Journal of Obstetrics and Gynaecology

ISSN: 0144-3615 (Print) 1364-6893 (Online) Journal homepage: http://www.tandfonline.com/loi/ijog20

Spontaneous septostomy in monochorionic diamniotic twins: Difficult diagnosis, difficult management E. Bevilacqua, C. Aliberti, V. D’Ambrosio, A. Giancotti, G. Perrone & R. La Torre To cite this article: E. Bevilacqua, C. Aliberti, V. D’Ambrosio, A. Giancotti, G. Perrone & R. La Torre (2014) Spontaneous septostomy in monochorionic diamniotic twins: Difficult diagnosis, difficult management, Journal of Obstetrics and Gynaecology, 34:4, 359-359 To link to this article: http://dx.doi.org/10.3109/01443615.2013.874408

Published online: 31 Jan 2014.

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Obstetric Case Reports 359 complications during pregnancy associated with Marfan syndrome is approximately 3.9% (Katsuragi et al. 2011), or five times higher than in women who are not pregnant (Pacini et al. 2009). The prognoses of mother and fetus are extremely poor when acute aortic dissection occurs during pregnancy. The mortality rate associated with aortic dissection during pregnancy is 22% (Weiss et al. 1998). Although rapid treatment of pregnant women with Marfan syndrome is necessary, use of anticoagulants during aortic repair make it difficult to determine the optimal treatment strategies. Aortic surgery with extracorporeal circulation requires the use of large quantities of anticoagulants. These may cause massive bleeding due to placental abrasions and uterine wounds. Haas et al. (2011) reported that when surgical repair of type A aortic dissection was performed with extracorporeal circulation just after an urgent caesarean section at 34 weeks’ gestation, uterine haemorrhage occurred immediately postoperatively, and hysterectomy was eventually performed because conservative therapy of the bleeding was impossible. It may be recommended here that hysterectomy after caesarean section is considered, so as to prevent bleeding caused by anticoagulants. In this case, we planned to perform the surgical repair of the aorta a few days after the caesarean section and hysterectomy, in consideration of wound healing following obstetric surgery and in order to avoid uterine bleeding caused by anticoagulants. However, because of worsened postoperative pneumonia, we had to postpone the aortic repair and associated extracorporeal circulation until day 9 after the obstetric operation. Declaration of interest: The authors declare that are no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

References Haas S, Trepte C, Rybczynski M et al. 2011. Type A aortic dissection during late pregnancy in a patient with Marfan syndrome. Canadian Journal of Anaesthesia 58:1024–1028. Immer FF, Bansi AG, Immer-Bansi SI et al. 2003. Aortic dissection in pregnancy: analysis of risk factors and outcome. Annals of Thoracic Surgery 76:309–314. Kats NM, Collea JV, Mront MG et al. 1984. Aortic dissection during pregnancy: treatment by emergency cesarean section immediately followed by operative repair of the aortic dissection. American Journal of Cardiology 54:699–701. Katsuragi S, Ueda K, Yamanaka K et al. 2011. Pregnancy-associated aortic dilatation or dissection in Japanese women with Marfan syndrome. Circulation Journal 75:2545–2551. Pacini L, Digne F, Boumendil A et al. 2009. Maternal complication of pregnancy in Marfan syndrome. International Journal of Cardiology 136:156–161. Weiss BM, von Segesser LK, Alon E et al. 1998. Outcome of cardiovascular surgery and pregnancy: A systematic review of the period 1984–1996. American Journal of Obstetrics and Gynecology 179:1643–1653. Zeebregts CJ, Schepens MA, Hameeteman TM et al. 1997. Acute aortic dissection complicating pregnancy. Annals of Thoracic Surgery 64:1345–1348.

is often unclear and can be attributed to chorioamnionitis, fetal movement, polyhydramnios and development disturbances (Gilbert et al. 1991). Monochorionic monoamniotic pregnancy (MM) has the highest perinatal mortality (30–70%), mainly due to cord entanglement (CE) (Shveiky et al. 2004). In MD, the perinatal mortality rate is 11.6% (Hack et al. 2008). Pseudo-monoamniotic pregnancy (PMM), derived from septostomy in MD, should be managed as MM, as the risk of CE rises significantly. CE was reported in about 72% of PMM, similar to the 70% risk in MM (Gilbert et al. 1991; Yoshimura et al. 2009; Tamura et al. 2011; Chadha et al. 2012; Lee et al. 2012; Abraham 2013; Suzuki 2013).

Case report A healthy 36-year-old woman, gravida 3, para 2, was referred to our Prenatal Centre, for MD. The 1st trimester screening for chromosomal abnormalities was negative. No invasive procedure was performed. Every 2 weeks, a scan examination showed reassuring biophysical profile scores and no evidence of developing twin-to-twin transfusion syndrome. Anomaly scan at 22 weeks’ gestation did not detect any alterations. At 24 weeks, the demise of both twins was detected and SS with CE was suspected. The woman was admitted to hospital for induction of labour. Histopathological examinations confirmed the diagnosis of monochorionic diamniotic placentation with entangled, knotted and closely inserted umbilical cords.

