SIMPLE ALGORITHM IN THE MANAGEMENT OF FETAL SACROCCOCYGEAL TERATOMA IN DEVELOPING COUNTRIES

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A. Eighemhenrio

Department of Surgery, Irrua Specialist Teaching Hospital, Irrua, Edo State, Nigeria.

Abstract

Sacroccocygeal teratoma is the commonest tumour in neonates. It is more common in females in about 80% of cases. Associated congenital abnormalities may be seen coexisting with sacroccocygeal teratomas in 10-20% of cases. Early diagnosis can be made through fetal ultrasound scan (USS).The presence of this tumour can have serious complications for both the fetus and mother. Apart from USS other specialized tests can be done, these may not be available in developing and low resource countries. A simple algorithm of management using USS and clinical findings can be adapted for use in these locations.

Keywords: Sacroccocygeal Teratoma, Algorithm, Ultrasound Scan

Introduction

Teratomas are embryonal tumours which originate from primordial germ cell. They are derived from at least two of the three germ cell layers. Teratomas involve the gonads as well as extragonadal parts of the body. The sacroccocygeum represents the commonest extragonadal location of teratomas. Sacroccocygeal teratoma (SCT) is the commonest neoplasm of the fetus and newborn.

The incidence of SCT is 1 in 20,000 to 1 in 40,000¹ Male to female ratio is 1:4.² In most studies about 80% of SCT are benign (mature).The malignan (immature) tumors are slightly more common in males and in neonates (68%) and older children (73%).³

Patients with SCT may have some associated abnormalities like anorectal malformations, spinal defects and oesophageal fistula in about 10% -20% of cases². Early prenatal diagnosis is crucial in the management and outcome of pregnancy and well being of both mother and child.

The presence of this tumour in a fetus can have significant effect on the health of the pregnant mother as it may be associated with severe anaemia, cardiac failure, maternal mirror syndrome and even death. The developing fetus with SCT is prone to high output cardiac failure, hydrops, placentomegaly and complications of delivery.

Understanding a simple way of managing this condition is important in developing countries to prevent morbidities and mortalities. A simplified algorithm of management will help in following the progress of pregnancy in such women and also aid in determining when and how to intervene. Some algorithms are present in literature, none however is designed for the developing countries where most centers are not sufficiently equipped.

This article describes a simple algorithm that can be adapted for developing countries in the management of fetal SCT.

PRENATAL DIAGNOSIS
Prenatal diagnosis is achieved by a maternal-fetal ultrasound scan (USS).This show the tumour protruding from the sacral region with or without intrapelvic extension. Antenatal diagnosis is usually made after 22 weeks of gestation.⁴ Some reported cases of earlier prenatal diagnosis have been documented.⁴ Routine USS can diagnose many cases of asymptomatic SCT. However the most common indication for antenatal USS in fetal SCT has been a uterus that is large for gestational age either due to polyhydramnios or rapid growth of the tumour.⁴

Screening methods such as amniotic fluid alphafetoprotein or maternal serum screening have not proven to be effective but can be used postnatally to differentiate between benign and malignant SCT.^5,6 Postnatal persistence in the level of alpha-fetoprotein may indicate a residual tumour, recurrence or malignant transformation.⁷
Other forms of prenatal diagnostic modalities include fetal magnetic resonance imaging (MRI), fetal echocardiography and doppler echocardiography. Fetal MRI is observed to give a clearer configuration of the tumour than USS.

In the developing countries these modalities are not readily available in most hospitals. In the few hospital where these investigations are available, they not affordable. This makes USS the most readily available investigative tool for evaluation and monitoring.