- Email: [email protected]
Sacrococcygeal teratomas in the fetus and newborn
61
Int. J. Gynecol. Obstet., 1990,32: 61-66 International Federation of Gynecology and Obstetrics
Sacrococcygeal
teratomas in the fetus and newborn
R.R. Leon Sosa* and P.J. Moore** Department of Obstetrics and Gynecology, Edendale Hospital, Pietermaritzburg (Republic of South Africa) (Received February 15th, 1989) (Accepted April 12th, 1989)
Abstract
Two cases of fetal sacrococcygeal teratomas, both resulting in obstructed labor and intrauterine death, are presented. A suggested scheme of antenatal and intrapartum management of these rare tumors is recommended. The diagnosis can be made antenatally by ultrasound examination. The mode of delivery depends on the tumor size and the fetal condition. Fetal prognosis is excellent if an atraumatic delivery is followed by appropriate and timely surgery in a tertiary ten ter. Keywords:
Teratoma; Sacrococcygeal teratoma; Tumor-fetal; Pregnancy; Ultrasound diagnosis; Labor dystocia. Introduction
Sacrococcygeal teratomas are rare tumors occurring in one in 40,000 births [18], but are the commonest congenital tumors in newborns [4]. The majority of these tumors are small and of little consequence during pregnancy and labor [ 111, but the importance of an accurate antenatal diagnosis is threefold: *Present address: Department of Obstetrics and Gynecology, University of Cape Town, Groote Schuur Hospital, Observatory 7925, Cape Town, Republic of South Africa. **Present address: Department of Experimental and Clinical Pharmacology, University of the Witwatersrand Medical School, Johannesburg 2193, Republic of South Africa.
(i) when the tumor is large, fetal complications such as hydramnios, preterm labor, non-immune hydrops fetalis, malpresentations and dystocia [3,19,25] are common, and adequate preparation can be made with respect to obstetric management (ii) 10% of these tumors are malignant at the time of delivery [ 131 and (iii) fetal outcome is good with appropriate obstetric and surgical pediatric treatment [6]. Two cases recently managed at Edendale Hospital are presented, the literature reviewed and a scheme of antenatal and labor management suggested. Case reports
Case no I A 20-year-old unbooked patient, gravida 2 para 1, was admitted to Edendale Hospital in active labor at 36 weeks gestation. She had had a previous normal vaginal delivery. On admission the cervix was 6 cm dilated and there was clinical evidence of an intrauterine fetal death. Forty-five minutes later she delivered the head and thorax of a fresh stillbirth, but despite traction and rotation maneuvres the remainder of the fetal body could not be delivered. In the operating theater under general anesthesia, a large cystic mass was palpated arising from the lower fetal spine. A 20guage needle was introduced into the mass and 2300 ml of dark brown fluid was drained. The remainder of the fetus was delivered Case Report
62
Leon Sosa and Moore
easily thereafter (Fig. 1). The puerperium was uneventful. The fetus was a female weighing 3250 g, and the mass was noted to arise from the sacrococcygeal region. Histological examination revealed a benign sacrococcygeal teratoma containing brain, cartilage and glandular elements. Case no. 2 A’ 25year-old patient, gravida 3 para 2, was referred to Edendale Hospital because of polyhydramnios. She had had two normal vaginal deliveries. Her menstrual dates were unknown, but from her only antenatal visit it was noted that the height of the uterine fundus had increased in size from 26 weeks to term over a period of 4 weeks. She had no diabetes, her blood group was 0, Rh-positive, and the VDRL was negative. Labor began on her way to hospital. Mild hypertension and marked polyhydramnios were found, and the cervix was 5 cm dilated with bulging membranes. Ultrasound showed a dead fetus with generalized edema and a
Fig. 1.
