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Prenatal ultrasonographic diagnosis of intracranial teratoma and massive craniomegaly with associated high-output cardiac failure
Volume 168 Number 1. Part 1
Placental interleukin-6
REFERENCES 1. Sehgal PB, Grieninger G, Tosato G. Regulation of the acute phase and immune responses: interleukin-6. Ann NY Acad Sci 1989;557:1-583. 2. Kameda T, Matsuzaki N, Sawai K, et al. Production of interleukin-6 by normal trophoblasts. Placenta 1990; 11:205-13. 3. Taniguchi T, Matsuzaki N, Kameda T, et al. The enhanced production of placental interleukin-l during labor and intrauterine infection. AM J OBSTET GY:-IECOL 1991; 165:131-7. 4. Hollander D. Diagnosis of chorioamnionitis. Clin Obstet Gynecol 1986;29:816-25. 5. Romero R, Avila C, Santhanam U, Sehgal PB. Amniotic fluid interleukin-6 in preterm labor. J Clin Invest 1990;85:1392-1400. 6. Romero R, Brody DT, Wu YK, et al. Infection and labor. III. Interleukin-l: a signal for the initiation of parturition. AM J OBSTET GYNECOL 1989;160: 1117-23.
7. Matsuzaki N, Saji F, Kameda T, et al. In vitro and in vivo production of interleukin-6 by fetal mononuclear cells. Clin Immunol Immunopathol 1990;55:305-14. 8. Gauldie J, Richards C, Harnish D, Lansdorp P, Baumann H. Interferon 132/B-cell stimulatory factor type 2 shares identity with monocyte-derived hepatocyte stimulating factor and regulates the major acute phase response in liver cells. Proc Natl Acad Sci USA 1987;84:7251-5. 9. Okada M, Kitahara M, Kishimoto S, Matsuda T, Hirano T, Kishimoto T. IL-6IBSF-2 functions as a killer helper factor in the in vitro induction of cytotoxic T cells. JImmunoI1988;141:1543-9. 10. Kishimoto T, Hirano T. Molecular regulation of B lymphocyte response. Ann Rev Immunol 1988;6:485-512. 11. Dinarello CA. Biology of interleukin-1. FASEB J 1988;2: 108-15. 12. GershenwaldJE, Fong Y, Fahey III TJ, et al. Interleukin 1 receptor blockade attenuates the host inflammatory response. Proc Natl Acad Sci USA 1990;87:4966-70.
Prenatal ultrasonographic diagnosis of intracranial teratoma and massive craniomegaly with associated high-output cardiac failure David M. Sherer, MD, Jacques S. Abramowicz, MD, Paula C. Eggers, RDMS, Leon A. Metlay, MD, Robert A. Sinkin, MD, and James R. Woods, Jr., MD Rochester, New York Congenital intracranial teratomas are rare and usually fatal. We present prenatal diagnosis of such a case associated with scalp, facial, and body skin edema, hepatomegaly, extramedullary hematopoiesis, polyhydramnios, and a hydropic placenta. These manifestations of high-output cardiac failure were thought to be the result of the large cardiac output required by massive intracranial tumor arteriovenous shunting. (AM J OaSTEr GYNECOL 1993;168:97-9.)
Key words: Ultrasonography, intracranial teratoma, hydrops, high-output cardiac failure Congenital intracranial teratomas are rare and usually fatal. I The ultrasonographic findings associated with these lesions involve complete loss of normal symmetric intracranial structures and the presence of a solid, irregular, midline, echogenic mass with multiple cystic areas peripheral to the tumor. We present a case From the Diviszon of Maternal-Fetal Medicine, Department of Obstetnes and Gynecology, the Department of Pathology, and the Division of Neonatology, Department of Pedzatncs, Strong Memorial HOSPItal, The University of Rochester School of MediCine and Dentistry. ReceIVed for publicatwnjuly 26,1992; accepted july 31,1992. Reprint requests: David M. Sherer, MD, DivlSzon of Maternal-Fetal MedICine, Department of Obstetrics and Gynecology, Strong Memonal Hospital, The UniversIty of Rochester School of MedICine and Dentistry, 601 Elmwood Ave., Box 8668, Rochester, NY 146428668. 6/1/41512 0002-9378/93 $1.00
+ 0.20
of fetal hydrops associated with intracranial teratoma and massive craniomegaly diagnosed at 31 weeks of gestation.
