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Fetal sacrococcygeal teratoma
Journal of Pediatric Surgery VOL XXI, NO 7
JULY 1986
Fetal Sacrococcygeal
Teratoma
By Alan W. Flake, Michael R. Harrison, N. Scott Adzick, Jean-Martin Laberge, and Steven L. Warsof,
San Francisco, Cafifornia and Norfolk, Virginia 9 Sacrococcygeal terstoma (SCT) is being diagnosed before birth with increasing frequency. W e were recently consulted about management of e 2 2 - w e e k fetus with SCT and reviewed our experience (6 cases) and the literature. W e found that (1) most fetal SCT present from 22 to 34 weeks gestation with a uterus enlarged by the tumor and/or associat,'d polyhydramnios; (2) although the American Academy of Pediatrics Surgical Section clinical classification is an important prognostic indicator in neonatal SCT, it does not appear to predict outcome in fetal SCT; (3) associated chromosomal abnormalities or life threatening anomalies are rare; (4) presentation after 3 0 - w e e k s gestation is a relatively good prognostic sign with fetal survival, after planned cesarean delivery, in 6 of 8 cases; and (5) hydrops and/or placentomagaly in association with fetal SCT predicts fetal demise soon after diagnosis with 7 of 7 cases dying in utero. 9 1986
by
Grune & Stratton, Inc.
heart size was noted with further worsening of the polyhydramnios. One week later, the patient was admitted with mild preeclampsia. U / S demonstrated fetal parameters consistent with 28 weeks gestational age and a t2 • 15 cm SCT. Fetal cardiac rate and motion were normal. In spite of conservative medical therapy she progressed to severe preeclampsia over the next 24 hours. Under general anesthesia, a classical cesarean incision was performed to minimize fetal trauma during delivery. Fetal heart tones were monitored until the patient was taken to the operating room, 15 minutes prior to delivery, and were normal. A 2,650 g stillborn female was delivered (Fig 2). Autopsy findings included a 19 x 10 • 7 cm benign S C T weighing 730 g with large multifocal areas of internal hemorrhage. There was no intrapelvic extension of the tumor, cord displacement, or other abnormality.
DISCUSSION
CASE REPORT
Neonatal SCT is a well-defined entity. Children born with AAPSS Type 1 SCT (ie, primarily extrapelvic) have an excellent prognosis with early surgical treatment. 4'5 Despite the large size of most tumors, malignancy is rare. The natural history of fetal SCT is not so well defined and needs further clarification. There are 27 reported cases of prenatally diagnosed SCT (Table 1) including our case report and a recent series of six cases from our institution. Analysis of the natural history is obscured by elective termination in five cases 2'6 8 and failure to report meaningful associated findings and autopsy results in many others. By eliminating uninformative cases, the following conclusions can be drawn from available experience: (1) the vast majority of cases present from 22 to 34 weeks gestation with a uterus large for gestational dates or
A 20-year-old G: P~ Ab0 white female was referred for ultrasound ( U / S ) because her uterus was large for gestational dates (Fig 1). U / S revealed a female fetus with a biparietal diameter of 55 m m consistent with a gestational age of 22 weeks. A 10 x 5 x 7 cm predominently solid mass extended from the sacrococcygeal region of the fetus. No intrapelvic extension of the tumor was noted and fetal bladder emptying and lower extremity motor function appeared normal (American Academy of Pediatrics Surgical Section [AAPSSI classification S C T Type 1). 3 Mild polyhydramnios was present. Repeat U / S 2 weeks later revealed growth of the tumor to 14 • 10 • 7 cm with associated cystic degeneration. The polyhydramnios had increased. The pregnancy progressed uneventfully and a third U / S 2 weeks later revealed further growth of the tumor to 14 • 12 x 10 cm with marked cystic degeneration. Prominent fetal
From the Department of Surgery, The Fetal Treatment Program and Division of Pediatric Surgery, University of California, San Francisco and the Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Eastern Virginia Medical School, Norfolk, Va. Presented at the 34th Annual Meeting of the Surgical Section of the American Academy of Pediatrics, San Antonio, Texas, October 19 20, 1985. Address reprint requests to Michael R. Harrison, MD, University of CaliJbrnia, Rm 585 HSE, San Francisco, CA 94143. ~ t986 by Grune. & Stratton, Inc. 0022 3468/86/2107 0001503.00/0
INDEX WORDS: Sacrococcygeal teratoma; fetal surgery.
