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First report of case of congenital afibrinogenemia with successful delivery
Ultrasonic diagnosis of cloaca! exstrophy
\'olumc l:'\:l {';umber 7
vertebral anomalies, and clubfoot. Single umbilical artery is a common finding in this rare pathologic condition. In the case described by us, polyhydramnios was present, the bladder was clearly demonstrated, and the so-called omphalocele was huge (Fig. I). We believe that a detailed scan of the anomaly should reveal most of the prominent features of this malformation. The presence or absence of a bladder is of less importance when screening for the sequence of cloaca( exstrophy anomalies, especially when polyhydramnios is present. In addition, we believe that a-fetoprotein is
oflimited value in establishing a correct diagnosis, since ultrasound scanning, if detailed, may detect most features of this rare malformation. REFERENCES I. Hesser .JW, Murata Y. Swalwell Cl. Exstrophy of the cloaca with omphalocele: two cases. A" .J Ossn:r (;y:-.;u:o1. 1984; 150: 1004. 2. Haygood VF. Wahbeh CJ. Prospects for the prenatal diagnosis and obstetric management of cloaca! exstrophv . .J Reprod :\led 1983:28:807. 3. :\feizner I, Bar-Ziv .J. In utero prenatal ultrasonic diagnosis of a rare case of cloaca! exstrophy. JCT (in press).
First report of case of congenital afibrinogenemia with successful delivery Yutaka Inamoto, M.D., and Toshihiko Terao, M.D. Hammna/.111 City,.Japan We report a case in which pregnancy was sustained in a woman with congenital afibrinogenemia with delivery by cesarean section. From this case it appears that a plasma fibrinogen level >60 mg/di would maintain implantation of the placenta and fetus even in the event of complications occurring during pregnancy. (AM J OBSTET GYNECOL 1985;153:803-4.)
Key words: Congenital afibrinogenemia, deliven We report the first case of a successful pregnanc\' and deli\'ery in a :H-vear-old woman with congenital afibrinogenemia. Case report A 34-year old woman, gra\'ida 3, abortions 2, with a historv of congenital afibrinogenemia, visited our hospital because of genital bleeding similar to that with her previous abortion. The patient was born !\larch I 7, 1949, with a normal \'aginal birth, and was found to have congenital afibrinogenemia through the investigation of another case of congenital atibrinogenemia in her lineage. The plasma tibrinogen level determined immunologically was 0 mg/di. She was married in l\la\', 1963. After two spontaneous abortions, she became pregnant after a last menstrual period of 5 da\'s, beginning August 30, 1982. She was seen at our hospital on October 4, I 982, with genital bleeding similar to that on the previous occasion. B-scan ultrasound examination
From the Department o(Ofotetrics and G_w1pro/og)·. llama11111/.111 Cnivenity Sdwol of ,Herliri11P. R.ereiverlfor puhlimtion Marr/1 2i, 1985; rr'11ised ,-\pnl 2i. 1985; accepted ,Way 21. 1985. Refirint reque.1/s: Yutaka lnrmwto, ,\f.D., Departmo1t of Oh.1tetrirs and Gynecology, llmnamatsu Uni1iersity Srlwol of Mediri11e, '3600 Handa-clw, Hamamalrn Cit.\', 4 31-3 I Japan.
revealed a gestational sac of I 5 by I 5 mm, and the diagnosis was threatened abortion. The patient was hospitalized for rest and recei\'ed 3 gm of tibrinogen bv intr;n·enous drip. Continuous intra\·enous drip of 8 gm/wk of fibrinogen was administered e\'en after the genital bleeding stopped because the previous abortion was considered to be due to the fact that no fibrinogen was added. On October 2:!, (the eighth week and second da\' of pregnanc\'), B-scan ultrasound examination revealed a fetal heartbeat. :'\lo genital bleeding was observed, and intravenous fibrinogen was decreased from 4 to 2 gm/wk, which maintained a plasma fibrinogen level of 5 to 40 mg/di. However, dut·ing the twent\'-tirst week of pregnancv, genital bleeding sudden(\' occurred. The plasma hbrinogen le\'el immediate(\' before bleeding began was 40 mg/di. The amount of intravenous fibrinogen was immediate)\' increased to 12 gm/wk in an attempt to stop bleeding. The plasma fibrinogen level was 84 mg/di when the bleeding stopped. After that {) gm/wk of tibrinogen was intravenously dripped to maintain a plasma fibrinogen level of 20 to 40 mg/di so that pregnatK\' rnuld be continued to the thirty-seventh week. The patient was a mature primipara with rigidity of the cervix, and it was clecided to perform a cesarean section. On May 18, 1983 (the thirty-seventh week and first day of pregnancy). the cesarean section was conducted with a drip of 8 gm of intra\'enous fibrinogen, and the plasma fibrinogen le\'el
803
lnamoto and Terao
was l 20 mg/di during operation. A male infant weighing 2380 gm was delivered. The Apgar score was 9 at the time of birth. The placenta weighed 490 gm. The baby obviously had congenital afibrinogenemia with a fibrinogen level of 55 mg/di in the umbilical vein. After the cesarean section, 6 to l 2 gm/wk of fibrinogen was given to maintain a plasma fibrinogen level of 60 to 120 mg/di for 2 weeks. The amount of lochia was normal, and the patient was discharged from our hospital on June 5.
