We report a case study of jejunal atresia and the results of a systematic literature review of all reported cases of bowel complications occurring after fetoscopic laser ablation (FLA) for the treatment of twin-to-twin transfusion syndrome (TTTS).

A systematic literature review was performed of bowel complications after FLA for TTTS according to PRISMA guidelines.

There are 11 published cases of small bowel atresia, 5 cases of necrotising enterocolitis (NEC), and 2 cases with foetal bowel perforations. Recipient twins were more likely to be affected by small bowel atresia (7 recipient and 4 donor cases) and NEC (3 recipient and 2 donor twins). Prenatal ultrasonographic abnormalities were demonstrated in 7 out of 9 cases with bowel atresia and in both cases of bowel perforation. The overall survival rate for neonates with bowel complications after FLA is 72%, but is much lower for co-twins at 22%. The survival rates for jejunoileal atresia and NEC are 91 and 40%, respectively.

It is uncertain as to whether these bowel anomalies are due to bowel ischaemia associated with TTTS, the treatment with FLA, or a combination of both. Cases with prenatal abdominal ultrasonographic abnormalities after FLA should have close prenatal and postnatal assessment to detect bowel complications.

Twin-to-twin transfusion syndrome typically occurs in the second trimester in 10-15% of monochorionic twin pregnancies. Vascular anastomoses of monochorionic placentae are the underlying cause of the development of the syndrome. If a blood flow imbalance occurs, one fetus becomes the so-called donor twin and the other the recipient. If untreated, perinatal mortality is 80-90%. Fetoscopic laser coagulation of the vascular anastomoses destroys the cause of the syndrome and leads to dual twin survival rates of around 70% and more than 90% of pregnancies with at least one survivor. However, unequal placental sharing, intrauterine death, and severe prematurity are still limiting factors for further improvement of survival rates and decreases in long-term morbidity. Prematurity and neurodevelopmental impairment affect the donor and recipient twins, whereas cardiovascular failure and obstruction of the right ventricular outflow tract are typical complications of recipients, which can lead to long-term morbidity. In this Review, we summarise the literature on follow-up data for survivors of twin-to-twin-transfusion syndrome after laser therapy, including neurodevelopmental outcomes, cardiovascular outcomes, growth, renal function, and ischaemic events, as well as the potential effects of intrauterine programming on later life.

Twin-to-twin transfusion syndrome (TTTS) is due to unbalanced inter-twin bloodflow through placental vascular anastomoses. We present a TTTS case treated with fetoscopic laser photocoagulation (FLP) that was complicated by perinatal meconium peritonitis in both twins. Ten weeks following laser treatment, the two fetuses showed intra-abdominal hyperechogenicity and ascites. After birth, the two newborns were surgically managed for peritonitis. We discuss the pathogenesis of this double insult. The present case highlights the role of end-circulation bowel thrombi as the potential cause of subsequent intestinal perforation.

The purpose of this study was to evaluate the phenomenon of vascular ischemic limb necrosis in twin-twin transfusion syndrome (TTTS).

This was a multicenter retrospective review of ischemic limb necrosis in patients with TTTS.

Twenty cases of fetal ischemic limb necrosis in association with TTTS were identified from 10 fetal medicine centers. The recipient was affected in 19 cases, and the lower limb was affected in 17 cases. The extent of the damage correlated with TTTS severity. Eighty percent of limb defects (16/20) clearly were unrelated to laser treatment (3 cases untreated, 7 cases after amnioreduction, 6 cases present at time of laser). The recipient was relatively polycythemic in 5 of 7 cases in which neonatal or fetal hemoglobin/hematocrit levels were available.

Ischemic limb necrosis is a rare complication of TTTS. The lesion is unrelated to therapy and may be the result of polycythemia, hypertension, and vasoconstriction.

Compared to singleton pregnancies, monochorionic twins have increased rates of perinatal morbidity and mortality, believed due in part to both twin-twin transfusion syndrome and an increased risk of congenital anomalies. Here we describe the prevalence of noncardiac structural anomalies in monochorionic twins with twin-twin transfusion syndrome who underwent laser surgery.

In a retrospective study of 221 consecutive cases of twin-twin transfusion syndrome treated with laser surgery, noncardiac anomalies were identified by review of antepartum and neonatal medical records.

Of 377 live-born twins, 19 (5.0%) had a noncardiac anomaly. This rate was increased for donor versus recipient twins (8.5% versus 2.0%; P < .01). The presence of an anomaly was unrelated to the Quintero stage, the presence of donor intrauterine growth restriction, or 30-day survival of the donor or recipient.

The prevalence of noncardiac anomalies in pregnancies complicated by twin-twin transfusion syndrome who underwent laser surgery was higher in donors versus recipients.