A C R O C O C C Y G E A L teratoma (SCT) is the most common tumor of the newborn with a reported incidence of l in 35,000 live births. ~ Most of these cases are asymptomatic during pregnancy and diagnosed at birth. However, with the improving accuracy of ultrasonography, there have been increasingly frequent reports of the antenatal diagnosis of SCT in the literature. We have recently reported six cases. 2 The following additional case proved particularly informative and raised some interesting questions about management.
S
JoumalofPediatnc Surgery, Vol 21, No 7 (July), 1986: pp 563-566
563
564
FLAKE ET AL
1 GEST. AGE:
22wks
TUMORSIZE: 1 0 x 5 x 7 c m ASSOCIATED FINDINGS:
Mild hydramnio$
24 wks
26 wks
28 wks
14x10x7cm
1 4 x 1 2 x 10cm
19x 1 0 x 7 c m
t Hydramnio$
IHydramnios Marked cystic degeneration of tumor
Massive hydramnios Eclampsio Pre-term labor Caesarean section
with symptoms of acute polyhydramnios; (2) presentation after 30 weeks gestation is a relatively good prognostic sign with fetal survival in 6 of 8 reported cases compared with survival in l of 14 cases presenting prior to 30 weeks; (3) although AAPSS classifica-
Fig 2. Stillborn infant showing massive tumor size and areas of internal hemorrhage.
Fig 1. Progression of tumor size and clinical findings with gestational age.
tion is an important prognostic indicator in neonatal SCT, it does not appear to predict fetal survival; (4) the presence of placentomegaly and/or hydrops in association with fetal SCT predicts fetal demise soon after diagnosis with 7 of 7 reported cases dying in utero; (5) the occurrence of chromosomal abnormalities or associated life threatening congenital anomalies is rare. SCT caused death in most fetuses but not by the mechanism usually responsible for death in neonatal SCT, ie, malignant invasion. Instead, death appears to result from the secondary effects of the SCT. Tumor mass and associated polyhydramnios frequently cause preterm labor and delivery with fetal survival dependent upon fetal lung maturity. Massive hemorrhage into the tumor with secondary fetal exsanguination may occur spontaneously in utero or be precipitated by labor and delivery. Dystocia, secondary to tumor bulk, or tumor rupture may occur during vaginal delivery or cesarean section. Finally, the presence of placentomegaly and hydrops in some cases suggests associated high output failure. Postulated mechanisms include anemia, from hemorrhage into the tumor, or a steal phenomenon secondary to either the high flow requirements of the tumor or arteriovenous fistulization.9'~~ These mechanisms combined to result in fetal death in 15 of 22 reported cases. Appropriate treatment for fetal SCT has not been defined. Certainly, cases diagnosed after 30 weeks should be delivered as soon as fetal lung maturity is deemed adequate for neonatal survival. Cesarean section is indicated to avoid dystocia, tumor rupture, or fetal exsanguination from hemorrhage into the tumor. Presentation prior to 30 weeks is more complicated with a high likelihood of fetal death prior to adequate maturation. More information regarding the natural history of fetal SCT diagnosed prior to 30 weeks
FETAL SACROCOCCYGEAL T E R A T O M A
565
Table Gestational Age at Diagnosis 25 26 16 19 22 20 29 31 33 25 28 22 25 Unknown 24 33 31 24 28 34 31 19 34 24 30 28 22
