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Obstetric Case Reports

If severe, e.g. ectopic vesicae, a separate corrective procedure is required. During the 7th week of embryo development, the cloacal membranes separate, creating the anal opening for the hindgut and ventral opening for the urogenital sinus with the perineal body forming between the two. The posterior aspect closes with the ectoderm and recanalises 2 weeks later. Development of imperforate anus is caused by a lack of recanalisation during the 9th week (Herman and Teitelbaum 2012). Concurrent ectopic positioning of the anal opening results in a fistula. Imperforate anus is commonly categorised into ‘high’ and ‘low’ lesions, depending on whether the rectum passed through the levator muscle (Waters 1982). Low lesions are less severe and may be repaired in a single stage procedure without the need for a colostomy. Our patient had a high lesion without fistula. The absence of a fistula predisposes to bowel obstruction and therefore a diverting colostomy was performed early in life. PSARP was first described by DeVries and Peña in 1982 for the repair of high anorectal malformations. A median sagittal incision from the sacrum to the anal dimple allows generous exposure of the anorectal region. The superficial and deep layers of the external sphincter are identified by electrostimulation and split in the midline. The dorsal and ventral portions of the levators are divided to allow the terminal bowel through. The bowel is frequently ectatic and needs to be tailored prior to reconstruction of the sphincter. The rectum and vagina may also be closely joined and share a common wall. This requires careful dissection and mobilisation of the terminal bowel. An anal canal is constructed and the bowel mucosa is sutured to the skin to create the anal opening. There is a paucity of evidence regarding the best mode of delivery for such patients. Breech (2010) suggested that patients who have had a simple repair of low imperforate anus are candidates for vaginal delivery, provided the perineal body is adequate (at least 3 cm; www.bardmedical.com/POPQ). Patients with repair of cloacal anomaly (confluence of urinary tract, vagina and rectum), extensive surgical repair, a neovagina or interposition graft should be delivered abdominally. A consultant should be present, as there is a risk of operative difficulty due to adhesions, distorted anatomy and potential risk of injury to bowel and bladder. The patient should be counselled appropriately. An increasing number of women who have undergone such corrective surgery will present to the obstetrician in pregnancy. Early consultant obstetrician involvement is crucial to make a definitive care plan for such patients. A full history and clinical assessment of continent status, strength of pelvic floor and the size and extent of scarring of the perineal body will assist in deciding the best mode of delivery. Details of the type of lesion (high or low) and corrective surgery(ies) performed should be obtained from the patient’s GP or paediatric hospital. A colorectal opinion may be sought, as in this case.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

References Breech L. 2010. Gynecologic concerns in patients with anorectal malformations. Seminars in Pediatric Surgery 19:139–145. DeVries PA, Peña A. 1982. Posterior sagittal anorectoplasty. Journal of Pediatric Surgery 17:638–643, 796–811. Herman RS, Teitelbaum DH. 2012. Anorectal malformations. Clinics in Perinatology 39:403–422. Peña A, DeVries PA. 1982. Posterior sagittal anorectoplasty: important technical considerations and new applications. Journal of Pediatric Surgery 17:796–811. Waters EG. 1982. Cloacal dysgenesis related anomalies and pregnancies. Obstetrics and Gynaecology 59:398–402.

A case of acute aortic dissection in a woman with Marfan syndrome at 29 weeks’ gestation A. Sato1, M. Ishida2, K. Ishibashi3, H. Shirasawa1, S. Akahira1, H. Miura1, M. Sato1, J. Kumagai1 & Y. Terada1 Departments of 1Obstetrics and Gynecology, 2Division of Cardiovascular Medicine, Department of Internal Medicine and 3Cardiovascular Surgery, Akita University School of Medicine, Akita, Japan DOI: 10.3109/01443615.2013.872608 Correspondence: A. Sato, Department of Obstetrics and Gynecology, Akita University School of Medicine, Akita 0108543, Japan. E-mail: [email protected]. akita-u.ac.jp

Case report Pregnancy complicated by Marfan syndrome confers a high risk of acute aortic dissection. Surgical management of acute aortic dissection in pregnancy has been reported, but the decision-making strategy regarding acute onset is often difficult for the individual case (Kats et al. 1984; Zeebregts et al. 1997; Immer et al. 2003; Haas et al. 2011). A 30-year-old female, gravid 4, para 0, was introduced to our department at 13 weeks’ gestation for management of pregnancy and delivery complicated by Marfan syndrome. She was diagnosed with Marfan syndrome at age 6 years and had undergone vertebral fixation for dorsal scoliosis at age 12 and mitral valve annuloplasty for mitral valve reflux at age 17. Evaluation of the aorta was performed mainly by ultrasonography, as MRI was not possible due to metal implants inserted during previous surgery for dorsal scoliosis. Ultra sonographic evaluation of cardiac activity was uneventful, and the aortic root size, which is a prognostic factor for acute aortic dissection, was 40 mm. Counselling regarding maternal pregnancy risks was provided to the patient and her family at 20 weeks’ gestation and resulted in the continuation of pregnancy. She was given 25 mg/day of atenolol and remained under close observation, with obstetric examinations every 2 weeks and a monthly echocardiography. Her blood pressure was maintained in normal range and was uneventful. She experienced sudden onset of acute severe chest pain at 29 weeks’ gestation, and presented to the emergency room. Echocardio graphy and sequential computed tomography confirmed the presence of acute type A aortic dissection, which extended from the aortic arch to the descending aorta and the bilateral common iliac artery. Taking into consideration the absence of cardiac tamponade and the lack of ongoing vascular occlusive ischaemic damage of multiple organs, temporary conservative therapy was agreed upon. The patient developed acute pneumonia. After its resolution, a caesarean section was performed under general anaesthesia at 30 weeks’ gestation. A female 1,497 g infant was delivered with Apgar scores of 4 at 1 min and 8 at 5 min. Hysterectomy was performed at the same time in order to prevent uterine bleeding due to anti coagulants in the extracorporeal circulation during aorta replacement. The acute pneumonia worsened after the first operation, and aortic repair therefore had to be postponed. After caesarean hysterectomy on the 8th day, once the acute pneumonia had resolved, the aortic root and arch replacements were performed using extracorporeal circulation. The acute pneumonia worsened again after the second operation and was treated with antibiotic agents for 1 month. Fever of unknown origin persisted for this length of time. The patient left the hospital 2 months after the onset of aortic dissection. She and her child were uneventful after a year.

Discussion The risk of acute aortic dissection associated with Marfan syndrome is known to increase during pregnancy. The frequency of aortic

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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.

A case of acute aortic dissection in a woman with Marfan syndrome at 29 weeks' gestation.

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