Fetus with sacrococcygeal
Int J Gynecol Obstet 32
teratoma: case no. 1.
large solid and cystic mass arising from the fetal buttocks, measuring 18 x 15 cm. A presumptive diagnosis of sacrococcygeal teratoma was made. Her membranes were ruptured and approximately 2 1 of clear amniotic fluid drained. One hour later, the cervix was fully dilated. In the operating theater the head and thorax were delivered easily, but once again, further descent did not occur. A lower segment cesarean section was performed, and the teratoma was amputated. The remainder of the fetus delivered vaginally, and the tumor was delivered through the abdominal incision. Prophylactic antibiotics were given, and the postoperative period was uncomplicated. The female fetus weighed 3420 g, and had features of anemia and hydrops fetalis. The tumor was a large solid fleshy mass with a few small cystic areas, measuring 20 x 16 x 12 cm (Fig. 2). The placenta weighed 1200 g. Histology revealed an immature sacrococcygeal teratoma, with premature ectodermal tissue as a prominent component of the tumor.
Sacrococcygeal teratoma
Fig. 2.
Fetus with sacrococcygeal
63
teratoma: case no. 2.
Discussion The management of sacrococcygeal teratomas requires an understanding of the embryology and pathology of these tumors. Teratomas are neoplasms composed of multiple tissues of kinds foreign to their site of origin [5]. They occur more commonly in females (75-90%), as ovarian differentiation occurs later than testicular differentiation, and the totipotent cells of the gonadal ridge have a longer time in which to escape from hormonal control and produce teratomas [18]. A positive family history of twinning is common (35-SO%), suggesting that the tumors are one end of a spectrum that stretches from teratomas through to the neonate with a double pelvis, fetus-in-fetu, conjoined twins and a normal set of twins [18]. They probably originate from a misplaced germ cell which failed to undergo meiosis and proceeded directly to mitosis [21]. Usually there are tissues from the three germinal layers, neuroectodermal elements being the most
common [20]. When immature elements are present the prognosis is poor and GonzalezCrussi et al. have proposed a classification for extragonadal teratomas based on similar methods used for the grading of ovarian teratomas [12]. Altman et al. have suggested an anatomical classification according to tumor location [3]. Malignant transformation occurs in 10% of cases when diagnosed prior to 2 months of extrauterine life but this rises to 6%90% when surgical excision is delayed beyond 4 months of age [2]. Malignant tumors are generally endodermal or yolk sac tumors [ 121. Malignancy is more common in males, in cases of solid tumors, and then there is no calcification, but interestingly there is no direct relationship to size of the tumor [l&29]. Prenatal diagnosis is usually made by ultrasound examination of the fetus 16,131, but other less sensitive methods include abdominal X-ray [17,20,29], amniography [26], and alpha-fetoprotein levels [ 131. The most comCase Report
64
Leon Sosa and Moore
mon indication for performing the ultrasound examination is the large-for-dates patient [13]. The usual finding is a solid and cystic mass attached to the lower pole of the fetus but, because the diversity of tissues present, the image is variable. The solid components are often not homogeneous in density and may contain calcifications while cysts are often irregular with angulated borders. In only 15% of cases is the tumor predominantly cystic [6,11,13,15,20,24,27,28,31,33]. The principles of management during the antenatal period include accurate diagnosis, strict follow-up and delivery in a tertiary center where facilities for immediate neonatal surgery exist. The critical questions are when and how to deliver the fetus [a]. Fetal maturity should be documented before delivery, unless a serious complication arises. Early delivery is indicated when there is (i) rapid growth of the tumor and obstruction of the gastrointestinal or urinary tract, both suggestive of malignancy and/or (ii) worsening of the hydramnios and rapid development of hydrops fetalis, which indicates fetal congestive