Indication for Sonography Size > dates Size > dates Spotting R o u t i n eUS Routine US Size > dates Size > dates Size > dates Size > dates Hydramnios Hydramnios Pre-eclampsia Hyd~arnnios Rapidgrowth Hydramnios Hydrarnnios Hydramnios Hydramnios Size > dates Hydramnios Hydramnios Inc AFP Size > dates Hydramnios Size > dates Hydramnios Size > dates
Hydramnios Yes Yes NC Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes NC Yes Yes Yes Yes NC Yes Yes NC No Yes NC Yes Yes
1. Clinical, P a t h o l o g i c ,
Placentomegaly Yes Yes No No Yes No NC Yes No Yes Yes Yes Yes NC Yes NC NC NC NC NC No NC No NC NC Yes No
Hydrops Yes Yes No No No No No No No Yes Yes Yes Yes NC Yes NC No NC NC No No Yes No NC No Yes No
and Sonographic
AAPSS Type NC II I I Ill III
NC NC I III I NC NC II I NC I I I III I
Findings
in F e t a l S C T
Clinical Course Termination Termination Termination Termination Termination Preterrn labor, C/S, 33 wk Preterm labor after death Elective C/S, 32 wk Pre-eclampsia, C/S, 34 wk Eclampsie and stillbirth Induced abortion after death Induced abortion after death Induced abortion after death NC eclarnpsia, rupture at delivery Preterrn labor, stillbirth Eclampsia, C/S, surge~ Preterm labor, C/S, 32 wk Preterm labor, C/S, stillbirth Preterrn labor, vaginal delivery Elective C/S, 34 wk Preterm labor, vaginal delivery, rupture Induced abortion after death, 24 wk Aspiration, C/S, surgery Preterm labor and delivery Preterm labor, C/S, 32 wk C/S Preterm labor, C/S, 28 wk
Fetal Outcome Death Death Death Death Death Lumbosacral plexopathy Died in utero Died after birth Alive Died in utero Died in utero Died in utero Died in utero Died at birth Died in utero Alive Alive Died in utero Diet at birth Alive Diedat birth Died in utero Alive Died in utero Alive Died at birth Died at birth
Reference 6 7 8 2 2 2 2 2 2 9 10 10 7 14 15 16 17 18 19 20 21 22 23 24 25 26 Current report
Abbreviations: NC, no comment by authors; C/S, cesarean section; Inc AFP, increased alpha-feto-protein.
without associated placentomegaly or hydrops is required to make recommendations. The development of placentomegaly or hydrops appears to be a preterminal event, which immediately preceeds fetal death. Potentially life saving prenatal interventions include intrauterine transfusion or fetal surgery. The following are prerequisites for consideration of fetal intervention~: (1) accurate prenatal diagnosis and welldefined natural history, ie, confidence that this is a correctable lesion that will prevent fetal/neonatal survival; (2) absence of other debilitating or life threatening anomalies; and (3) the ability to perform the procedure without undue risk to the mother's life or future fertility. ~2']3 The diagnosis of AAPSS Type 1 SCT prior to 28 weeks with associated hydrops and/or
placentomegaly appears to satisfy the first two requirements. The physiologic rationale for ligating an arteriovenous fistula or removing the cause of a circulatory steal is sound. However, the feasibility and safety of debulking or iigating a vascular tumor mass in utero is not known. In addition, the pathophysioiogy of high output failure in fetal SCT needs further clarification to permit rational prenatal intervention. Appropriate therapy for fetal SCT can only be defined through a better understanding of its natural history. Experience with prenatal diagnosis of this rare tumor should be shared so that relevant data may be accumulated and optimal management strategies devised.