Case report A 27-year-old, Hispanic woman, gravida 3, para 2, was referred to Strong Memorial Hospital with rapid abdominal girth enlargement at 31 weeks' gestation. Her medical history was unremarkable. Her current gestation had been uneventful with a normal maternal serum a-fetoprotein level at 18 weeks. Midtrimester ultrasonography had not been performed. Physical examination on admission revealed a nondistressed, normotensive gravid woman. Fundal height was 45 cm. No edema or proteinuria was noted. Ultrasonographic examination disclosed massive polyhydramnios with a breech presenting fetus. The fetal head was markedly 97
98
Sherer et al. Am
January 1993
J Obstct Gynecol
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.q;~
...
w
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A Fig. 1. A, Midsagittal view offetal vertex. Note solid echogenic mass (curved arrow) and cervical spine (small arrow). B, Transverse view of fetal vertex demonstrating absence of normal symmetric intracranial anatomy. Note solid echogenic core surrounded with peripheral cystic structures.
Fig. 2. Neonate with massive craniomegaly. Head circumference was 58.5 cm. Note facial edema (periorbital, periauricular, and oral orifice).
enlarged with the biparietal diameter measuring approximately 16 cm (mean normal for 31 weeks 7.7 cm). None of the normal intracranial structures were identifiable. A solid irregular, midline, echo genic mass was seen within the fetal head (Fig. 1, A). This mass was surrounded by peripheral, septated, cystic structures (Fig. 1, B). Scalp edema of 1 cm in thickness was noted. The remainder of the fetal anatomy was normal. Maternal TORCH (toxoplasmosis, rubella, cytomegalovirus, and herpes simplex) titers and Venereal Disease Research Laboratory test result were negative. The patient and her husband were informed of the grave fetal prognosis. At 33 weeks, with the onset of orthopnea, tachypnea, and general maternal discomfort as a result of polyhydramnios (without evidence of hypoxia), 4 L of amniotic fluid was removed by amniocentesis. As expected, the fluid reaccumulated within 48 hours. At 34 weeks the patient presented with severe orthopnea. A nons tress test was nonreactive with decreased beatto-beat variability, in spite of fetal movements that were perceived by the mother and documented with realtime ultrasonography. External vibratory acoustic stimulation failed to evoke either fetal movements or fetal heart rate accelerations. An institutional fetal therapy committee, consisting of perinatologists, neonatologists, and a neuroradiologist, decided that given the grave fetal prognosis delivery would be performed for maternal indications, in spite of prematurity. Although transabdominal cephalocentesis was considered, this was not performed because of what were thought to represent noncommunicating cystic structures. With the patient under general anesthesia, the fetus was delivered by breech extraction through a transverse lowsegment incision. The female infant (Fig. 2) weighed 5250 gm and had Apgar scores of 1, 4, and 4 at 1, 5, and 10 minutes, respectively. The neonatal head circumference was 58.5 cm (mean normal for 34 weeks 31
Volume 168 Number I. Part I
cm). Marked scalp edema, periorbital and periauricular edema, edema of the lips, and mild body skin edema were noted (Fig. 2). Neonatal ultrasonography confirmed the prenatal findings. The infant died at 90 minutes of age. The mother's postoperative course was normal. At autopsy, the brain weighed 1390 gm (mean normal for 34 weeks 246 gm). A large, rounded, knobby mass was located centrally in the brain, filling the third ventricle and causing extensive, obstructive hydrocephalus. Results of histopathologic examination were consistent with a mature teratoma with immature components. The most likely site of origin was thought to be pineal or hypothalamic. Hepatomegaly with marked extramedullary hematopoiesis was noted and the placenta was hydropic.
Comment The prenatal ultrasonographic features of intracranial teratoma involve gross distortion of normal cerebral architecture by an echogenic mass with multicystic areas. Polyhydramnios and craniomegaly are often noted. I Obstetric management is usually dictated by the cranial enlargement, which is caused by the tumor itself and as such rarely permits decompressive measures, such as transabdominal or transcervical cephalocente-
Fetal intracranial teratoma and high-output failure
99
sis. Abdominal delivery has been advocated, often with vertical uterine incision. In our case, in spite of massive craniomegaly with a head circumference of 58.5 cm, delivery was achieved through a transverse, lower-segment uterine incision in the presence of of a welldeveloped lower uterine segment. High-output cardiac failure, manifested in this case by scalp, facial, and body edema, hepatomegaly, extramedullary hematopoiesis, polyhydramnios, and a hydropic placenta, was most probably due to the large cardiac output required by tumor arteriovenous shunting in the massive intracranial teratoma. This mechanism has recently been described as the underlying pathophysiologic cause of high-output failure in a case of fetal sacrococcygeal teratoma. 2 REFERENCES
1. Lipman SH, Pretorius DH, Rumack eM, Manco-Johnson ML. Fetal intracranial teratoma: U.S. diagnosis of three cases, and a review of the literature. Radiology 1985;157: 491-4. 2. Bond SJ, Harrison MR, Schmidt KG, et al. Death due to high output cardiac failure in fetal sacrococcygeal teratoma. J Pediatr Surg 1990;25:1287-91.
