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16

17

18

19

immunologic protein S levels are decreased during pregnancy. Blood 1986;68:881-885. Lao TT, Yuen PMP, Yin JA. Protein S and protein C levels in Chinese women during pregnancy, delivery and the puerperium. Br J Obstet Gynaecol 1989;96:167-170. Gonzalez R, Alberta I, Vincente V. Protein C levels in late pregnancy, post-partum and in women on oral contraceptives. Thromb Res 1985;39:637-640. Mannucci PM, Vigano S, Botasso B et al. Protein C antigen during pregnancy, delivery and puerperium. Thromb Haemost 1984;52:217. Hopmeier P, Halbmayer M, Schwarz HP et al. Protein C and S in mild and moderate preeclampsia. Thromb Haemostas 1987;58:794-795.

20 Gilabert J, Fernandez JA, Espana F et al. Physiological coagulation inhibitors (protein S, protein C and antithrombin III) in severe pre-eclamptic states and in users of oral contraceptives. Thromb Res 1988;49:319-329. 21 Brenner B, Shapira A, Bahari C et al. Hereditary protein C deficiency during pregnancy. Am J Obstet Gynecol 1987;157:1160-1161. 22 Clause LH, Comp PC. The regulation of hemostasis: the protein C system. N Engl J Med 1986;315:1298-1304. 23 Vogel JJ, de Moerloose PA, Bounameaux H. Protein C deficiency and pregnancy: A case report. Obstet Gynecol 1989:73:455-456.

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European Journal of Obstetrics & Gynecology and Reproductive Biology, 45 (1992) 210-214.

0 1992 Elsevier Science Publishers B.V. All rights reserved 0028-2243/92/$05.00 EUROBS 01268

Prenatal ultrasound diagnosis of an intracranial teratoma influencing management: case report and review of the literature Edith D.M. ten Broeke, George W. Verdonk and Frans J.M.E. Roumen Department of Obstetrics and Gynecology, St. Elisabeth Clinic - School for Midwifery, Heerlen, Netherlands

Accepted for publication 15 August 1991

Summary Of seventeen cases of prenatally diagnosed intracranial teratomas, fourteen babys were delivered through a cesarean section. All children died before or shortly after delivery. In this report another case is presented of ultrasound diagnosis of an intracranial teratoma. The teratoma filled the whole fetal cranium, and eroded through the skull base into the left maxillary region. In full agreement with the wishes of the parents, conservative management resulted in the spontaneous vaginal delivery of a fetus that died during labor as a result of brain compression and acidosis during the second stage. In contrast with other cases in the literature, no encephalocentesis was performed. In order to minimize maternal morbidity, it is advised to inouce or accept labor as soon as the diagnosis is clear and to pursue a vaginal delivery. Intracranial teratoma; Ultrasonography; Prenatal diagnosis

Correspondence:

Dr. Edith D.M. ten Broeke, Department of Obstetrics Midwifery, Zandweg 180, 6418 PB Heerlen, the Netherlands.

and Gynecology, St. Elisabeth

Clinic - School for

211

Introduction Congenital intracranial teratomas of the fetus are extremely rare tumors, that can be diagnosed antenatally by ultrasound [1,2]. Several cases have been reported in the literature. Although the neonatal mortality of this condition is lOO%, a cesarean section is frequently performed. A new case is reported in which conservative management after ultrasound diagnosis resulted in a spontaneous vaginal delivery of a stillborn infant. Case report A 23-year-old, Caucasian female, was referred to our hospital during her first pregnancy at 29 weeks’ gestation, because of hypertension and spontaneous rupture of the membranes. The course of her pregnancy till then had been uneventful. At admission, a polyhydramnios was diagnosed with a large for date fundal height (32 cm). Ultrasound examination showed an extremely and asymmetrically enlarged fetal head (biparietal diameter 104 mm). A normal cerebral structure could not bc identified, no midline echo or ventricular system could be ascertained. The cranium was completely filled with bizarre cystic and echogenic regions, with variable echo intensities (Fig. 1). The fetal abdominal circumference of 258 mm was in accordance with the gestational age. The stomach was repeatedly empty, there was a marked polyhydramnios. The (Yfetoprotein levels were elevated: 1030 units/ml in maternal serum, and 3700 units/ml in amniotic fluid. All other laboratory investigations were normal.

Fig. 1. Ultrasonography of the fetal head in utero, showing an irregular echogenic mass intracranially with one large and multiple smaller cysts. (C = cyst, T = teratoma, H = hand of the fetus; arrowhead indicates frontal bone).

