Long-term functional outcome of sacrococcygeal teratoma in a UK regional center (1993 to 2006).
ABSTRACT Sacrococcygeal teratoma (SCT) is the commonest neonatal neoplasm. Its long-term effects are important in prenatal counseling and the delivery of an appropriate postoperative plan.
To determine the long-term functional outcome after SCT excision in a UK regional center.
Follow-up data for all patients with a SCT excised at the John Radcliffe Hospital in Oxford was collected retrospectively from notes and prospectively in clinic visits.
Clinical evidence of bowel or bladder impairment, mortality.
Over a 14-year period, 18 patients had a histologic diagnosis of SCT. Nine patients (50%) were born, 7 (39%) were terminated, and 2 (11%) were stillbirths. Of the 9 patients who had SCT resection, 4 (44%) were antenatally diagnosed. There were no perioperative deaths and alpha-fetoprotein levels normalized by 6 to 12 months after tumor resection. Median follow-up of patients was 30 months (range: 6 to 132 mo) with 1 patient lost to follow-up at 6 months, although he was asymptomatic at the time. Three patients developed urologic complications (2 within 1 year of tumor resection), including 2 patients with neurogenic bladder dysfunction and 1 patient with detrusor sphincter dyssynergia.
Approximately one-third of patients will develop major urologic complications after resection of SCT. Routine ultrasonography in the first postoperative year after tumor resection may help to identify patients with neuropathic bladder at the early stage and predict late complications. Parents need to be aware of this potential long-term complication during prenatal counseling and the need for regular long-term follow-up with the pediatric surgical team.
[Show abstract] [Hide abstract]
ABSTRACT: To evaluate the association between the fetal hemodynamic changes seen in vascular tumors of fetal or placental origin and risk of adverse pregnancy outcome. All cases of placental chorioangioma, sacrococcygeal teratoma and pulmonary sequestration during a 10 year were included. Ultrasound data, pregnancy and long-term neurodevelopmental outcomes were assessed in this cohort. A survival analysis was performed to asses the relation between the cardiovascular profile score (CVPS) and adverse pregnancy outcomes. There were 56 fetal-placental tumors, including 28 chorioangioma, 10 sacrococcygeal teratomas and 18 pulmonary sequestrations diagnosed at a median gestation of 23 + 3 weeks. Abnormal CVPS (≤8) scores were seen in 30% of sacrococcygeal teratomas and 46% of chorioangioma, but none of the pulmonary sequestration cases. Adverse pregnancy outcomes occurred in 11 cases (3 stillbirths, 3 neonatal deaths and 5 cases of developmental delay) and only in those cases where the tumours were associated with a CVPS of ≤8. Fetal and placental vascular tumors are predisposed to cardiac dysfunction in fetal life. These cases are at increased risk of both fetal/neonatal demise as well as overt long-term neurodevelopmental handicap only when the CVPS is poor (≤8). The long-term neurodevelopmental outcome should be formally and prospectively assessed in cases of fetal and placental vascular tumors.Ultrasound in Obstetrics and Gynecology 12/2013; 43(6). DOI:10.1002/uog.13272 · 3.56 Impact Factor
Acta Paediatrica 08/2013; 102(8):838. DOI:10.1111/apa.12300 · 1.97 Impact Factor
[Show abstract] [Hide abstract]
ABSTRACT: Foetal counselling is best achieved by a multidisciplinary team that can favourably influence the perinatal management of prenatally diagnosed anomalies and provide this information to prospective parents. Prenatal diagnosis has remarkably improved our understanding of surgically correctable congenital malformations. It has allowed us to influence the delivery of the baby, offer prenatal surgical management and discuss the options of termination of pregnancy for seriously handicapping or lethal conditions. Antenatal diagnosis has also defined an in utero mortality for some lesions such as diaphragmatic hernia and sacrococcygeal teratoma so that true outcomes can be measured. The limitation of in-utero diagnosis cannot be ignored. The aim of prenatal counselling is to provide information to prospective parents on foetal outcomes, possible interventions, appropriate setting, time and route of delivery and expected postnatal outcomes, immediate and long term.Early human development 12/2011; 88(1):9-13. DOI:10.1016/j.earlhumdev.2011.11.004 · 2.12 Impact Factor