Placental interleukin-6
REFERENCES 1. Sehgal PB, Grieninger G, Tosato G. Regulation of the acute phase and immune responses: interleukin-6. Ann NY Acad Sci 1989;557:1-583. 2. Kameda T, Matsuzaki N, Sawai K, et al. Production of interleukin-6 by normal trophoblasts. Placenta 1990; 11:205-13. 3. Taniguchi T, Matsuzaki N, Kameda T, et al. The enhanced production of placental interleukin-l during labor and intrauterine infection. AM J OBSTET GY:-IECOL 1991; 165:131-7. 4. Hollander D. Diagnosis of chorioamnionitis. Clin Obstet Gynecol 1986;29:816-25. 5. Romero R, Avila C, Santhanam U, Sehgal PB. Amniotic fluid interleukin-6 in preterm labor. J Clin Invest 1990;85:1392-1400. 6. Romero R, Brody DT, Wu YK, et al. Infection and labor. III. Interleukin-l: a signal for the initiation of parturition. AM J OBSTET GYNECOL 1989;160: 1117-23.
7. Matsuzaki N, Saji F, Kameda T, et al. In vitro and in vivo production of interleukin-6 by fetal mononuclear cells. Clin Immunol Immunopathol 1990;55:305-14. 8. Gauldie J, Richards C, Harnish D, Lansdorp P, Baumann H. Interferon 132/B-cell stimulatory factor type 2 shares identity with monocyte-derived hepatocyte stimulating factor and regulates the major acute phase response in liver cells. Proc Natl Acad Sci USA 1987;84:7251-5. 9. Okada M, Kitahara M, Kishimoto S, Matsuda T, Hirano T, Kishimoto T. IL-6IBSF-2 functions as a killer helper factor in the in vitro induction of cytotoxic T cells. JImmunoI1988;141:1543-9. 10. Kishimoto T, Hirano T. Molecular regulation of B lymphocyte response. Ann Rev Immunol 1988;6:485-512. 11. Dinarello CA. Biology of interleukin-1. FASEB J 1988;2: 108-15. 12. GershenwaldJE, Fong Y, Fahey III TJ, et al. Interleukin 1 receptor blockade attenuates the host inflammatory response. Proc Natl Acad Sci USA 1990;87:4966-70.
Prenatal ultrasonographic diagnosis of intracranial teratoma and massive craniomegaly with associated high-output cardiac failure David M. Sherer, MD, Jacques S. Abramowicz, MD, Paula C. Eggers, RDMS, Leon A. Metlay, MD, Robert A. Sinkin, MD, and James R. Woods, Jr., MD Rochester, New York Congenital intracranial teratomas are rare and usually fatal. We present prenatal diagnosis of such a case associated with scalp, facial, and body skin edema, hepatomegaly, extramedullary hematopoiesis, polyhydramnios, and a hydropic placenta. These manifestations of high-output cardiac failure were thought to be the result of the large cardiac output required by massive intracranial tumor arteriovenous shunting. (AM J OaSTEr GYNECOL 1993;168:97-9.)
Key words: Ultrasonography, intracranial teratoma, hydrops, high-output cardiac failure Congenital intracranial teratomas are rare and usually fatal. I The ultrasonographic findings associated with these lesions involve complete loss of normal symmetric intracranial structures and the presence of a solid, irregular, midline, echogenic mass with multiple cystic areas peripheral to the tumor. We present a case From the Diviszon of Maternal-Fetal Medicine, Department of Obstetnes and Gynecology, the Department of Pathology, and the Division of Neonatology, Department of Pedzatncs, Strong Memorial HOSPItal, The University of Rochester School of MediCine and Dentistry. ReceIVed for publicatwnjuly 26,1992; accepted july 31,1992. Reprint requests: David M. Sherer, MD, DivlSzon of Maternal-Fetal MedICine, Department of Obstetrics and Gynecology, Strong Memonal Hospital, The UniversIty of Rochester School of MedICine and Dentistry, 601 Elmwood Ave., Box 8668, Rochester, NY 146428668. 6/1/41512 0002-9378/93 $1.00
+ 0.20
of fetal hydrops associated with intracranial teratoma and massive craniomegaly diagnosed at 31 weeks of gestation.
Case report A 27-year-old, Hispanic woman, gravida 3, para 2, was referred to Strong Memorial Hospital with rapid abdominal girth enlargement at 31 weeks' gestation. Her medical history was unremarkable. Her current gestation had been uneventful with a normal maternal serum a-fetoprotein level at 18 weeks. Midtrimester ultrasonography had not been performed. Physical examination on admission revealed a nondistressed, normotensive gravid woman. Fundal height was 45 cm. No edema or proteinuria was noted. Ultrasonographic examination disclosed massive polyhydramnios with a breech presenting fetus. The fetal head was markedly 97
98
Sherer et al. Am
January 1993
J Obstct Gynecol
,.