cardiac failure due to the vascularity of the tumor which behaves as an arteriovenous malformation [7,19]. The possible methods of delivery suggested in the literature include vaginal delivery [20], elective cesarean section [6], cesarean section after partial delivery [25], vaginal or abdominal aspiration of cystic lesions followed by vaginal or abdominal delivery [24,34] or destructive procedures [ 11. Vaginal delivery is recommended only for small tumors (less than 5 cm in diameter) [13], with the possible exception of a large cystic teratoma where aspiration under ultrasound guidance can be attempted just prior to delivery [24]. With larger tumors, elective cesarean section is the procedure of choice. Destructive procedures should be performed only in exceptional circumstances. Unfortunately, the tumor is occasionally diagnosed only when dystocia supervenes [1,8,9,22,23,25,30,32,34]. This serious comInt J Gynecol Obstet 32
plication has been estimated to occur in 613% of cases and it is usually associated with tumors larger than 10 cm. The large tumors prevent descent of the fetus into or through the pelvis resulting in fetal death from birth trauma or rupture of the tumor with resultant bleeding [ 1,22,34]. However, pregnancy and labor are often uncomplicated and the tumor is recognized only at birth or soon afterwards in 90% of cases [lo]. These are small teratomas that slip through the pelvis during labor behind the fetus which usually presents cephalically. When the obstetrician is confronted with a sudden stop in the second stage of labor after delivery of the fetal head and thorax, the management should be decided according to the condition of the partially delivered infant and the consistency of the teratoma (Fig. 3). If the partially delivered fetus is alive and gentle traction and fundal pressure are of no avail, the infant should be intubated and ventilated, while the patient is immediately prepared for a laparotomy. In theater, if ultrasound or examination under anesthesia reveals a solid mass, an assistant can gently direct the head from below and the infant delivered abdominally in a retrograde manner [25]. Amputation of a solid tumor, on account of its enormous vascularity, is not recommended. With cystic tumors, aspiration can be attempted under ultrasound guidance, and the fetus is then delivered vaginally if the procedure has been successful [24]. If the partially delivered fetus is dead, the procedure is similar, but amputation of the tumor can be performed at laparotomy, and the infant delivered vaginally. Neonatal treatment consists of immediate surgical resection of the tumor, including the coccyx. Delay allows malignant elements to flourish within the tumor and can lead to obstruction of the urinary and gastrointestinal tracts. The prognosis is excellent if the appropriate treatment is performed soon after birth with a cure rate of more than 90% [3,5,10,14,16,18,30].
sacrococcygeai teratoma 85,84,85
65
Undiagnosed sacrococcygeal teratoma Partially delivered infant: I
I
I
Alive R!suscitation
Of infant fl
Emergezyrasound m
Dead
examination in theater
I
I
Cystic
Cystic
Solid
I
as pirL\
Cesarean
Solid
Vaginal _:_:I;:/ParotoLY
+ Abdominal Delivery of infant delivery of infant Fig. 3.
Management of sacrococcygeal teratomas obstructing labor.
Conclusions
Ultrasound examination is the procedure of choice for the early diagnosis of sacrococcygeal teratomas. This diagnosis should be considered whenever a complex solid and cystic mass arising from the sacral region is identified. Prenatal diagnosis may allow for appropriate preparations for an atraumatic delivery and immediate neonatal surgery. When the diagnosis is made at an obstructed delivery, the prognosis is poor, but delivery with the least trauma to the mother and infant is still possible if active measures are taken. The prognosis for the infant is good provided it survives the perinatal period.
3
4 5
6
7
a 9
10
11
References 12 1 2
Abbott PD. Bowman A, Kantor HI: Dystocia caused by
sacrococcygeal teratoma. Obstet Gynecol27: 571, 1966. Abramovich DR: Amniotic fluid. In: Human Reproductive Physiology (ed RP Shearman), p 156. Blackwell Scientific Publications, Oxford, 1979.