REFERENCES 1. Pantoja E, Llobet R, Gonzales-Flores B: Retroperitoneal teratoma: Historical review. J Urol 115:520- 523, 1976 2. Holzgreve W, Mahoney BS, Glick PS: Fetal sacrococcygeal teratoma. Prenat Diagn 5:245-257, 1985 3. Airman RP, Randolph JG, Lilly JR: Sacrococcygeal teratoma. J Pediatr Surg 9:389-398, 1974 4. Ein SH, Adeyemi SD, Mancer K: Benign sacrococcygeal teratomas in infants and children. A 25 year review. Ann Surg 191:382-84, 1980 5. Valdiserri RO, Yunis EJ: Sacrococcygeal teratomas: A review of 68 cases. Cancer 48:217-222, 1981 6. Feige A, Gille J, Maillot KV, et al: Praenatale Diagnostik eines Steissbeinteratoms mit Hypertrophie der Placenta. Geburtsh Frauenheilk 42:20-24, 1982 7. Sand H, Bock JE: Prenatal diagnosis of soft tissue malforma-
tions by ultrasound and X-ray. Acta Obstet Gynecol Scand 55:191199, 1976 8. Seeds JW, Mittelstaedt CA, Cefalo RC, et al: Prenatal diagnosis of sacrococcygeal teratoma: An anechoic caudal mass. Clin Ultrasound 10:193-195, 1982 9. Cousins L, Benirschke K, Porreco R, et al: Placetomegaly due to fetal congestive failure in a pregnancy with a sacrococcygeal teratoma. J Reprod Med 25:142-144, 1980 10. Kohga S, Nambu T, Tanaka K: Hypertrophy of the placenta and sacrococcygeal teratoma: A report of two cases. Virchows Arch [A] 386:223-229, 1980 11. Harrison MR, Golbus MS, Filly RA, et al: Fetal surgical treatment. Ped Ann 11:896 903, 1982 12. Harrison MR, Anderson J, Rosen MA, et al: Fetal surgery in
566
the primate I. Anesthetic, surgical, and tocolytic management to maximize fetal-neonatal survival. J Pediatr Surg 17:115-121, 1982 13. Harrison MR, Golbus MS, Filly RA, et al: Management of the fetus with congenital hydronephrosis..I Pediatr Surg 17:728742, 1982 14. Santos-Ramos R, Duenhoelter JH: Diagnosis of congenital fetal abnormalities by sonography. Obstet Gynecol 45:279-283, 1975 15. Gergely RZ, Eden R, Schifrin BS, et al: Antenatal diagnosis of congenital sacral teratoma. J Reprod Med 24:229-231, 1975 16. Dube S, Legros G, Rosenfeld R, et al: Sacrococcygeal tumour in infancy. Can J Surg 23:363-364, 1980 17. Horger EO, McCarter LM: Prenatal diagnosis of sacrococcygeal teratoma. Am J Obstet Gynecol 134:228-229, 1979 18. Verma U, Weiss RR, Almonte R, et al: Early prenatal diagnosis of soft tissue malformation. Obstet Gynecol 53:660-663, 1979 19. Zaleski AM, Cooperberg PL, Kliman MR: Ultrasonic diagnosis of extrafetal masses. J Can Assoc Radiol 30:55-56, 1982
FLAKE ET AL
20. Rayburn WF, Barr M: Teratomas: Concordance in mother and fetus. Am J Obstet Gynecol 144:110-112, 1982 21. Lees RF, Williamson BR, Brenbridge NA: Sonography of benign sacrococcygeal teratoma in utero. Radiology 134:717-718, 1980 22. Brock DJH, Richmond DH, Liston WA: Normal second trimester amniotic fluid alphafetoprotein and acetylcholinesterase associated with fetal sacrococcygeal teratoma. Prenat Diag 3:343345, 1983 23. Mintz MC, Mennuti M, Fishman M: Prenatal aspiration of sacrococcygeal teratoma. Am J Radiol 141:367-368, 1983 24. Hecht F, Kaiser-Hecht B: Sacrococcygeal teratoma: Prenatal diagnosis with elevated alphafetoprotein and acetylcholinesterase in amniotic fluid. Prenat Diag 2:229-231, 1982 25. Bendel RP, Alexander GL Jr: Prenatal diagnosis of sacrococcygeal teratoma by ultrasound. Minn Med 62:623-624, 1980 26. Moerman P, Fryns J-P, Goddeeris P, et al: Nonimmunologic hydrops fetalis. Arch Pathol Lab Med 106:635~40, 1982
JULY 1986
Fetal Sacrococcygeal
Teratoma
By Alan W. Flake, Michael R. Harrison, N. Scott Adzick, Jean-Martin Laberge, and Steven L. Warsof,