.q;~
...
w
"~t:""',-j. "':: '
"H-"
A Fig. 1. A, Midsagittal view offetal vertex. Note solid echogenic mass (curved arrow) and cervical spine (small arrow). B, Transverse view of fetal vertex demonstrating absence of normal symmetric intracranial anatomy. Note solid echogenic core surrounded with peripheral cystic structures.
Fig. 2. Neonate with massive craniomegaly. Head circumference was 58.5 cm. Note facial edema (periorbital, periauricular, and oral orifice).
enlarged with the biparietal diameter measuring approximately 16 cm (mean normal for 31 weeks 7.7 cm). None of the normal intracranial structures were identifiable. A solid irregular, midline, echo genic mass was seen within the fetal head (Fig. 1, A). This mass was surrounded by peripheral, septated, cystic structures (Fig. 1, B). Scalp edema of 1 cm in thickness was noted. The remainder of the fetal anatomy was normal. Maternal TORCH (toxoplasmosis, rubella, cytomegalovirus, and herpes simplex) titers and Venereal Disease Research Laboratory test result were negative. The patient and her husband were informed of the grave fetal prognosis. At 33 weeks, with the onset of orthopnea, tachypnea, and general maternal discomfort as a result of polyhydramnios (without evidence of hypoxia), 4 L of amniotic fluid was removed by amniocentesis. As expected, the fluid reaccumulated within 48 hours. At 34 weeks the patient presented with severe orthopnea. A nons tress test was nonreactive with decreased beatto-beat variability, in spite of fetal movements that were perceived by the mother and documented with realtime ultrasonography. External vibratory acoustic stimulation failed to evoke either fetal movements or fetal heart rate accelerations. An institutional fetal therapy committee, consisting of perinatologists, neonatologists, and a neuroradiologist, decided that given the grave fetal prognosis delivery would be performed for maternal indications, in spite of prematurity. Although transabdominal cephalocentesis was considered, this was not performed because of what were thought to represent noncommunicating cystic structures. With the patient under general anesthesia, the fetus was delivered by breech extraction through a transverse lowsegment incision. The female infant (Fig. 2) weighed 5250 gm and had Apgar scores of 1, 4, and 4 at 1, 5, and 10 minutes, respectively. The neonatal head circumference was 58.5 cm (mean normal for 34 weeks 31
Volume 168 Number I. Part I
cm). Marked scalp edema, periorbital and periauricular edema, edema of the lips, and mild body skin edema were noted (Fig. 2). Neonatal ultrasonography confirmed the prenatal findings. The infant died at 90 minutes of age. The mother's postoperative course was normal. At autopsy, the brain weighed 1390 gm (mean normal for 34 weeks 246 gm). A large, rounded, knobby mass was located centrally in the brain, filling the third ventricle and causing extensive, obstructive hydrocephalus. Results of histopathologic examination were consistent with a mature teratoma with immature components. The most likely site of origin was thought to be pineal or hypothalamic. Hepatomegaly with marked extramedullary hematopoiesis was noted and the placenta was hydropic.
Comment The prenatal ultrasonographic features of intracranial teratoma involve gross distortion of normal cerebral architecture by an echogenic mass with multicystic areas. Polyhydramnios and craniomegaly are often noted. I Obstetric management is usually dictated by the cranial enlargement, which is caused by the tumor itself and as such rarely permits decompressive measures, such as transabdominal or transcervical cephalocente-
Fetal intracranial teratoma and high-output failure
99
sis. Abdominal delivery has been advocated, often with vertical uterine incision. In our case, in spite of massive craniomegaly with a head circumference of 58.5 cm, delivery was achieved through a transverse, lower-segment uterine incision in the presence of of a welldeveloped lower uterine segment. High-output cardiac failure, manifested in this case by scalp, facial, and body edema, hepatomegaly, extramedullary hematopoiesis, polyhydramnios, and a hydropic placenta, was most probably due to the large cardiac output required by tumor arteriovenous shunting in the massive intracranial teratoma. This mechanism has recently been described as the underlying pathophysiologic cause of high-output failure in a case of fetal sacrococcygeal teratoma. 2 REFERENCES
1. Lipman SH, Pretorius DH, Rumack eM, Manco-Johnson ML. Fetal intracranial teratoma: U.S. diagnosis of three cases, and a review of the literature. Radiology 1985;157: 491-4. 2. Bond SJ, Harrison MR, Schmidt KG, et al. Death due to high output cardiac failure in fetal sacrococcygeal teratoma. J Pediatr Surg 1990;25:1287-91.