The parents were extensively informed of the poor prognosis for the baby. They agreed with our suggestion of an expectative management, probably resulting in the demise of the fetus. Five days after admission preterm labor started spontaneously. Cardiotocography, which was normal before labor, showed an extremely silent pattern with deep decelerations during the second stage, resulting in a persistent bradycardia (Fig. 2). The fetus died before birth. A male infant of 1700 g with a completely normal body was delivered. The circumference of the head was 38 cm (Fig. 3). A tumor-like mass distorted the left half of the face, pushing aside the palatum and left cyc. The right half of the face was normal. At autopsy, normal brain tissue was pressed into a small part of the backside of the intracranium by a large, massive tumor of very weak consistency with multiple cysts. The tumor extended into the left orbit, jaw and nose. No other malformations were detected at autopsy, the lungs were normal without hypoplasia. Histo-pathological examination of the tumor revealed tissues from all three germ layers. The bulk of the tumor was composed of immature neural elements, including glial tissue and neuroepithelial canals. There wcrc no signs of malignancy. Comment Teratomas are the most frequent congenital tumors. However, in only 2% of all cases the tumor is located intracranially and arises from the central nervous system [3]. In the present case, the greatest part of the tumor consisted of immature neural elements, which makes an origin from the central nervous system very likely. An intracranial teratoma can be diagnosed prenatally by ultrasound [1,2]. As summarized in Table I, only I7 cases were reported in literature. Intracranial teratomas appear as irregular echogenic masses with transsonic areas. The normal cerebral structure can not be recognized, a midline echo is mostly absent. The head is enlarged. The differential diagnosis includes other intracranial neoplasms, hydrocephalus, hydranencephaly, holoprosencephaly, porencephaly, arachnoid cysts and meningo-encephaloceles [7,9]. The most readily obtainable and reliable sonographic indicator of hydrocephalus is dilatation of the bodies of the lateral ventricles. A midline echo, which is always present, can be shifted, resulting in asymmetry of the ventricles. Ventricular enlargement can occur in the presence of macro- or microcephaly [16]. In hydranencephaly, ultrasound findings resemble massive hydro-

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213

TABLE Revxw

J of the literature

on ultrasound

diagnosis

of a fetal inlracranial

teratoma

Ref.

GA

Presenting symptom

Hoff and Mackay, 1980 [ 11 Crade, 1982 [2] Kirkinen et al., 1982 [41 Paes et al., 1982 [5] Vinters et al., 1982 161 Gadwood and Reynes, 1983 [7j Chervenak et al., 1985 [8] Lipman et al., 1985 [9] Lipman et al., 1985 [9] Lipman et al., 1985 [9] Rostad et al., 1985 [lo] Rostad et al., 1985 [lOI Miiller et al., 1986 [ 111 Ode11 et al., 1987 [12] Richards, 1987 [13] Daita et al., lY8Y [141 Dolkart et ai.. 1990 [15]

28 40 36 36 31 32 32 36 22 32 37 21 35 29 2.5 28 37

S>D none S>D none S>D S > D, breech S>D S > D, breech S>D none S > D, breech S>D none

S>D S>D

16.5 14.0 10.5 15.3 13.0

Present

29

S > D, hypertension

10.4

case

BPD

diameter

HC

_ 10.5

> 14.8 _ 15.0

_ _ 10.0 12.3 12.0 _ _

S>D S > D, pre-eclampsia

GA, gestational age in weeks; BPD, biparietal CS, ccsarcan section; EC. encephaloccntesis

in utero

in centimeters;

_ 38 _ 59

3x 32 5.5 44, 37

Mode of delivery

Fetal death

cs cs

in minutes in 2 hours stillborn in 4 days stillborn

Vaginal. EC Vaginal, induction, EC CS, repeat, EC cs cs CS, head too large CS, failed induction Vaginal, induction, EC CS, head too large CS, failed induction cs cs CS, failed induction, EC CS, head too large cs Vaginal

HC, head circumference

shortly in 1 hour in 3 minutes in 5 minutes stillborn in minutes shortly in 5 days in 9 hours stillborn shortly in 35 days stillborn

in centimeters;

-

S > D, bize > date;

cephalus 1161. The midline echo is absent in alobar holoprosencephaly and incomplete in the semilobar variety. Hypotelorism and ventriculomegaly are frequently seen in holoprosencephaly. The head can be too great or too small [17]. Solitary cysts are found in porencephaly or as arachndid cysts. Most cases of intracranial teratomas in the literature presented with an uterine size larger than expected for gestational age, with polyhydramnios. The ultrasound findings in the present case of polyhydramnios and a repeatedly empty fetal stomach could be interpreted as an inability of the fetus to swallow the amniotic fluid. This could be caused by two different mechanisms: destruction of the fetal brain stem with a paralysis of the pharynx muscles as a result, or mechanical obstl-uction of the pharynx by the tumor mass. All babies with a prenatally detected intracranial teratoma described in the literature died bcforc delivery or shortly afterwards. One baby died of the complications of prematurity shortly after birth [8]. Three other babies died of cardiovascular or respiratory failure [11,12,15]. One baby died during vaginal delivery, because the head was so compressed that a mass of tumor was squeezed out of it [4]. In the other twelve cases described in literature no reason for the fetal or Fig. 3. Fetal intracranial teratoma, distorting the left half of the face with suppression of the left eye and palatum.