13
Altman RP, Randolph JG, Lilly JR: Sacrococcygeal teratoma. American Academy of Pediatrics Surgical Section Survey 1973. J Pediatr Surg 9: 389,1974. Barson AJ: Congenital neoplasia: the Society’s experience. Arch Dis Child 53: 435, 1978. Carney JA, Thompson F, Johnson CL, Lynn HB: Teratomas in children: clincial and pathologic aspects. J Pediatr Surg 7: 271, 1972. Chervenak FA, Isaacson G, Touloukian R, Tortora M, Berkowitz RL, Hobbins JC: Diagnosis and management of fetal teratomas. Obstet Gynecol66: 666, 1985. Cousins L, Benirschke K, Porreco R, Resnik R: Placentomegaly due to fetal congestive failure in a pregnancy with a sacrococcygeal teratoma. J Reprod Med 25: 142, 1980. Desai V: Dystocia due to fetal sacrococcygeal teratoma. J Obstet Gynaecol Br Commonw 75: 1074, 1968. Edwards WR: A fetal sacrococcygeal tumor obstructing labor after attempted home confinement. Obstet Gynecol 61: 19S, 1983. Ein SH, Adeyemi SD, Mancer K: Benign sacrococcygeal teratomas in infants and children. A 25 year review. Ann Surg 191: 382, 1980. Gergely RZ, Eden R, Schifrin BS, Wade ME: Antenatal diagnosis of congenital sacral teratoma. J Reprod Med 24: 229, 1980. Gonzalez-Crussi F, Winkler RF, Mirkin DL: Sacrococcygeal teratomas in infants and children. Arch Path01 Lab Med 102: 420, 1978. Gross SJ, Benzie RJ, Sermer M, Skidmore MB, Wilson SR: Sacrococcygeal teratoma: Prenatal diagnosis and management. Am J Obstet Gynecol 156: 393, 1987. Case Report
66
Leon Sosa and Moore
Hickey RC, Layton JM: Sacrococcygeal teratoma. Emphasis on the biological history and early therapy. Cancer 7: 1031,1954. 15 Horger III ED, McCarter LM: Prenatal diagnosis of sacrococcygeal teratoma. Am J Obstet Gynecol 134: 228, 1979. 16 Hunt P, Van Leeuwen G, Bingham H, Sights W: Sacrococcygeal teratoma. A report of three cases and survey of present knowledge. Clin Pediatr 7: 165, 1968. 17 Hunt PT, Davidson KC, Ashcraft KW, Holder TM: Radiography of hereditary presacral teratoma. Radiology 122: 187,1977. 18 Izant RJ, Filston HC: Sacrococcygeal teratoma. Analysis of forty-three cases. Am J Surg 130: 617, 1975. 19 Kohler HG: Sacrococcygeal teratoma and non-immunological hydrops fetalis. Br Med J 2: 422,1976. 20 Lees RF, Williamson BRJ, Brenbridge ANAG, Buschi AJ, Teja K: Sonography of benign sacral teratoma in utero. Radiology 134: 717, 1980. 21 Linder D, Hecht F, McCaw BK, Campbell JR: Origin of extragonadal teratomas and endodermal sinus tumors. Nature 254: 597, 1975. 22 Litwiller MR: Dystocia caused by sacrococcygeal teratoma. Two case reports. Obstet Gynecol34: 783,1969. 23 Lu T, Lee KH: Two cases of congenital sacral teratoma obstructing labor. J Obstet Gynaecol Br Commonw 73: 853, 1966. 24 Mintz MC, Menutti M, Fishman M: Prenatal aspiration of sacrococcygeal teratoma. Am J Radio1 141: 367, 1983. 25 Musci MN, Clark MJ, Ayres RE, Finkel MA: Management of dystocia caused by a large sacrococcygeal teratoma. Obstet Gynecol62: lOS, 1983. 26 Queenan JT, Gadow EC: Amniography for detection of congenital malformations. Obstet Gynecol35: 648, 1970. 14
Int J Gynecol Obstet 32
27
28
29
30
31
32 33
34
Sand H, Bock JE: Prenatal diagnosis of soft-tissue malformations by ultrasound and X-ray. Acta Obstet Gyneco1 Stand 55: 191, 1976. Santos-Ramos R, Duenhoelter JH: Diagnosis of congenital fetal abnormalities by sonography. Obstet Gynecol45: 279, 1975. Schey WL, Shkolnik A, White H: Clinical and radiographic considerations of sacrococcygeal teratomas: an analysis of 26 new cases and review of the literature. Radiology 125: 189,1977. Schiffer MA, Greenberg E: Sacrococcygeal teratoma in labor and the newborn. Am J Obstet Gynecol 72: 1054, 1956. Seeds JW, Mittelstaedt CA, Cefalo RC, Parker TF: Prenatal diagnosis of sacrococcygeal teratoma: an anechoid caudal mass. J Clin Ultrasound 10: 193, 1982. Tanaree P: Delivery obstructed by sacrococcygeal teratoma. Am J Obstet Gynecol 142: 239,1982. Verma U, Weiss RR, Almonte R et al: Early prenatal diagnosis of soft tissue malformations. Obstet Gynecol 53: 660, 1979. Weiss DB, Wajntraub G, Abulafia Y, Schiller M: Vaginal surgical intervention for a sacrococcygeal teratoma obstructing labor. Acta Obstet Gynecol Stand 55: 183, 1976.