San Francisco, Cafifornia and Norfolk, Virginia 9 Sacrococcygeal terstoma (SCT) is being diagnosed before birth with increasing frequency. W e were recently consulted about management of e 2 2 - w e e k fetus with SCT and reviewed our experience (6 cases) and the literature. W e found that (1) most fetal SCT present from 22 to 34 weeks gestation with a uterus enlarged by the tumor and/or associat,'d polyhydramnios; (2) although the American Academy of Pediatrics Surgical Section clinical classification is an important prognostic indicator in neonatal SCT, it does not appear to predict outcome in fetal SCT; (3) associated chromosomal abnormalities or life threatening anomalies are rare; (4) presentation after 3 0 - w e e k s gestation is a relatively good prognostic sign with fetal survival, after planned cesarean delivery, in 6 of 8 cases; and (5) hydrops and/or placentomagaly in association with fetal SCT predicts fetal demise soon after diagnosis with 7 of 7 cases dying in utero. 9 1986
by
Grune & Stratton, Inc.
heart size was noted with further worsening of the polyhydramnios. One week later, the patient was admitted with mild preeclampsia. U / S demonstrated fetal parameters consistent with 28 weeks gestational age and a t2 • 15 cm SCT. Fetal cardiac rate and motion were normal. In spite of conservative medical therapy she progressed to severe preeclampsia over the next 24 hours. Under general anesthesia, a classical cesarean incision was performed to minimize fetal trauma during delivery. Fetal heart tones were monitored until the patient was taken to the operating room, 15 minutes prior to delivery, and were normal. A 2,650 g stillborn female was delivered (Fig 2). Autopsy findings included a 19 x 10 • 7 cm benign S C T weighing 730 g with large multifocal areas of internal hemorrhage. There was no intrapelvic extension of the tumor, cord displacement, or other abnormality.
DISCUSSION
CASE REPORT
Neonatal SCT is a well-defined entity. Children born with AAPSS Type 1 SCT (ie, primarily extrapelvic) have an excellent prognosis with early surgical treatment. 4'5 Despite the large size of most tumors, malignancy is rare. The natural history of fetal SCT is not so well defined and needs further clarification. There are 27 reported cases of prenatally diagnosed SCT (Table 1) including our case report and a recent series of six cases from our institution. Analysis of the natural history is obscured by elective termination in five cases 2'6 8 and failure to report meaningful associated findings and autopsy results in many others. By eliminating uninformative cases, the following conclusions can be drawn from available experience: (1) the vast majority of cases present from 22 to 34 weeks gestation with a uterus large for gestational dates or
A 20-year-old G: P~ Ab0 white female was referred for ultrasound ( U / S ) because her uterus was large for gestational dates (Fig 1). U / S revealed a female fetus with a biparietal diameter of 55 m m consistent with a gestational age of 22 weeks. A 10 x 5 x 7 cm predominently solid mass extended from the sacrococcygeal region of the fetus. No intrapelvic extension of the tumor was noted and fetal bladder emptying and lower extremity motor function appeared normal (American Academy of Pediatrics Surgical Section [AAPSSI classification S C T Type 1). 3 Mild polyhydramnios was present. Repeat U / S 2 weeks later revealed growth of the tumor to 14 • 10 • 7 cm with associated cystic degeneration. The polyhydramnios had increased. The pregnancy progressed uneventfully and a third U / S 2 weeks later revealed further growth of the tumor to 14 • 12 x 10 cm with marked cystic degeneration. Prominent fetal
From the Department of Surgery, The Fetal Treatment Program and Division of Pediatric Surgery, University of California, San Francisco and the Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Eastern Virginia Medical School, Norfolk, Va. Presented at the 34th Annual Meeting of the Surgical Section of the American Academy of Pediatrics, San Antonio, Texas, October 19 20, 1985. Address reprint requests to Michael R. Harrison, MD, University of CaliJbrnia, Rm 585 HSE, San Francisco, CA 94143. ~ t986 by Grune. & Stratton, Inc. 0022 3468/86/2107 0001503.00/0
INDEX WORDS: Sacrococcygeal teratoma; fetal surgery.