Discussion A total of 16 articles reporting 25 cases of PMM have been published so far (Gilbert et al. 1991; Yoshimura et al. 2009; Tamura et al. 2011; Chadha et al. 2012; Lee et al. 2012; Abraham 2013; Suzuki 2013). The most important concern following SS is CE, occurring in 18 out of the 25 reported cases (72%). Mortality rates in MM and PMM are similar: 33% and 21%, respectively (Suzuki 2013). Therefore, it is important to diagnose PMM. PMM should be suspected in the presence of the following markers: very thin inter-twin membrane; close cord insertion; male gender; grandmultiparous women; polyhydramnios; invasive procedures (Yoshimura et al. 2009). In our case, we did not suspect SS because the dividing inter-twin membrane, even if very thin, was clearly visible in every scan (Figure 1). Criteria for diagnosis of SS and CE have been defined, but the diagnosis remains very challenging. Ultrasound findings suggesting SS are: absent or ruptured membrane; fetuses seen on the same side of the ruptured membrane; CE; polyhydramnios. The diagnosis of CE is made by the identification of a free floating braid or loop of umbilical cords, or the demonstration of crossing vessels or differing arterial

Spontaneous septostomy in monochorionic diamniotic twins: Difficult diagnosis, difficult management E. Bevilacqua, C. Aliberti, V. D’Ambrosio, A. Giancotti, G. Perrone & R. La Torre Department of Gynaecology, Obstetrics and Urologic Sciences, University of Rome ‘Sapienza’, Italy DOI: 10.3109/01443615.2013.874408 Correspondence: E. Bevilacqua, Dipartimento di Scienze Ginecologiche, Perinatologia e Puericultura, Via del Policlinico 155, 00161 Roma. E-mail: [email protected]

Introduction Spontaneous septostomy (SS) in monochorionic diamniotic pregnancy (MD) is an uncommon severe event. Aetiology of SS

Figure 1. Transabdominal ultrasonography at 22 weeks’ gestation shows the thin inter-twin membrane.

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waveforms within that loop of cords by colour Doppler (Chadha et al. 2012). In conclusion, it is important to detect the two insertions of the umbilical cords and their distance; to scan the whole length of the inter-twin dividing membrane in twin pregnancy at every scan, along with a search for evidence of CE; to counsel appropriately the couple to ‘let them prepare’ for adverse events; to manage PMM as the MM. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

References

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Abraham RJ. 2013. The protective effect of oligohydramnios in a case of missed diagnosis of spontaneous septostomy in monochorionic diamniotic twins. Journal of Obstetrics and Gynaecology 33:205–207. Chadha R, Lange IR, Bratz L et al. 2012. A rare case of antepartum spontaneous septostomy in a monochorionic diamniotic twin pregnancy. Case Reports in Obstetrics and Gynecology 2012:748614. Gilbert WM, Davis SE, Kaplan C et al. 1991. Morbidity associated with prenatal disruption of the dividing membrane in twin gestations. Obstetrics and Gynecology 78:623–630. Hack KE, Derks JB, Elias SG et al. 2008. Increased perinatal mortality and morbidity in monochorionic versus dichorionic twin pregnancies: clinical implications of a large Dutch cohort study. British Journal of Obstetrics and Gynaecology 115:58–67. Lee KJ, Kim MK, Lee SY et al. 2012. Spontaneous rupture of the dividing membrane in a monochorionic pregnancy resulting in a pseudo-monoamniotic pregnancy with cord entanglement. Journal of Obstetrics and Gynaecology Research 38:863–866. Shveiky D, Ezra Y, Schenker JG et al. 2004. Monoamniotic twins: an update on antenatal diagnosis and treatment. Journal of Maternal-Fetal and Neonatal Medicine 16:180–186. Suzuki S. 2013. Case series of monoamniotic and pseudo-monoamniotic twin gestations. ISRN Obstetrics and Gynecology 2013:369419. Tamura T, Miura A, Suzuki S. 2011. Spontaneous disruption of the dividing membrane in monochorionic diamniotic twin pregnancy. Fetal Diagnosis and Therapy 30:241–242. Yoshimura K, Aiko Y, Inagaki H et al. 2009. Prenatal spontaneous disruption of the dividing membrane in monochorionic diamniotic twins detected at the time of fetoscopic laser photocoagulation. Journal of Obstetrics and Gynaecology Research 35:1129–1131.

