Journal of Perinatology (2008) 28, 377–379 r 2008 Nature Publishing Group All rights reserved. 0743-8346/08 $30 www.nature.com/jp PERINATAL/NEONATAL CASE PRESENTATION Amniotic band syndrome following septostomy in management of twin–twin transfusion syndrome: a case report

J Rujiwetpongstorn1 and T Tongsong2 1Maternal-Fetal Medicine Unit, Department of Obstetrics and Gynecology, Faculty of Medicine, Srinakharinwirot University, Bangkok, Thailand and 2Department of Obstetrics and Gynecology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand

pregnancies.1 TTTS is commonly associated with various Septostomy, a rupture of the diamniotic membrane separating monozygotic complications and high perinatal mortality, particularly when the twins essentially creating a monoamniotic gestation, is a potential syndrome has an early onset. Several techniques have been used to therapeutic modality for twin–twin transfusion syndrome (TTTS). This improve the prognosis including serial amnioreduction,2,3 laser may be associated with complications including cord entanglement or coagulation of anastomotic vessels,4,5 selective fetocide6 and cord complete rupture of the membranes. We report a case of severe amniotic ligation when death of one fetus appears imminent.7 Septostomy, a band syndrome with cord amputation after septostomy. A 33-year-old rupture of the diamniotic membrane separating monozygotic twins woman with a Mo-Di twin pregnancy was diagnosed with TTTS at 18 weeks essentially creating a monoamniotic gestation, has also been of gestation. Septostomy as well as amnioreduction were performed at 24 reported as a potential therapeutic modality for TTTS.3,8 Some weeks of gestation. A repeat cesarean delivery was performed at 31 weeks complications of septostomy have been reported, including cord resulting in a live recipient baby of 1340 g and a dead donor with amniotic entanglement and complete rupture of membranes of both twins, band syndrome. The donor showed pieces of membrane tightening both but amniotic band syndrome (ABS) associated with septostomy has legs. The right thigh became entangled in the bands connecting to the never been reported. We report a case of severe ABS with limb umbilical cord of the live fetus. The umbilical cord of the dead twin was amputation after septostomy. completely amputated, whereas the umbilical cord of the live infant was also entrapped within the amniotic band resulting in small diameter and some degree of stricture. This is the first report of a rare but serious Case presentation complication following septostomy. A 33-year-old pregnant woman, gravida 2 para 1, attended an Journal of Perinatology (2007) 28, 377–379; doi:10.1038/sj.jp.7211927 antenatal clinic starting at 18 weeks of gestation. Screening Keywords: amniotic band syndrome; monochorionic diamniotic twins; ultrasound at the first visit showed a twin pregnancy with fetal prenatal diagnosis; septostomy; twin-twin transfusion syndrome; biometry consistent with a date of 18 weeks. Additionally, the ultrasound gestation was found to be monochorionic diamniotic with polyhydramnios with deepest amniotic fluid pocket of 8.8 cm in one sac and oligohydramnios with deepest amniotic fluid pocket of Introduction 2.8 cm in the other. The estimated fetal weight of both fetuses was Twin gestations account for 1 to 2.5% of all pregnancies and are at equal without any sign of hydrops fetalis. Detailed ultrasound a higher risk of adverse perinatal outcomes, in particular a examination revealed normal structures of both twins. Early TTTS monochorionic placenta that is seen in 20% of all twins.1 was suspected and serial ultrasound examinations were performed Twin–twin transfusion syndrome (TTTS) is one of the severe every 2 weeks. At 23 weeks of gestation, the patient was admitted complications in monochorionic twin pregnancies resulting from because of acute appendicitis and an uneventful appendectomy was an imbalance in the net flow of blood across the placental vascular performed. At 24 weeks of gestation, the patient complained of communications from one fetus, the donor, to the other, the abdominal discomfort and ultrasound revealed marked recipient. Severe TTTS may occur in about 15% of monochorionic polyhydramnios in one twin with some degree of discordancy but normal structures. Umbilical artery Doppler of the donor showed Correspondence: Dr J Rujiwetpongstorn, Maternal-Fetal Medicine Unit, Department of absent end-diastolic flow. Amnioreduction with septostomy was Obstetrics and Gynecology, Faculty of Medicine, Srinakharinwirot University, Sukhumvit 23, performed and corticosteroids for lung maturity were administered. Klong Toei, Bangkok 10110, Thailand. E-mail: [email protected] After the procedure, both fetuses had relatively slow growth rate; Received 1 August 2007; revised 12 November 2007; accepted 19 November 2007 however, the discordancy still existed. Amniotic band syndrome following septostomy J Rujiwetpongstorn and T Tongsong 378

At 30 weeks of gestation, a biophysical profile of the recipient was reassuring but the donor was found to be dead. The patient was admitted to the hospital due to preterm premature rupture of membrane. A second course of dexamethasone for fetal lung maturity as well as antibiotics to prolong latency period were administered. Basic laboratory studies were normal, with no signs of chorioamnionitis. At 31 weeks of gestation, leukocytosis, white blood cells of 14 840 mmÀ3 with predominant polymorphonuclear cells, suggestive of subclinical chorioamnionitis was encountered. Therefore, delivery was performed by repeat cesarean, resulting in a healthy female baby with a birth weight of 1340 g and a macerated dead female fetus. The dead twin showed pieces of membrane tightening both legs. The right thigh had become entangled in the bands connecting to the umbilical cord of the live fetus. The umbilical cord of the dead twin was completely amputated, whereas the umbilical cord of the Figure 1 Right thigh is entangled in the bands connecting to the umbilical cord live infant was entrapped with an amniotic band resulting in small of the live fetus. diameter and some degree of stricture. The placenta was normal in appearance. Postnatal findings of the dead twin are shown in Figures 1–3.

