Dr Maher Maaita MRCOG Twin to twin transfusion syndrome (TTTS) Dr Maher Maaita MRCOG King Hussien Medical Centre

tre Brief History:

A 30 year-old women, parity 3, all by caesarean sections, referred to King Hussein Medical Centre at 29 weeks gestation with severe polyhydraminos. Ultrasound examination revealed Twin pregnancy. First twin in cephalic presentation with polyhydraminos, amniotic fluid index ( AFI ) of 30, Large bladder, growth measurements > 50 centile with normal Doppler studies. Second twin stuck to the uterine wall with anhydraminos, absent bladder, growth measurements below 5th centile with absent end

diastolic flow in the umbilical artery on Doppler studies. Diagnosis

The diagnosis of twin to twin transfusion syndrome (TTTS) grade 3 was made. Management

The patient was counselled regarding the potential poor prognosis. She was admitted to hospital and given Dexamethazone. The patient underwent 3 amnioreductions, draining 3,2,2 litres over a period of 10 days. At 30+ weeks, the patient ruptured her membranes and underwent an emergency caesarean section. The first baby weighed 1.5 kg and was born freshly dead. The second twin weighed 750 mg was born alive and was admitted to the neonatal unit and was put on the ventilator. Both babies were girls as shown in Fig 1. The patient made a good recovery and discharged home on day 3.

Figure 1: TTTS

Figure 2 : ( TTTS Babies in NICU)

Figure 3: Face profile showing scalp oedema

Figure 4: Recipient twin with oligohydramnios

Discussion It is important to scan all twins early and determine chorionicity before 14 weeks and identify this high risk group, for which increased monitoring may improve outcome. It is essential for genetic counselling, management for discordant anomalies and TTTS, fetal compromise and and intrauterine fetal death. It is a matter of counting the layers that separate the twins. If there are tow thin layers (two amniotic sacs) and two thick separate chorionic plates or one fused chorion (beyond 9 weeks) that forms the lambda at insertion of the placenta, then they are dichorionic diamniotic twins. However, if there are two thin layers (two amniotic sacs) inserting as a T on the placental disc, then they are monochorionic diamniotic twins. Our patient was unbooked at our hospital and presented late with stage (III) TTTS.

She and her husband were aware of the poor prognosis and the high mortality. We performed amnioreduction. Available data suggests that amnioreduction is only effective in mild cases of TTTS ( Stages I-II). Where as amnioreduction is a palliative and repetitive measure, fetoscopic laser coagulation of the vascular anastomoses seeks to address the underlying cause of the disease through a single intervention. A recent controlled study showed that laser is superior to amnioreduction in the treatment of TTTS. Unfortunately we do not have the facility to do laser coagulation at our centre. Monochorionic twins are high risk pregnancies and should be referred to a tertiary centre and followed closely by a fetal medicine specialist every 2 weeks from 16 weeks onward to look for complications and mainly to look for TTTS.

Twin to twin Transfusion Syndrome (Key Points)

Twin to twin transfusion (TTTS) is a complication unique to monochorionic multiple pregnancies. In most monochorionic twin gestations, interfetal transfusion across the anastomoses is a constant but balanced phenomenon.

However in 10% to 15% of monochorionic twins, a chronic imbalance in net flow develops, resulting in TTTS. Hypovolemia, oliguria and oligohydraminos develop in the donor twin producing the stuck twin phenomenon. Hypervolemia, polyuria, and hydraminos evolve in the recipient twin, who can develop circulatory overload and hydrops. TTTS usually occurs between 15 and 26 weeks. There is a staging system based on the sonographic time sequence of cases with progressive deterioration. Stage I cases include those with hydraminos in the recipient sac but the bladder of the donor twin still visible. In stage II, the bladder of the donor twin remains empty (stuck twin). Stage III is characterized by severely abnormal Doppler studies. Fetal hydrops means stage IV and the end stage V corresponds to fetal death of one or both twins. In view of the poor survival rates with conservative management, there is little disagreement that therapy should be offered.

The four most commonly used therapies for midtrimester TTTS are amnioreduction, fetoscopic laser coagulation of the vascular anastomoses, septostomy, and selective

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