Crimean-congo haemorrhagic fever in pregnancy and in newborn: A case with a unique clinical course A. Ünlüsoy-Aksu1, C. Havali1, A. Tapisiz1, F. Aktaş2 & F. Ezgu1 Departments of 1Pediatrics and 2Infectious Diseases, Faculty of Medicine, Gazi University, Ankara, Turkey DOI: 10.3109/01443615.2013.874984 Correspondence: F. Ezgu, Department of Pediatrics, Faculty of Medicine, Gazi University, 10th Floor, Besevler, Ankara 06500, Turkey. E-mail: [email protected]

Introduction Crimean-Congo haemorrhagic fever (CCHF) is a potentially fatal viral infection described in Asia, Africa, the Middle-East and Eastern Europe (Ergonul 2006). Mortality rates are reported to range from 15% to 70% (Mardani and Keshtkar-Jahromi 2007). The virus is transmitted primarily through the bite of infected Hyalomma ticks, but also through contact with tissues and body fluids of infected animals (Paragas et al. 2004; Mardani and Keshtkar-Jahromi 2007). Although the clinical manifestations of CCHF are well described in adults and young children, there are very limited data about the clinical and laboratory course in the newborn.

Case report A 23-year-old pregnant woman was referred at 36 weeks’ gestation with complaints of fever, epistaxis, gingival bleeding and

back, abdominal and leg pain that had started 7 days earlier. Initial laboratory investigations revealed thrombocytopenia and increased prothrombin time (PT) and activated partial thromboplastin time (aPTT). CCHF disease was confirmed by detection of viral RNA in a reverse transcriptase-polymerase chain reaction (RT-PCR) test. She was treated with fresh frozen plasma, thrombocyte and erythrocyte infusions and with ribavirin (2 g initial loading dose, then 1 g every 6 h) which was commenced 2 days before delivery. On the 3rd day after admission, a female infant was born, by spontaneous vaginal delivery; birth weight of 2,260 g (10th percentile), length 48 cm (50th percentile), with Apgar scores of 8 and 10, at 1 and 5 min, respectively. Physical examination was unremarkable. One hour after birth, a complete blood count showed: haemoglobin: 15.8 g/dl (reference range: 16.5 ⫾ 1.5); platelets: 216,000/mm3 (290,000 ⫾ 100,000), as well as mildly increased lactate dehydrogenase at 936 IU/l (170–580). Coagulopathy was evident: PT 56.6 s (13.0 ⫾ 1.43) and aPTT 82.9 s (42.9 ⫾ 5.8). FFP (10 ml/kg) was transfused. After 12 hours, the child’s coagulation parameters returned to normal (PT: 14 s, aPTT: 32 s). On the second day, PT was 12.4 s and aPTT 29.8 s. Ribavirin was initiated with a loading dose of 30 mg/ kg, followed by 15 mg/kg every 6 h for 4 days, then 7.5 mg/kg every 6 h for 6 days. A blood sample for detection of CCHF was obtained on the day of birth. Viral RNA by RT-PCR and immunoglobulin M antibody by enzyme immunoassay were negative, suggesting that the child had not been infected in utero. During follow-up for 3 weeks, the child’s laboratory and clinical findings were completely normal. A maternal RT-PCR test repeated after ribavarin treatment was also negative.

Discussion In the literature, fetal effects of maternal CCHF acquired at any time during pregnancy, is very limited. The first case was born from a mother diagnosed with CCHF with RT-PCR at 38th weeks’ gestation. The PCR for CCHF was positive and oral ribavirin was administered after delivery. On the 5th day of birth, the baby died because of massive bleeding. Another baby, whose mother was diagnosed with CCHF at 19 weeks’ gestation with positive PCR and IgM, was also reported. Amniotic fluid CCHFV-PCR was found to be negative. After vaginal delivery, the baby was severely ill and had necrotising enterocolitis. His laboratory findings were normal except the high white blood cell count. On the 5th day, thrombocytopenia occurred and he died because of massive bleeding. His CCHF IgM and PCR were negative (Celikbas et al. 2006). Our baby was born from a mother infected with CCHF at 36 weeks’ gestation. Mildly increased LDH and significantly increased PT and activated PTT were thought to be a sign for CCHF and ribavirin treatment was initiated (Money 2003). Recent studies suggest that ribavirin is effective against CCHF in adults, although definitive studies are not available. Lower fatality rate was reported in ribavirin group and early administration of ribavirin for all suspected cases of CCHF was recommended. Also, prognosis is better for younger patients (Izadi and Salehi 2009). Studies concerning ribavirin treatment during the newborn period reveal that ribavirin is at least an effective and safe agent (Chávez-Bueno et al. 2007). For CCHF, there is only one newborn in the literature given ribavirin treatment who died (Celikbas et al. 2006). Actually, whether the elevations in the PT and PTT as well as the LDH levels were signs of a very mild disease resulting from ribavirin treatment that is immunologically undetectable or the newborn was uninfected, remains unclear. Both of these scenarios are unique and have not been reported in the literature before. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

References Celikbas A, Ergonul O, Yildirim U et al. 2006. Intrauterine infection of Crimean-Congo haemorrhagic fever: the courses of two episodes. 16th European Congress of Clinical Microbiology and Infectious Diseases, Nice, France, 1–4 April.

Spontaneous septostomy in monochorionic diamniotic twins: difficult diagnosis, difficult management.

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