Address for reprints: R.R. Leon Sosa P.O. Box 427 Gonubie 5256 South Africa
Int. J. Gynecol. Obstet., 1990,32: 61-66 International Federation of Gynecology and Obstetrics
Sacrococcygeal
teratomas in the fetus and newborn
R.R. Leon Sosa* and P.J. Moore** Department of Obstetrics and Gynecology, Edendale Hospital, Pietermaritzburg (Republic of South Africa) (Received February 15th, 1989) (Accepted April 12th, 1989)
Abstract
Two cases of fetal sacrococcygeal teratomas, both resulting in obstructed labor and intrauterine death, are presented. A suggested scheme of antenatal and intrapartum management of these rare tumors is recommended. The diagnosis can be made antenatally by ultrasound examination. The mode of delivery depends on the tumor size and the fetal condition. Fetal prognosis is excellent if an atraumatic delivery is followed by appropriate and timely surgery in a tertiary ten ter. Keywords:
Teratoma; Sacrococcygeal teratoma; Tumor-fetal; Pregnancy; Ultrasound diagnosis; Labor dystocia. Introduction
Sacrococcygeal teratomas are rare tumors occurring in one in 40,000 births [18], but are the commonest congenital tumors in newborns [4]. The majority of these tumors are small and of little consequence during pregnancy and labor [ 111, but the importance of an accurate antenatal diagnosis is threefold: *Present address: Department of Obstetrics and Gynecology, University of Cape Town, Groote Schuur Hospital, Observatory 7925, Cape Town, Republic of South Africa. **Present address: Department of Experimental and Clinical Pharmacology, University of the Witwatersrand Medical School, Johannesburg 2193, Republic of South Africa.
(i) when the tumor is large, fetal complications such as hydramnios, preterm labor, non-immune hydrops fetalis, malpresentations and dystocia [3,19,25] are common, and adequate preparation can be made with respect to obstetric management (ii) 10% of these tumors are malignant at the time of delivery [ 131 and (iii) fetal outcome is good with appropriate obstetric and surgical pediatric treatment [6]. Two cases recently managed at Edendale Hospital are presented, the literature reviewed and a scheme of antenatal and labor management suggested. Case reports
Case no I A 20-year-old unbooked patient, gravida 2 para 1, was admitted to Edendale Hospital in active labor at 36 weeks gestation. She had had a previous normal vaginal delivery. On admission the cervix was 6 cm dilated and there was clinical evidence of an intrauterine fetal death. Forty-five minutes later she delivered the head and thorax of a fresh stillbirth, but despite traction and rotation maneuvres the remainder of the fetal body could not be delivered. In the operating theater under general anesthesia, a large cystic mass was palpated arising from the lower fetal spine. A 20guage needle was introduced into the mass and 2300 ml of dark brown fluid was drained. The remainder of the fetus was delivered Case Report
62
Leon Sosa and Moore
easily thereafter (Fig. 1). The puerperium was uneventful. The fetus was a female weighing 3250 g, and the mass was noted to arise from the sacrococcygeal region. Histological examination revealed a benign sacrococcygeal teratoma containing brain, cartilage and glandular elements. Case no. 2 A’ 25year-old patient, gravida 3 para 2, was referred to Edendale Hospital because of polyhydramnios. She had had two normal vaginal deliveries. Her menstrual dates were unknown, but from her only antenatal visit it was noted that the height of the uterine fundus had increased in size from 26 weeks to term over a period of 4 weeks. She had no diabetes, her blood group was 0, Rh-positive, and the VDRL was negative. Labor began on her way to hospital. Mild hypertension and marked polyhydramnios were found, and the cervix was 5 cm dilated with bulging membranes. Ultrasound showed a dead fetus with generalized edema and a
Fig. 1.
