Sacrococcygeal teratoma with hydrops fetalis and bilateral hydronephrosis

Abstract

A 29-year-old Japanese primipara with fetal sacrococcygeal teratoma and hydronephrosis was referred to our department at 22 weeks of gestation. The preload index of the inferior vena cava (PLI) was 0.75 and fetal cardiac failure was suspected at 28 weeks of gestation. The sodium and chloride concentrations and osmolarity of the fetal urine were 96 mEq/L, 81 mEq/L and 204 mOsm/L, respectively, and we predicted a good renal function. Then, a vesico-amniotic shunting operation was performed at 28 weeks of gestation to keep the renal function and PLI decreased 0.41 after this operation. At 30 weeks of gestation, the fetus was delivered by a cesarean section owing to preterm PROM and a huge growing sacrococcygeal teratoma. The baby was female, weighing 2,020 g, and the 1 minute Apgar score was 1. She died almost 7 hours after birth due to respiratory insufficiency. An autopsy confirmed bilateral hydronephrosis, urethral stenosis, sacrococcygeal immature teratoma, and pulmonary hypoplasia. The size of this tumor revealed 80 x 70 x 45 mm in intrapelvic cavity and 130 x 90 x 50 mm out of body, and this tumor was classified as Type II according to the American Academy of Pediatrics Surgery Section classification.

Management and outcome in prenatally diagnosed sacrococcygeal teratomas

“…A postnatally useful clinical grading scheme was proposed by the American Academy of Pediatrics Surgical Section, describing types I–IV with regard to their location 8 . Depending on its size and location, SCT, especially type III (tumor primarily presacral, small external component) and type IV (tumor completely presacral, may extend into the pelvis or abdomen) often leads to secondary effects such as bladder outlet obstruction, hydronephrosis, 9,10 rectal atresia, 11 sacral bony abnormality, or fetal cardiac failure 12–14 . A marked increase in tumor size is often associated with local compression effects and the development of fetal hydrops 10 …”

Sacrococcygeal Teratoma

“…4,5 Sacrococcygeal teratomas are classified according to the American Academy of Pediatrics Surgery Section as types I through IV based on their location. 4,6,7 Type I tumors, which are the most common, are predominately external, with a small presacral component. Type II tumors have an external component, with significant intrapelvic extension.…”

“…5 By using the information obtained from serial sonography scans, pregnancy management and appropriate treatment can reduce the mortality rate of such cases. 7 …”

Fetal Sakrokoksigeal Teratom-Prenatal Tanı ve Yönetim Olgu Sunumu

“…Sakrokoksigeal teratomu olan fetusta hidrops gelişimi, fetal ölümün erken işaretidir. Fetal hidrops, büyü-yen tümörün vasküler kaçağı sonucu yüksek outputlu kalp yetmezliğine bağlı olarak ortaya çıkar (6). Ultrasonografide solid komponent hakimiyeti olan ve artmış vaskülarizasyonu olan teratomlu fetuslarda in utero hidrops gelişme riski yüksektir (7).…”