to mislocation Tissue loss, sepsis, strictures: inadequate mobilization 18
Long term follow up/urology Renal ultrasound and cystatin C for assessment of kidney growth and function Assessment around age 5 for likelihood of successful potty training/continence: bladder capacity and characteristics (video urodynamics), anatomic considerations, kidney size and function, success of intermittent self catheterization,.... Long term follow up/urology Interventions considered: Mitrofanoff appendicovesicostomy:? Location, Bladder augmentation +/- ureteric re-implantation, Bladder neck reconstruction or closure.... 19
Long term follow up/gyn s/p reconstruction and best assessment of anatomy Opportunities to assess anatomy: EUA/vaginoscopy imaging abdominal procedures (cpt code).... Long term follow up/gyn Pubertal milestones/thelarche Reassess mullerian structures once endometrial proliferation would be expected Reproductive potential/obstetrical complications/menstrual eggress Menstrual history/dysmenorrhea/obstruction Education about tampon use Address vaginal septum if not already corrected Anticipatory counseling for patient and families.... 20
Long term follow up/gyn Post menses Concerns: outflow track obstruction/introitus and assessment vaginal caliber/length re: sexual functioning If vagina is not adequate for sexual activity, consider options Audience experience re: use of vaginal dilators/our current practice Anticipatory counseling re: sexuality/body image/contraception/sexual practices Role and timing of introitoplasty.... Long term follow up/gyn Obstetrical considerations Limited literature Potential obstetrical complication/preconceptual counseling Reproductive potential Mullerian abnormalities Medical co-morbities: TEF, Nissen, cardiac, renal, regional anesthetic.... 21
Long term follow up/gyn Mode of delivery No good data Non-obstestrical indications for cesearan section: cloacal reconstruction and/or nonnative vagina Shared medical decision making with PSARP and adequate perineum comprehensive patient counseling.... Audience experience with delivery Has anyone had this experience? A. Yes B. No How would you approach this now after that experience? 22
Surgical conduit placement Mitrofanoff and/or Malone placement/location and surgical delivery risks/considerations Bowel management: Predictions of continence 23
Laxatives vs Enemas Acquired sensation Water soluble fiber Antegrade vs Rectal enema systems Antegrade enemas.... 24