Comment There is no sex-related difference in the incidence of congenital afibrinogenemia as it is an inherited autosomal recessive trait and has been reported in more than I 00 cases. According to my investigation, there have been 73 cases in female subjects. Five of the women were reported to be married. Among these five, there were two pregnancies that resulted in abortion during the second' and third' months of pregnancy, respectively. On the other hand, many articles have reported on cases of pregnancy in patients with congenital hypofibrinogenemia, and almost all had normal deliveries if the plasma fibrinogen level was >60 mg/di. Because of the three pregnancies in this patient and
December I, 1985 Am J Obstet Gynecol
in the patients with congenital afibrinogenemia or hypofibrinogenemia reported on so far, fibrinogen may not be necessary for implantation. This is suggested by the fact that in the cases reported by Matsuno et al.' and Dube et al.' the patients became pregnant despite congenital afibrinogenemia. However, fibrinogen may be required to maintain the placenta and fetus. It appears that a plasma fibrinogen level >60 mg/di is needed to maintain implantation of the placenta and fetus especially because of the bleeding that occurred during pregnancy in this case. Dube et al.' stated in their report that their case was the first of congenital afibrinogenemia and pregnancy in the world. The case reported here is the first of congenital afibrinogenemia and a successful delivery in the world. REFERENCES I. Matsuno K, Mori K, Amikawa H, et al. A case of congenital afibrinogenemia with abortion, intracranial hemorrhage, and peritonitis . .Jpn.J Clin Hematol 1977;18:1438. 2. Dube B, Agarwal SP, Gupta ~1\1, Chawla SC. Congenital deficiency of fibrinogen in two sisters. A clinical and haematological study. Acta Haematol 1970;43:120.
Abdominal pregnancy following gonadotropin treatment F. 0. Saracoglu, M.D., E. Goksin, M.D., and T. Durukan, M.D. Ankara, Turkey An abdominal pregnancy after treatment with human menopausal and chorionic gonadotropins is reported. The role of induction of ovulation with human menopausal and chorionic gonadotropins as a cause of ectopic pregnancy has not been delineated. However, it appears that ultrasonography has become one of the most important aids in the diagnosis of abdominal pregnancy. (AM J OssTET GvNECOL 1985;153:804-5.)
Key words: Abdominal pregnancy, gonadotropin treatment, abruptio placentae, ultrasonography Abdominal pregnancy is one of the rare but serious problems in obstetrics. Several cases of abdominal pregnancy have been reported and the current diagnostic and management techniques have been discussed in the literature, but, to our knowledge, this is the first case which occurred after induction of ovulation with human menopausal and chorionic gonadotropins and was
From the Department of ObJtetrics and GJ•nerology, Harette/Je Universi~y School of Medicine. Received for publirationjamuiry 2, 1985; revised March 12. 1985: accepted May 20, 1985. Reprint requests: Dr. F. 0. Saracoglu, Gazi Mmtafa Kema/ Buh•ari, No. 120119, Maltepe, Ankara, Turkey.
complicated with nearly total abruptio placentae. This is also the third English-literature report of an abdominal pregnancy diagnosed by ultrasonography at less than 15 weeks' gestation.'
Case report A 34-year-old woman, gravida I, para 0, was seen in the obstetric department of Hacettepe University Medical Center because of acute pain in the lower abdomen. She had failed to ovulate after clomiphene therapy, but conceived after ovulation had been induced with human menopausal and chorionic gonadotropins in the first cycle. She was 13 weeks pregnant by estimated gestational age at this time.