Twin-twin transfusion syndrome (TTTS) is a severe complication of monochorionic twin pregnancies associated with high perinatal mortality and morbidity rates. Management in TTTS is a major challenge for obstetricians and neonatologists. Twins which are often born prematurely and may suffer from typical conditions associated with prematurity. In addition, surviving twins with TTTS are at increased risk for other complications including neurological, cardiovascular, renal and hematologic morbidity. Rare complications such as hypoxic-ischemic lesions to limbs or intestines and amniotic band syndrome have also been reported in TTTS survivors. This review focuses on the neonatal and pediatric mortality and morbidity in TTTS survivors, with special emphasis on the long-term neurodevelopmental outcome.

Twin-to-twin transfusion syndrome (TTTS) occurs in 9% of monochorionic twin pregnancies. An imbalanced blood flow across placental vascular communications produces a hypovolemic condition in the donor fetus and hypervolemia in the recipient fetus, leading to a variety of postnatal complications. We report 3 cases of intestinal injury in TTTS after fetoscopic laser ablation of the communicating vessels: 2 cases of intestinal atresia, and 1 case of necrotizing enterocolitis of 1 twin. Intestinal ischemic diseases have been reported after prenatal laser treatment in TTTS; with this report, we add 3 more cases.

Ileal atresia is considered to be an acquired anomaly. It is thought to be caused by a mesenteric vascular accident during fetal life which leads to necrosis of downstream tissues. We present a case in which ileal atresia occurred after intrauterine laser coagulation therapy for a twin-to-twin transfusion syndrome. The laser treatment might have been the etiological factor of the atresia. A second case in which thrombo-embolic calcifications were found in the liver is presented as well.

The mechanisms of intrauterine vascular disruptions that result in the development of jejunoileal atresia (JIA) are not fully understood. Monochorionic twinning with fetal death of a cotwin is known to be correlated with the development of JIA in the survivor through placental communication. The aim of this study was to evaluate whether other placental vascular compromises might contribute to the development of JIA.

Forty-five newborns (23 boys and 22 girls) who were treated for JIA at Tsukuba University Hospital from 1978 to 2003 were reviewed. Placental findings were informative in 23 cases.

No or slight abnormality of the placenta was found in 19 cases. Significant placental abnormalities were found in 4 patients who also had a low birth weight. One patient with apple peel atresia (APA) had excessive torsion of the umbilical cord (UC), which was inserted at the margin of the placenta, and there was an adjacent area of infarction. One patent with multiple atresia (MA) was a surviving monochorionic twin with intrauterine fetal death of the other. Another case of MA showed marginal insertion of the UC. Severe placental abnormalities including wide infarction, cyst formation, and marginal insertion of the UC were found in 1 case of MA. These 3 cases of MA were complicated with other anomalies including brain anomaly.

Placental vascular compromises were involved infrequently in JIA but might possibly be responsible for the development of JIA as well as associated anomalies and a low birth weight as chronic insults since an early stage of gestation in some cases.

A case of twin-to-twin transfusion syndrome (TTTS) was associated with ischemic injury of the left leg of the recipient twin. Five reduction amniocenteses were performed between 17 6/7 and 23 3/7 weeks followed by fetoscopic laser ablation of chorioangiopagus vessels at 23 6/7 weeks of gestation. Anatomic assessment of the affected limb indicated timing of the injury at 19 4/7 weeks gestational age. The published literature suggests that ischemic fetal injuries in severe TTTS are associated with blood hyperviscosity in the affected twin.

Monochorionic compared with dichorionic twins have disproportionately high fetal loss rates, perinatal mortality and morbidity. This is because of the unpredictable vascular anastomoses and the often asymmetrical distribution of the single placenta between both twins.

The pathophysiology of twin-to-twin transfusion syndrome is usually explained on an angioarchitectural basis, although certain hemodynamic and hormonal factors also may be involved. The results of the large randomized trials on amnioreduction, fetoscopic laser coagulation and septostomy are still awaited. An update is given on hardware and instruments required for fetoscopic laser. Subsequently, the problem of the monochorionic twin set with severe early discordant growth is addressed. Several etiological mechanisms have been proposed, but little is known of its natural history. Also, umbilical artery Doppler waveforms may not have the same predictive value as in singletons. Prophylactic laser coagulation of the vascular anastomoses to protect against the adverse effects of single intrauterine demise, has so far not been shown to confer any benefit in outcome. Finally, pathophysiology and management of discordant structural and chromosomal anomalies in monochorionic twins are discussed. Laser and monopolar coagulation, which can be introduced through a needle, may be used for selective feticide in early pregnancy or low hemodynamic conditions. Bipolar coagulation seems more effective at later gestational ages and normal hemodynamic conditions.

Our insight into the complications associated with monochorionic twins has increased in recent years. It is hoped that this will lead to better surveillance and ultimately an improved outcome for these high-risk pregnancies.