Case Presentation Ryan Hsi, MD

Case Presentation HPI: 2 day-old F transferred with sacral mass found incidentally at birth. +stooling. Birth History: 39 wk EGA, Apgars Mother smoking cessation ~6 wks EGA.

Family History: Maternal GM with ovarian teratoma (great GM used DES) Exam: 2x4cm midline pedunculated mass just cranial to anus Outside U/S

Urologic conditions in children with sacrococcygeal teratoma Ryan Hsi, MD

Outline Case presentation BackgroundClassification Associated urological abnormalities Late urological sequelae after resection –Etiology –Urodynamic findings –Comments Summary

Background Pluripotent cells in the primitive streak Contains tissue from 3 embryologic layers 1:27,000-40,000 newborns ~50% prenatal detection rate Most common neonatal neoplasm 3-4 : 1 female predominance

Background Treatment consist of surgery and selective chemotherapy Overall, 17% malignant (Altman et al.) –Benign tumors >95% survival (Altman et al.) –Malignant tumors up to 89% survival at 7.6 years (Rescorla et al.)

Classification Altman et al. (1974) –Type I predominantly external (46.7%) –Type II external with significant intrapelvic extension (34.7%) –Type III visible externally but predominately pelvic and abdominal extension (8.8%) –Type IV entirely presacral (9.8%)

Associated Defects –Anorectal malformation, sacral partial agenesis, presacral mass (Currarino’s Triad) –Congenital heart disease –Exomphalos with scoliosis –Congenital heart disease –Cerebral ventriculomegaly –Cleft palate –Congenital dislocation of the hip –Esophageal atresia

Milam et al. J Urol, –Usually related to direct obstruction Urinary retention (21%) Hydronephrosis (21%) Hydrocele (14%) Undescended testis (3%) –Malformations of renal tract not increased Associated Urologic Defects

Overall associated in 22-28% of casesHydronephrosis Vesicoureteral reflux Bladder distention Urinary retention Hydrocele Undescended testes Urethral obstruction from distortion and compression. Ozkan et al. J Urol, 2006

Outline Case presentation BackgroundClassification Associated urological abnormalities Late urological sequelae after resection –Etiology –Urodynamic findings –Comments Summary

Late urologic sequelae Reports of urinary tract complications are few Discussion of neurogenic bladder dysfunction and urodynamic evaluation rare Commonly associated with bowel symptoms –Constipation/diarrhea –Fecal incontinence

Long term urologic sequelae Reinberg et al. J Urol, –Most common urologic problems associated with SCT Neurogenic bladder (12%) Ureteral obstruction (10%) Vesicoureteral reflux (7%) –81% incidence of urological complications with Type IV (presacral disease)

Etiology Neurological injury associated with tumor resection Relationship of tumor to the major pelvic nerves –Pudendal nerve (somatic to sphincter) –Presacral nerves (parasympathetic) –Pelvic nerve plexus (sympathetic)

Campbell’s Urology, 9 th ed. Figure

Urodynamic Studies Boemers et al. J Urol, –9 of 11 children with benign sacrococcygeal teratoma had abnormal urological studies 2 detrusor instability 2 anatomical infravesical obstruction 5 neurogenic bladder with sphincter dysfunction –2 detrusor hyperreflexia with sphincter dyssynergia –2 hyporeflexic bladder-sphincter function –1 detrusor hyporeflexia with normal sphincter

Urodynamic Studies Ozkan et al. J Urol, –14 patients referred to urologist with UTI or incomplete emptying after resection at infancy 8/14 detrusor instability 2/14 detrusor hyporeflexia 5/13 detrusor – sphincter dyssynergia –Sphincter EMG in 7/13 patients demonstrating lower motor neuron lesion –Vesicoureteral reflux in 7 patients –Hydronephrosis in 6 patients ( (5 with VUR)

Ozkan et al. J Urol, (cont’d) –11/14 patients required clean intermittent catheterization and/or anticholinergics to stay dry –4 patients required bladder augmentation for small hyperreflexic bladder –Antireflux surgery performed in 6 children for grade 4 or 5 reflux

Comments After teratoma resection, children with bowel disturbance commonly also have a bladder-voiding disorder Onset of bowel and bladder dysfunction may present as late as school age Functional disorders improve over time

Summary Perinatal urological manifestations of sacrococcygeal teratoma are primarily from obstruction Postoperative complications usually neurogenic in origin.

Summary Neurogenic bladder most common long-term urologic sequelae, followed by ureteral obstruction and vesicoureteral reflux Urologic workup may require urinary tract ultrasound, VCUG, and urodynamics with EMG Treatment options individualized May need lifelong follow-up

References Altman P et al. Sacrococcygeal teratoma: American Academy of Pediatrics surgical section Survey. J Pediatr Surg 1993;28: Rescorla et al. Long-term outcome for infants and children with sacrococcygeal teratoma: A report from the Childrens Cancer Group. J Pediatr Surg 1998;33:171-6 Ozkan KU et al. Neurogenic bladder dysfunction after sacrococcygeal teratoma resection. J Urol 2006;175: Milam D et al. Urological manifestations of sacrococcygeal teratoma. J Urol 1993;149:574. Tailor J et al. Long-term functional outcome of sacrococcygeal teratoma in a UK regional center (1993 to 2006). J Pediatr Hematol Oncol 2009;31: Draper H et al. Long-term functional results following resection of neonatal sacrococcygeal teratoma. Pediatr Surg Int 2009;25: Cozzi F et al. The functional sequelae of sacrococcygeal teratoma: a longitudinal and cross-sectional follow-up study. J Pediatr Surg 2008;43: Gabra HO et al. Sacrococcygeal teratoma – a 25-year experience in a UK regional center. J Pediatr Surg 2006;41: Schmidt B et al. Sacrococcygeal teratoma: clinical course and prognosis with a special view to long-term functional results. Pediatr Surg Int 1999;15: Reinberg Y et al. Urological aspects of sacrococcygeal teratoma in children. J Urol, 1993;150:948. Havranek P et al. Sacrococcygeal teratoma in Sweden between 1978 and 1989: long-term functional results. J Pediatr Surg 1993;27: Lahdenne P et al. Late urologic sequelae after surgery for congenital sacrococcygeal teratoma. Pediatr Surg Int 1992;7: Malone PS et al. The functional sequelae of sacrococcygeal teratoma. J Pediatr Surg 1990;25: Boemers TM et al. Lower urinary tract dysfunction in children with benign sacrococcygeal teratoma. J Urol 1994:151:174. Pictures Kumar Robbins and Cotran Pathologic Basis of Disease, Professional Edition, 8th ed. Adam - Grainger & Allison's Diagnostic Radiology, 5th ed. Campbell’s Urology, 9th ed. Figure Townsend - Sabiston Textbook of Surgery, 18th ed.