\'olumc l:'\:l {';umber 7
vertebral anomalies, and clubfoot. Single umbilical artery is a common finding in this rare pathologic condition. In the case described by us, polyhydramnios was present, the bladder was clearly demonstrated, and the so-called omphalocele was huge (Fig. I). We believe that a detailed scan of the anomaly should reveal most of the prominent features of this malformation. The presence or absence of a bladder is of less importance when screening for the sequence of cloaca( exstrophy anomalies, especially when polyhydramnios is present. In addition, we believe that a-fetoprotein is
oflimited value in establishing a correct diagnosis, since ultrasound scanning, if detailed, may detect most features of this rare malformation. REFERENCES I. Hesser .JW, Murata Y. Swalwell Cl. Exstrophy of the cloaca with omphalocele: two cases. A" .J Ossn:r (;y:-.;u:o1. 1984; 150: 1004. 2. Haygood VF. Wahbeh CJ. Prospects for the prenatal diagnosis and obstetric management of cloaca! exstrophv . .J Reprod :\led 1983:28:807. 3. :\feizner I, Bar-Ziv .J. In utero prenatal ultrasonic diagnosis of a rare case of cloaca! exstrophy. JCT (in press).
First report of case of congenital afibrinogenemia with successful delivery Yutaka Inamoto, M.D., and Toshihiko Terao, M.D. Hammna/.111 City,.Japan We report a case in which pregnancy was sustained in a woman with congenital afibrinogenemia with delivery by cesarean section. From this case it appears that a plasma fibrinogen level >60 mg/di would maintain implantation of the placenta and fetus even in the event of complications occurring during pregnancy. (AM J OBSTET GYNECOL 1985;153:803-4.)
Key words: Congenital afibrinogenemia, deliven We report the first case of a successful pregnanc\' and deli\'ery in a :H-vear-old woman with congenital afibrinogenemia. Case report A 34-year old woman, gra\'ida 3, abortions 2, with a historv of congenital afibrinogenemia, visited our hospital because of genital bleeding similar to that with her previous abortion. The patient was born !\larch I 7, 1949, with a normal \'aginal birth, and was found to have congenital afibrinogenemia through the investigation of another case of congenital atibrinogenemia in her lineage. The plasma tibrinogen level determined immunologically was 0 mg/di. She was married in l\la\', 1963. After two spontaneous abortions, she became pregnant after a last menstrual period of 5 da\'s, beginning August 30, 1982. She was seen at our hospital on October 4, I 982, with genital bleeding similar to that on the previous occasion. B-scan ultrasound examination
From the Department o(Ofotetrics and G_w1pro/og)·. llama11111/.111 Cnivenity Sdwol of ,Herliri11P. R.ereiverlfor puhlimtion Marr/1 2i, 1985; rr'11ised ,-\pnl 2i. 1985; accepted ,Way 21. 1985. Refirint reque.1/s: Yutaka lnrmwto, ,\f.D., Departmo1t of Oh.1tetrirs and Gynecology, llmnamatsu Uni1iersity Srlwol of Mediri11e, '3600 Handa-clw, Hamamalrn Cit.\', 4 31-3 I Japan.
revealed a gestational sac of I 5 by I 5 mm, and the diagnosis was threatened abortion. The patient was hospitalized for rest and recei\'ed 3 gm of tibrinogen bv intr;n·enous drip. Continuous intra\·enous drip of 8 gm/wk of fibrinogen was administered e\'en after the genital bleeding stopped because the previous abortion was considered to be due to the fact that no fibrinogen was added. On October 2:!, (the eighth week and second da\' of pregnanc\'), B-scan ultrasound examination revealed a fetal heartbeat. :'\lo genital bleeding was observed, and intravenous fibrinogen was decreased from 4 to 2 gm/wk, which maintained a plasma fibrinogen level of 5 to 40 mg/di. However, dut·ing the twent\'-tirst week of pregnancv, genital bleeding sudden(\' occurred. The plasma hbrinogen le\'el immediate(\' before bleeding began was 40 mg/di. The amount of intravenous fibrinogen was immediate)\' increased to 12 gm/wk in an attempt to stop bleeding. The plasma fibrinogen level was 84 mg/di when the bleeding stopped. After that {) gm/wk of tibrinogen was intravenously dripped to maintain a plasma fibrinogen level of 20 to 40 mg/di so that pregnatK\' rnuld be continued to the thirty-seventh week. The patient was a mature primipara with rigidity of the cervix, and it was clecided to perform a cesarean section. On May 18, 1983 (the thirty-seventh week and first day of pregnancy). the cesarean section was conducted with a drip of 8 gm of intra\'enous fibrinogen, and the plasma fibrinogen le\'el
803
lnamoto and Terao
was l 20 mg/di during operation. A male infant weighing 2380 gm was delivered. The Apgar score was 9 at the time of birth. The placenta weighed 490 gm. The baby obviously had congenital afibrinogenemia with a fibrinogen level of 55 mg/di in the umbilical vein. After the cesarean section, 6 to l 2 gm/wk of fibrinogen was given to maintain a plasma fibrinogen level of 60 to 120 mg/di for 2 weeks. The amount of lochia was normal, and the patient was discharged from our hospital on June 5.