Fetus with sacrococcygeal
Int J Gynecol Obstet 32
teratoma: case no. 1.
large solid and cystic mass arising from the fetal buttocks, measuring 18 x 15 cm. A presumptive diagnosis of sacrococcygeal teratoma was made. Her membranes were ruptured and approximately 2 1 of clear amniotic fluid drained. One hour later, the cervix was fully dilated. In the operating theater the head and thorax were delivered easily, but once again, further descent did not occur. A lower segment cesarean section was performed, and the teratoma was amputated. The remainder of the fetus delivered vaginally, and the tumor was delivered through the abdominal incision. Prophylactic antibiotics were given, and the postoperative period was uncomplicated. The female fetus weighed 3420 g, and had features of anemia and hydrops fetalis. The tumor was a large solid fleshy mass with a few small cystic areas, measuring 20 x 16 x 12 cm (Fig. 2). The placenta weighed 1200 g. Histology revealed an immature sacrococcygeal teratoma, with premature ectodermal tissue as a prominent component of the tumor.
Sacrococcygeal teratoma
Fig. 2.
Fetus with sacrococcygeal
63
teratoma: case no. 2.
Discussion The management of sacrococcygeal teratomas requires an understanding of the embryology and pathology of these tumors. Teratomas are neoplasms composed of multiple tissues of kinds foreign to their site of origin [5]. They occur more commonly in females (75-90%), as ovarian differentiation occurs later than testicular differentiation, and the totipotent cells of the gonadal ridge have a longer time in which to escape from hormonal control and produce teratomas [18]. A positive family history of twinning is common (35-SO%), suggesting that the tumors are one end of a spectrum that stretches from teratomas through to the neonate with a double pelvis, fetus-in-fetu, conjoined twins and a normal set of twins [18]. They probably originate from a misplaced germ cell which failed to undergo meiosis and proceeded directly to mitosis [21]. Usually there are tissues from the three germinal layers, neuroectodermal elements being the most
common [20]. When immature elements are present the prognosis is poor and GonzalezCrussi et al. have proposed a classification for extragonadal teratomas based on similar methods used for the grading of ovarian teratomas [12]. Altman et al. have suggested an anatomical classification according to tumor location [3]. Malignant transformation occurs in 10% of cases when diagnosed prior to 2 months of extrauterine life but this rises to 6%90% when surgical excision is delayed beyond 4 months of age [2]. Malignant tumors are generally endodermal or yolk sac tumors [ 121. Malignancy is more common in males, in cases of solid tumors, and then there is no calcification, but interestingly there is no direct relationship to size of the tumor [l&29]. Prenatal diagnosis is usually made by ultrasound examination of the fetus 16,131, but other less sensitive methods include abdominal X-ray [17,20,29], amniography [26], and alpha-fetoprotein levels [ 131. The most comCase Report
64
Leon Sosa and Moore
mon indication for performing the ultrasound examination is the large-for-dates patient [13]. The usual finding is a solid and cystic mass attached to the lower pole of the fetus but, because the diversity of tissues present, the image is variable. The solid components are often not homogeneous in density and may contain calcifications while cysts are often irregular with angulated borders. In only 15% of cases is the tumor predominantly cystic [6,11,13,15,20,24,27,28,31,33]. The principles of management during the antenatal period include accurate diagnosis, strict follow-up and delivery in a tertiary center where facilities for immediate neonatal surgery exist. The critical questions are when and how to deliver the fetus [a]. Fetal maturity should be documented before delivery, unless a serious complication arises. Early delivery is indicated when there is (i) rapid growth of the tumor and obstruction of the gastrointestinal or urinary tract, both suggestive of malignancy and/or (ii) worsening of the hydramnios and rapid development of hydrops fetalis, which indicates fetal congestive