A C R O C O C C Y G E A L teratoma (SCT) is the most common tumor of the newborn with a reported incidence of l in 35,000 live births. ~ Most of these cases are asymptomatic during pregnancy and diagnosed at birth. However, with the improving accuracy of ultrasonography, there have been increasingly frequent reports of the antenatal diagnosis of SCT in the literature. We have recently reported six cases. 2 The following additional case proved particularly informative and raised some interesting questions about management.
S
JoumalofPediatnc Surgery, Vol 21, No 7 (July), 1986: pp 563-566
563
564
FLAKE ET AL
1 GEST. AGE:
22wks
TUMORSIZE: 1 0 x 5 x 7 c m ASSOCIATED FINDINGS:
Mild hydramnio$
24 wks
26 wks
28 wks
14x10x7cm
1 4 x 1 2 x 10cm
19x 1 0 x 7 c m
t Hydramnio$
IHydramnios Marked cystic degeneration of tumor
Massive hydramnios Eclampsio Pre-term labor Caesarean section
with symptoms of acute polyhydramnios; (2) presentation after 30 weeks gestation is a relatively good prognostic sign with fetal survival in 6 of 8 reported cases compared with survival in l of 14 cases presenting prior to 30 weeks; (3) although AAPSS classifica-
Fig 2. Stillborn infant showing massive tumor size and areas of internal hemorrhage.
Fig 1. Progression of tumor size and clinical findings with gestational age.
tion is an important prognostic indicator in neonatal SCT, it does not appear to predict fetal survival; (4) the presence of placentomegaly and/or hydrops in association with fetal SCT predicts fetal demise soon after diagnosis with 7 of 7 reported cases dying in utero; (5) the occurrence of chromosomal abnormalities or associated life threatening congenital anomalies is rare. SCT caused death in most fetuses but not by the mechanism usually responsible for death in neonatal SCT, ie, malignant invasion. Instead, death appears to result from the secondary effects of the SCT. Tumor mass and associated polyhydramnios frequently cause preterm labor and delivery with fetal survival dependent upon fetal lung maturity. Massive hemorrhage into the tumor with secondary fetal exsanguination may occur spontaneously in utero or be precipitated by labor and delivery. Dystocia, secondary to tumor bulk, or tumor rupture may occur during vaginal delivery or cesarean section. Finally, the presence of placentomegaly and hydrops in some cases suggests associated high output failure. Postulated mechanisms include anemia, from hemorrhage into the tumor, or a steal phenomenon secondary to either the high flow requirements of the tumor or arteriovenous fistulization.9'~~ These mechanisms combined to result in fetal death in 15 of 22 reported cases. Appropriate treatment for fetal SCT has not been defined. Certainly, cases diagnosed after 30 weeks should be delivered as soon as fetal lung maturity is deemed adequate for neonatal survival. Cesarean section is indicated to avoid dystocia, tumor rupture, or fetal exsanguination from hemorrhage into the tumor. Presentation prior to 30 weeks is more complicated with a high likelihood of fetal death prior to adequate maturation. More information regarding the natural history of fetal SCT diagnosed prior to 30 weeks
FETAL SACROCOCCYGEAL T E R A T O M A
565
Table Gestational Age at Diagnosis 25 26 16 19 22 20 29 31 33 25 28 22 25 Unknown 24 33 31 24 28 34 31 19 34 24 30 28 22