214

neonatal demise was mentioned. All cases showed a massive destruction or compression of the brain tissue. Probably, the absence of sufficient normal brain tissue is the most important factor contributing to fetal or neonatal death. In fourteen cases (82.4%) a cesarean section was performed, in eleven of these cases without a trial of labor. In one case a repeat section was done. In three cases the head was expected to be too large for a vaginal delivery. Only four women delivered vaginally, two of them after induction of labor and fetal encephalocentesis, and one of them after encephalocentesis. Only the patient of the present case delivered spontaneously, without encephalocentesis or any other medical interference, which was in full agreement with the patients’ wish and the wish of her partner. Cardiotocography before labor did not show the pattern of fetal brain death [18]. So, in this case, there must have been some influence of the distorted and displaced fetal brain on the fetal heart rate. Compression of the fetal brain during the second stage of labor must have resulted in this very silent heart rate pattern with deep decelerations, leading to extreme acidosis and demise of the fetus. In the present case rupture of membranes induced spontaneous onset of labor, resulting in a vaginal delivery before the head of the fetus was too large to cause mechanical obstruction. As neonatal mortality of children with a massive congenital intracranial teratoma is lOO%, it is advisable to induce or accept labor, as soon as the diagnosis is clear. A vaginal delivery with or without fetal encephalocentesis should be pursued and a cesarean section should be prevented, in order to minimize maternal morbidity. References Hoff NR, Mackay IM. Prenatal ultrasound diagnosis of intracranial teratoma. JCU 1980:8:247-249. Crade M. Ultrasonic demonstration in utero of an intracranial teratoma. JAMA 1982;247:1173. Gonzalez-Crussi F. Extragonadal teratomas. In: Hartmann WH, Cowan WR, eds. Atlas of tumor pathology, Second Series, Fascicle 18, Armed Forces Institute of Pathology, Washington DC, 1982.

4 Kirkinen P, Suramo I, Jouppila P et al. Combined use of ultrasound and computed tomography in the evaluation of fetal intracranial abnormality. J Perinat Med 1982;10:257265. 5 Paes BA, deSA DJ, Hunter DJS et al. Benign intracranial teratoma: prenatal diagnosis influencing early delivery. Am J Obstet Gynecol 1982;143:600-601. 6 Vinters HV, Murphy J, Wittmann B et al. Intracranial teratoma: Antenatal diagnosis at 31 weeks’ gestation by ultrasound. Acta Neuropathol (Berll 1982;58:233-236. 7 Gadwood KA, Reynes CJ. Intracranial teratoma. Ill Med J 1983;164:196-198. 8 Chervenak FA, Isaacson G, Touloukian R et al. Diagnosis and management of fetal teratomas. Obstet Gynecol 1985;66:666-671. 9 Lipman SP, Pretorius DH, Rumack CM et al. Fetal intracranial teratoma: US diagnosis of three cases and a review of the literature. Radiology 1985;157:491-494. 10 Rostad S, Kleinschmidt-DeMasters BK, Manchester DK. Two massive congenital intracranial immature teratomas with neck extension. Teratology 1985;32:163-169. 11 Miiller K, Kiwit JCW, Terinde R. Kongenitales intrakranielles Teratom, Sonographische Pranataldiagnostik, Klinik und Neuropathologie. Monatsschr Kinderheilkd 1986;134:550-553. 12 Odell JM, Allen JK, Badura RJ et al. Massive congenital intracranial teratoma: a report of two cases. Pediatr Pathol 1987;7:333-340. 13 Richards SR. Ultrasonic diagnosis of intracranial teratoma in utero, a case report and literature review. J Reprod Med 1987;32:73-75. 14 Daita G, Yonemasu Y, Ishikawa M et al. Intracranial malignant teratoma diagnosed in a fetus, case report. Neurol Med Chir (Tokyo) 1989;29:1026-1029. 15 Dolkart LA, Balcom RJ, Eisinger G. Intracranial teratoma: prolonged neonatal survival after prenatal diagnosis. Am J Obstet Gynecol 1990;162:768-769. 16 Chetvenak FA, Berkowitz RL, Bomero R et I ‘. The diagnosis of fetal hydrocephalus. Am J Obstet G. lecol 1983;147:703-716. 17 Chervenak FA, Isaacson G, Hobbins JC et al. Diagnc ;s and management of fetal holoprosencephaly. Obstet Gynecol 1985;66:322-326. 18 Niihuis JG, Crevels AJ, van Dongen PWJ. Fetal brain death:the definition of a fetal heart rate pattern and its clinical consequences. Obstet Gynecol Smv 1990;45:229232.

Prenatal ultrasound diagnosis of an intracranial teratoma influencing management: case report and review of the literature.

Of seventeen cases of prenatally diagnosed intracranial teratomas, fourteen babies were delivered through a cesarean section. All children died before...
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