cardiac failure due to the vascularity of the tumor which behaves as an arteriovenous malformation [7,19]. The possible methods of delivery suggested in the literature include vaginal delivery [20], elective cesarean section [6], cesarean section after partial delivery [25], vaginal or abdominal aspiration of cystic lesions followed by vaginal or abdominal delivery [24,34] or destructive procedures [ 11. Vaginal delivery is recommended only for small tumors (less than 5 cm in diameter) [13], with the possible exception of a large cystic teratoma where aspiration under ultrasound guidance can be attempted just prior to delivery [24]. With larger tumors, elective cesarean section is the procedure of choice. Destructive procedures should be performed only in exceptional circumstances. Unfortunately, the tumor is occasionally diagnosed only when dystocia supervenes [1,8,9,22,23,25,30,32,34]. This serious comInt J Gynecol Obstet 32
plication has been estimated to occur in 613% of cases and it is usually associated with tumors larger than 10 cm. The large tumors prevent descent of the fetus into or through the pelvis resulting in fetal death from birth trauma or rupture of the tumor with resultant bleeding [ 1,22,34]. However, pregnancy and labor are often uncomplicated and the tumor is recognized only at birth or soon afterwards in 90% of cases [lo]. These are small teratomas that slip through the pelvis during labor behind the fetus which usually presents cephalically. When the obstetrician is confronted with a sudden stop in the second stage of labor after delivery of the fetal head and thorax, the management should be decided according to the condition of the partially delivered infant and the consistency of the teratoma (Fig. 3). If the partially delivered fetus is alive and gentle traction and fundal pressure are of no avail, the infant should be intubated and ventilated, while the patient is immediately prepared for a laparotomy. In theater, if ultrasound or examination under anesthesia reveals a solid mass, an assistant can gently direct the head from below and the infant delivered abdominally in a retrograde manner [25]. Amputation of a solid tumor, on account of its enormous vascularity, is not recommended. With cystic tumors, aspiration can be attempted under ultrasound guidance, and the fetus is then delivered vaginally if the procedure has been successful [24]. If the partially delivered fetus is dead, the procedure is similar, but amputation of the tumor can be performed at laparotomy, and the infant delivered vaginally. Neonatal treatment consists of immediate surgical resection of the tumor, including the coccyx. Delay allows malignant elements to flourish within the tumor and can lead to obstruction of the urinary and gastrointestinal tracts. The prognosis is excellent if the appropriate treatment is performed soon after birth with a cure rate of more than 90% [3,5,10,14,16,18,30].
sacrococcygeai teratoma 85,84,85
65
Undiagnosed sacrococcygeal teratoma Partially delivered infant: I
I
I
Alive R!suscitation
Of infant fl
Emergezyrasound m
Dead
examination in theater
I
I
Cystic
Cystic
Solid
I
as pirL\
Cesarean
Solid
Vaginal _:_:I;:/ParotoLY
+ Abdominal Delivery of infant delivery of infant Fig. 3.
Management of sacrococcygeal teratomas obstructing labor.
Conclusions
Ultrasound examination is the procedure of choice for the early diagnosis of sacrococcygeal teratomas. This diagnosis should be considered whenever a complex solid and cystic mass arising from the sacral region is identified. Prenatal diagnosis may allow for appropriate preparations for an atraumatic delivery and immediate neonatal surgery. When the diagnosis is made at an obstructed delivery, the prognosis is poor, but delivery with the least trauma to the mother and infant is still possible if active measures are taken. The prognosis for the infant is good provided it survives the perinatal period.
3
4 5
6
7
a 9
10
11
References 12 1 2
Abbott PD. Bowman A, Kantor HI: Dystocia caused by
sacrococcygeal teratoma. Obstet Gynecol27: 571, 1966. Abramovich DR: Amniotic fluid. In: Human Reproductive Physiology (ed RP Shearman), p 156. Blackwell Scientific Publications, Oxford, 1979.