Indication for Sonography Size > dates Size > dates Spotting R o u t i n eUS Routine US Size > dates Size > dates Size > dates Size > dates Hydramnios Hydramnios Pre-eclampsia Hyd~arnnios Rapidgrowth Hydramnios Hydrarnnios Hydramnios Hydramnios Size > dates Hydramnios Hydramnios Inc AFP Size > dates Hydramnios Size > dates Hydramnios Size > dates
Hydramnios Yes Yes NC Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes NC Yes Yes Yes Yes NC Yes Yes NC No Yes NC Yes Yes
1. Clinical, P a t h o l o g i c ,
Placentomegaly Yes Yes No No Yes No NC Yes No Yes Yes Yes Yes NC Yes NC NC NC NC NC No NC No NC NC Yes No
Hydrops Yes Yes No No No No No No No Yes Yes Yes Yes NC Yes NC No NC NC No No Yes No NC No Yes No
and Sonographic
AAPSS Type NC II I I Ill III
NC NC I III I NC NC II I NC I I I III I
Findings
in F e t a l S C T
Clinical Course Termination Termination Termination Termination Termination Preterrn labor, C/S, 33 wk Preterm labor after death Elective C/S, 32 wk Pre-eclampsia, C/S, 34 wk Eclampsie and stillbirth Induced abortion after death Induced abortion after death Induced abortion after death NC eclarnpsia, rupture at delivery Preterrn labor, stillbirth Eclampsia, C/S, surge~ Preterm labor, C/S, 32 wk Preterm labor, C/S, stillbirth Preterrn labor, vaginal delivery Elective C/S, 34 wk Preterm labor, vaginal delivery, rupture Induced abortion after death, 24 wk Aspiration, C/S, surgery Preterm labor and delivery Preterm labor, C/S, 32 wk C/S Preterm labor, C/S, 28 wk
Fetal Outcome Death Death Death Death Death Lumbosacral plexopathy Died in utero Died after birth Alive Died in utero Died in utero Died in utero Died in utero Died at birth Died in utero Alive Alive Died in utero Diet at birth Alive Diedat birth Died in utero Alive Died in utero Alive Died at birth Died at birth
Reference 6 7 8 2 2 2 2 2 2 9 10 10 7 14 15 16 17 18 19 20 21 22 23 24 25 26 Current report
Abbreviations: NC, no comment by authors; C/S, cesarean section; Inc AFP, increased alpha-feto-protein.
without associated placentomegaly or hydrops is required to make recommendations. The development of placentomegaly or hydrops appears to be a preterminal event, which immediately preceeds fetal death. Potentially life saving prenatal interventions include intrauterine transfusion or fetal surgery. The following are prerequisites for consideration of fetal intervention~: (1) accurate prenatal diagnosis and welldefined natural history, ie, confidence that this is a correctable lesion that will prevent fetal/neonatal survival; (2) absence of other debilitating or life threatening anomalies; and (3) the ability to perform the procedure without undue risk to the mother's life or future fertility. ~2']3 The diagnosis of AAPSS Type 1 SCT prior to 28 weeks with associated hydrops and/or
placentomegaly appears to satisfy the first two requirements. The physiologic rationale for ligating an arteriovenous fistula or removing the cause of a circulatory steal is sound. However, the feasibility and safety of debulking or iigating a vascular tumor mass in utero is not known. In addition, the pathophysioiogy of high output failure in fetal SCT needs further clarification to permit rational prenatal intervention. Appropriate therapy for fetal SCT can only be defined through a better understanding of its natural history. Experience with prenatal diagnosis of this rare tumor should be shared so that relevant data may be accumulated and optimal management strategies devised.