Comment There is no sex-related difference in the incidence of congenital afibrinogenemia as it is an inherited autosomal recessive trait and has been reported in more than I 00 cases. According to my investigation, there have been 73 cases in female subjects. Five of the women were reported to be married. Among these five, there were two pregnancies that resulted in abortion during the second' and third' months of pregnancy, respectively. On the other hand, many articles have reported on cases of pregnancy in patients with congenital hypofibrinogenemia, and almost all had normal deliveries if the plasma fibrinogen level was >60 mg/di. Because of the three pregnancies in this patient and
December I, 1985 Am J Obstet Gynecol
in the patients with congenital afibrinogenemia or hypofibrinogenemia reported on so far, fibrinogen may not be necessary for implantation. This is suggested by the fact that in the cases reported by Matsuno et al.' and Dube et al.' the patients became pregnant despite congenital afibrinogenemia. However, fibrinogen may be required to maintain the placenta and fetus. It appears that a plasma fibrinogen level >60 mg/di is needed to maintain implantation of the placenta and fetus especially because of the bleeding that occurred during pregnancy in this case. Dube et al.' stated in their report that their case was the first of congenital afibrinogenemia and pregnancy in the world. The case reported here is the first of congenital afibrinogenemia and a successful delivery in the world. REFERENCES I. Matsuno K, Mori K, Amikawa H, et al. A case of congenital afibrinogenemia with abortion, intracranial hemorrhage, and peritonitis . .Jpn.J Clin Hematol 1977;18:1438. 2. Dube B, Agarwal SP, Gupta ~1\1, Chawla SC. Congenital deficiency of fibrinogen in two sisters. A clinical and haematological study. Acta Haematol 1970;43:120.
Abdominal pregnancy following gonadotropin treatment F. 0. Saracoglu, M.D., E. Goksin, M.D., and T. Durukan, M.D. Ankara, Turkey An abdominal pregnancy after treatment with human menopausal and chorionic gonadotropins is reported. The role of induction of ovulation with human menopausal and chorionic gonadotropins as a cause of ectopic pregnancy has not been delineated. However, it appears that ultrasonography has become one of the most important aids in the diagnosis of abdominal pregnancy. (AM J OssTET GvNECOL 1985;153:804-5.)
Key words: Abdominal pregnancy, gonadotropin treatment, abruptio placentae, ultrasonography Abdominal pregnancy is one of the rare but serious problems in obstetrics. Several cases of abdominal pregnancy have been reported and the current diagnostic and management techniques have been discussed in the literature, but, to our knowledge, this is the first case which occurred after induction of ovulation with human menopausal and chorionic gonadotropins and was
From the Department of ObJtetrics and GJ•nerology, Harette/Je Universi~y School of Medicine. Received for publirationjamuiry 2, 1985; revised March 12. 1985: accepted May 20, 1985. Reprint requests: Dr. F. 0. Saracoglu, Gazi Mmtafa Kema/ Buh•ari, No. 120119, Maltepe, Ankara, Turkey.
complicated with nearly total abruptio placentae. This is also the third English-literature report of an abdominal pregnancy diagnosed by ultrasonography at less than 15 weeks' gestation.'
Case report A 34-year-old woman, gravida I, para 0, was seen in the obstetric department of Hacettepe University Medical Center because of acute pain in the lower abdomen. She had failed to ovulate after clomiphene therapy, but conceived after ovulation had been induced with human menopausal and chorionic gonadotropins in the first cycle. She was 13 weeks pregnant by estimated gestational age at this time.