13
Altman RP, Randolph JG, Lilly JR: Sacrococcygeal teratoma. American Academy of Pediatrics Surgical Section Survey 1973. J Pediatr Surg 9: 389,1974. Barson AJ: Congenital neoplasia: the Society’s experience. Arch Dis Child 53: 435, 1978. Carney JA, Thompson F, Johnson CL, Lynn HB: Teratomas in children: clincial and pathologic aspects. J Pediatr Surg 7: 271, 1972. Chervenak FA, Isaacson G, Touloukian R, Tortora M, Berkowitz RL, Hobbins JC: Diagnosis and management of fetal teratomas. Obstet Gynecol66: 666, 1985. Cousins L, Benirschke K, Porreco R, Resnik R: Placentomegaly due to fetal congestive failure in a pregnancy with a sacrococcygeal teratoma. J Reprod Med 25: 142, 1980. Desai V: Dystocia due to fetal sacrococcygeal teratoma. J Obstet Gynaecol Br Commonw 75: 1074, 1968. Edwards WR: A fetal sacrococcygeal tumor obstructing labor after attempted home confinement. Obstet Gynecol 61: 19S, 1983. Ein SH, Adeyemi SD, Mancer K: Benign sacrococcygeal teratomas in infants and children. A 25 year review. Ann Surg 191: 382, 1980. Gergely RZ, Eden R, Schifrin BS, Wade ME: Antenatal diagnosis of congenital sacral teratoma. J Reprod Med 24: 229, 1980. Gonzalez-Crussi F, Winkler RF, Mirkin DL: Sacrococcygeal teratomas in infants and children. Arch Path01 Lab Med 102: 420, 1978. Gross SJ, Benzie RJ, Sermer M, Skidmore MB, Wilson SR: Sacrococcygeal teratoma: Prenatal diagnosis and management. Am J Obstet Gynecol 156: 393, 1987. Case Report
66
Leon Sosa and Moore
Hickey RC, Layton JM: Sacrococcygeal teratoma. Emphasis on the biological history and early therapy. Cancer 7: 1031,1954. 15 Horger III ED, McCarter LM: Prenatal diagnosis of sacrococcygeal teratoma. Am J Obstet Gynecol 134: 228, 1979. 16 Hunt P, Van Leeuwen G, Bingham H, Sights W: Sacrococcygeal teratoma. A report of three cases and survey of present knowledge. Clin Pediatr 7: 165, 1968. 17 Hunt PT, Davidson KC, Ashcraft KW, Holder TM: Radiography of hereditary presacral teratoma. Radiology 122: 187,1977. 18 Izant RJ, Filston HC: Sacrococcygeal teratoma. Analysis of forty-three cases. Am J Surg 130: 617, 1975. 19 Kohler HG: Sacrococcygeal teratoma and non-immunological hydrops fetalis. Br Med J 2: 422,1976. 20 Lees RF, Williamson BRJ, Brenbridge ANAG, Buschi AJ, Teja K: Sonography of benign sacral teratoma in utero. Radiology 134: 717, 1980. 21 Linder D, Hecht F, McCaw BK, Campbell JR: Origin of extragonadal teratomas and endodermal sinus tumors. Nature 254: 597, 1975. 22 Litwiller MR: Dystocia caused by sacrococcygeal teratoma. Two case reports. Obstet Gynecol34: 783,1969. 23 Lu T, Lee KH: Two cases of congenital sacral teratoma obstructing labor. J Obstet Gynaecol Br Commonw 73: 853, 1966. 24 Mintz MC, Menutti M, Fishman M: Prenatal aspiration of sacrococcygeal teratoma. Am J Radio1 141: 367, 1983. 25 Musci MN, Clark MJ, Ayres RE, Finkel MA: Management of dystocia caused by a large sacrococcygeal teratoma. Obstet Gynecol62: lOS, 1983. 26 Queenan JT, Gadow EC: Amniography for detection of congenital malformations. Obstet Gynecol35: 648, 1970. 14
Int J Gynecol Obstet 32
27
28
29
30
31
32 33
34
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