REFERENCES 1. Pantoja E, Llobet R, Gonzales-Flores B: Retroperitoneal teratoma: Historical review. J Urol 115:520- 523, 1976 2. Holzgreve W, Mahoney BS, Glick PS: Fetal sacrococcygeal teratoma. Prenat Diagn 5:245-257, 1985 3. Airman RP, Randolph JG, Lilly JR: Sacrococcygeal teratoma. J Pediatr Surg 9:389-398, 1974 4. Ein SH, Adeyemi SD, Mancer K: Benign sacrococcygeal teratomas in infants and children. A 25 year review. Ann Surg 191:382-84, 1980 5. Valdiserri RO, Yunis EJ: Sacrococcygeal teratomas: A review of 68 cases. Cancer 48:217-222, 1981 6. Feige A, Gille J, Maillot KV, et al: Praenatale Diagnostik eines Steissbeinteratoms mit Hypertrophie der Placenta. Geburtsh Frauenheilk 42:20-24, 1982 7. Sand H, Bock JE: Prenatal diagnosis of soft tissue malforma-
tions by ultrasound and X-ray. Acta Obstet Gynecol Scand 55:191199, 1976 8. Seeds JW, Mittelstaedt CA, Cefalo RC, et al: Prenatal diagnosis of sacrococcygeal teratoma: An anechoic caudal mass. Clin Ultrasound 10:193-195, 1982 9. Cousins L, Benirschke K, Porreco R, et al: Placetomegaly due to fetal congestive failure in a pregnancy with a sacrococcygeal teratoma. J Reprod Med 25:142-144, 1980 10. Kohga S, Nambu T, Tanaka K: Hypertrophy of the placenta and sacrococcygeal teratoma: A report of two cases. Virchows Arch [A] 386:223-229, 1980 11. Harrison MR, Golbus MS, Filly RA, et al: Fetal surgical treatment. Ped Ann 11:896 903, 1982 12. Harrison MR, Anderson J, Rosen MA, et al: Fetal surgery in
566
the primate I. Anesthetic, surgical, and tocolytic management to maximize fetal-neonatal survival. J Pediatr Surg 17:115-121, 1982 13. Harrison MR, Golbus MS, Filly RA, et al: Management of the fetus with congenital hydronephrosis..I Pediatr Surg 17:728742, 1982 14. Santos-Ramos R, Duenhoelter JH: Diagnosis of congenital fetal abnormalities by sonography. Obstet Gynecol 45:279-283, 1975 15. Gergely RZ, Eden R, Schifrin BS, et al: Antenatal diagnosis of congenital sacral teratoma. J Reprod Med 24:229-231, 1975 16. Dube S, Legros G, Rosenfeld R, et al: Sacrococcygeal tumour in infancy. Can J Surg 23:363-364, 1980 17. Horger EO, McCarter LM: Prenatal diagnosis of sacrococcygeal teratoma. Am J Obstet Gynecol 134:228-229, 1979 18. Verma U, Weiss RR, Almonte R, et al: Early prenatal diagnosis of soft tissue malformation. Obstet Gynecol 53:660-663, 1979 19. Zaleski AM, Cooperberg PL, Kliman MR: Ultrasonic diagnosis of extrafetal masses. J Can Assoc Radiol 30:55-56, 1982
FLAKE ET AL
20. Rayburn WF, Barr M: Teratomas: Concordance in mother and fetus. Am J Obstet Gynecol 144:110-112, 1982 21. Lees RF, Williamson BR, Brenbridge NA: Sonography of benign sacrococcygeal teratoma in utero. Radiology 134:717-718, 1980 22. Brock DJH, Richmond DH, Liston WA: Normal second trimester amniotic fluid alphafetoprotein and acetylcholinesterase associated with fetal sacrococcygeal teratoma. Prenat Diag 3:343345, 1983 23. Mintz MC, Mennuti M, Fishman M: Prenatal aspiration of sacrococcygeal teratoma. Am J Radiol 141:367-368, 1983 24. Hecht F, Kaiser-Hecht B: Sacrococcygeal teratoma: Prenatal diagnosis with elevated alphafetoprotein and acetylcholinesterase in amniotic fluid. Prenat Diag 2:229-231, 1982 25. Bendel RP, Alexander GL Jr: Prenatal diagnosis of sacrococcygeal teratoma by ultrasound. Minn Med 62:623-624, 1980 26. Moerman P, Fryns J-P, Goddeeris P, et al: Nonimmunologic hydrops fetalis. Arch Pathol Lab Med 106:635~40, 1982