Discussion Septostomy using the intentional perforation of the interamniotic membrane to equalize the volume of amniotic fluid in cases of severe TTTS was first introduced by Saade et al.8 Septostomy interrupts the polyhydramnios–oligohydramnios mechanism and induces an amniotic fluid volume increase in the gestational sac of the ‘donor’ twin, which has been associated with an increase in the circulatory volume, as well as renal perfusion and urinary output. Similarly, septostomy also may benefit the ‘recipient’ fetus, because when the volume of amniotic fluid and hydrostatic pressure between the gestational sacs are normalized, the inflow of water from the maternal compartment is prevented.9 Septostomy can reduce the number of amnioreductions needed to correct the polyhydramnios.10 Figure 2 The umbilical cord of the dead twin is completely amputated. Septostomy is minimally invasive, easy to perform, cheap and can be carried out in the same setting of amnioreduction for treatment of TTTS. Technically, a needle is introduced into the inter-twin membrane to make a hole under ultrasound guidance. Although some studies have shown the relative safety of septostomy and the low morbidity rates,11–13 Moise et al.12 mentioned one case of complete rupture of the interamniotic membrane in a case treated with septostomy. Theoretically, the procedure-related risks may be the same as that of amnioreduction including preterm labor, preterm premature rupture of membrane, cord entanglement and the most serious complication, shredded membrane flaps, which possibly lead to ABS. Inadvertent iatrogenic septostomy of the dividing membrane in monochorionic diamniotic twins has been reported to be associated with cord entanglement.14 However, ABS secondary to septostomy has never been reported. The case presented here is the first case implying a serious complication of septostomy. Figure 3 The umbilical cord of the dead twin is completely amputated.

Journal of Perinatology Amniotic band syndrome following septostomy J Rujiwetpongstorn and T Tongsong 379

Amniotic band syndrome is a destructive fetal complex caused References by the disruption of the amnion followed by entanglement of the 1 Jain V, Fisk NM. The twin–twin transfusion syndrome. Clin Obstet Gynecol 2004; 47: fetal parts in the amniotic bands resulting in bizarre and 181–202. asymmetrical defects that can involve several organs, especially 2 Hubinont C, Bernard P, Pirot N, Biard J, Donnez J. Twin-to-twin transfusion syndrome: limbs, cranium or spine.15 At present, we consider that defective treatment by amniodrainage and septostomy. Eur J Obstet Gynecol Reprod Biol 2000; neo-angiogenesis results in the disruption of vascular supply and 92: 141–144. 3 Johnson JR, Rossi KQ, O’Shaughnessy RW. Amnioreduction versus septostomy in internal organ dysfunction.16,17 Typically, ABS is associated with twin–twin transfusion syndrome. Am J Obstet Gynecol 2001; 185: 1044–1047. rupture of the amnion either due to spontaneous rupture or 4 Gray PH, Cincotta R, Chan FY, Soong B. Perinatal outcomes with laser surgery for possible iatrogenic septostomy. Rupture of the amnion can lead to twin–twin transfusion syndrome. Twin Res Hum Genet 2006; 9: 438–443. entrapment of fetal structures by sticky mesodermic bands that 5 Huber A, Diehl W, Bregenzer T, Hackeloer BJ, Hecher K. Stage-related outcome in originate from the chorionic side of the amnion, followed by twin–twin transfusion syndrome treated by fetoscopic laser coagulation. Obstet disruption.15 Entrapment of fetal parts may cause amputation Gynecol 2006; 108: 333–337. 6 Chang YL, Chao AS, Hsu JJ, Chang SD, Soong YK. Selective fetocide reversed mirror or slash defects in random sites, unrelated to embryologic syndrome in a dichorionic triplet pregnancy with severe twin–twin transfusion development. The estimated date of insult ranges from 8 to 18 syndrome: a case report. Fetal Diagn Ther 2007; 22: 428–430. 18 weeks after the last menstrual period. However, the case 7 Taylor MJ, Shalev E, Tanawattanacharoen S, Jolly M, Kumar S, Weiner E et al. presented here is an evidence of late occurring destruction never Ultrasound-guided umbilical cord occlusion using bipolar diathermy for stage III/IV described elsewhere. This may give an insight about the twin–twin transfusion syndrome. Prenat Diagn 2002; 22: 70–76. pathogenesis of ABS. 8 Saade GR, Belfort MA, Berry DL, Bui TH, Montgomery LD, Johnson A et al. Amniotic septostomy for the treatment of twin oligohydramnios-polyhydramnios sequence. Fetal The evidence of amniotic band that tightened the thighs, Diagn Ther 1998; 13: 86–93. amputation of umbilical cord of the dead fetus and pieces of 9 Adegbite AL, Ward SB, Bajoria R. Perinatal outcome following amniotic septostomy in amniotic membrane entrapped the umbilical cord of live fetus chronic TTTS is independent of placental angioarchitecture. J Perinatol 2003; 23: suggested a unique complication of septostomy, ABS. 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