Frans Klumper

Twin Anemia-Polycythemia Sequence: Diagnostic Criteria, Classification, Perinatal Management and Outcome

Monochorionic twins share a single placenta with intertwin vascular anastomoses, allowing the transfer of blood from one fetus to the other and vice versa. These anastomoses are the essential anatomical substrate for the development of several complications, including twin-twin transfusion syndrome (TTTS) and twin anemia-polycythemia sequence (TAPS). TTTS and TAPS are both chronic forms of fetofetal transfusion. TTTS is characterized by the twin oligopolyhydramnios sequence, whereas TAPS is characterized by large intertwin hemoglobin differences in the absence of amniotic fluid discordances. TAPS may occur spontaneously in up to 5% of monochorionic twins and may also develop after incomplete laser treatment in TTTS cases. This review focuses on the pathogenesis, incidence, diagnostic criteria, management options and outcome in TAPS. In addition, we propose a classification system for antenatal and postnatal TAPS.

Fetoscopic Laser Surgery in 100 Pregnancies with Severe Twin-to-Twin Transfusion Syndrome in the Netherlands

Objective: In this prospective cohort study, we evaluated the initial results of fetoscopic laser surgery for severe second trimester twin-to-twin transfusion syndrome (TTTS) treated at our centre. Method: A total of 100 consecutive pregnancies with severe second trimester TTTS treated at our centre with selective fetoscopic laser coagulation of vascular anastomoses on the placental surface between August 2000 and November 2004 were included in the study. Perinatal survival was analysed in relation to Quintero stage. Results: Median gestational age was 20 weeks at fetoscopy (range: 16–26) and 33 weeks at delivery (range: 18–40). Perinatal survival rate was 70% (139/200). The treatment resulted in at least one survivor at the age of 4 weeks in 81% of pregnancies. Perinatal survival was significantly higher when treatment was performed in the early Quintero stages (95% in stage 1, 76% in stage 2, 70% in stage 3, 50% in stage 4) (p = 0.02). Conclusion: Results of fetoscopic laser surgery for TTTS in our centre are similar to those in specialised centres in other countries. Diagnosis and treatment in the early Quintero stages resulted in significantly higher perinatal survival.

Benefits and risks of fetal red-cell transfusion after 32 weeks gestation

OBJECTIVE To compare the outcome after intrauterine transfusion (IUT) between fetuses treated before and those treated after 32 weeks gestation. SETTING National referral center for intrauterine treatment of red-cell alloimmunization in The Netherlands. STUDY DESIGN Retrospective evaluation of an 11 year period, during which 209 fetuses were treated for alloimmune hemolytic disease with 609 red-cell IUTs. We compared fetal and neonatal outcome in three groups: fetuses only treated before 32 weeks gestation (group A, n=46), those treated both before and after 32 weeks (group B, n=117), and those where IUT was started at or after 32 weeks (group C, n=46). RESULTS Survival rate was 48% in group A, 100% in group B, and 91% in group C. Moreover, fetuses in group A were hydropic significantly more often. Short-term perinatal loss rate after IUT was 3.4% in the 409 procedures performed before 32 weeks and 1.0% in the 200 procedures performed after 32 weeks gestation. CONCLUSION Perinatal losses were much more common in fetuses only treated before 32 weeks gestation. Two procedure-related perinatal losses in 200 IUT after 32 weeks remain a matter of concern because of the good prospects of alternative extrauterine treatment.

Non-invasive tests to predict fetal anemia in Kell-alloimmunized pregnancies

To compare test characteristics of ultrasound and Doppler parameters in the prediction of fetal anemia in Kell‐alloimmunized pregnancies.

Successful ultrasound‐guided laser treatment of fetal hydrops caused by pulmonary sequestration

Microcystic or solid‐looking fetal lung lesions associated with hydrops are associated with a high mortality rate. The most common of these lesions are microcystic congenital cystic adenomatoid malformations and pulmonary sequestrations (PS). Performing open fetal surgery by hysterotomy and lobectomy has, until now, been the only therapeutic option available following diagnosis of these lesions in the second trimester. Here we describe a new minimally invasive technique for the treatment of PS in the second trimester. Laser coagulation of the feeding systemic artery of the sequestration was performed under ultrasound guidance. The blood flow to the lung tumor was successfully arrested and hydrops reversed. The child was born at term and, at the time of writing, was alive and well at 2 years of age.

Short- and long-term outcome in stage 1 twin-to-twin transfusion syndrome treated with laser surgery compared with conservative management.

OBJECTIVE We sought to compare short- and long-term outcome in Quintero stage 1 twin-to-twin transfusion syndrome (TTTS), managed with laser surgery or conservatively. STUDY DESIGN We conducted a retrospective study of all monochorionic twin pregnancies with stage 1 TTTS referred to our center. Primary outcomes were perinatal survival, neonatal morbidity, and long-term neurodevelopmental outcome. RESULTS Fifty women presented with stage 1 TTTS of which 40% (20/50) was treated with laser and 60% (30/50) was managed conservatively. Perinatal survival of both or at least 1 twin was 65% (13/20) and 85% (17/20) in the laser group, and 77% (23/30) and 97% (29/30) in the conservatively managed group (P = .52 and P = .29), respectively. Long-term neurodevelopmental impairment of the surviving infants was found in 0% (0/21) vs 23% (7/30), respectively (P = .03). CONCLUSION In this retrospective study, long-term outcome in stage 1 TTTS was better after laser surgery than with conservative management, suggesting the need for a randomized controlled trial.

Management of Twin Anemia-Polycythemia Sequence Using Intrauterine Blood Transfusion for the Donor and Partial Exchange Transfusion for the Recipient

Twin anemia-polycythemia sequence (TAPS) is a rare condition which may occur either spontaneously in uncomplicated monochorionic twin pregnancies or may develop after laser treatment in twin-twin transfusion syndrome. TAPS is characterized by a large intertwin discordance in hemoglobin levels without discordance in amniotic fluid levels, and may lead to severe complications including fetal hydrops, hematological morbidity and perinatal mortality. Several treatments have been proposed including intrauterine transfusion, laser surgery, elective delivery and expectant management. The optimal treatment remains unclear. In this case series we report 3 TAPS cases managed recently at our center with a combination of intrauterine blood transfusion for the anemic twin and intrauterine partial exchange transfusion for the polycythemic twin. In 1 case, the donor was found to have severe cerebral injury on neuroimaging examination. We propose etiologic mechanisms for cerebral injury in TAPS, discuss the rationale behind this treatment alternative, and evaluate the pros and cons of the various management options.

Obstetric history and antibody titer in estimating severity of Kell alloimmunization in pregnancy.

OBJECTIVE: To evaluate the usefulness of the obstetric history and the maternal serum Kell antibody titer in the management of pregnancies with Kell alloimmunization. METHODS: In a retrospective cohort study of 41 pregnancies complicated by Kell alloimmunization, the obstetric history, divided into presence or absence of a previous Kell-positive child, and Kell antibody titers in the index pregnancy were correlated with the gestational age at the onset of fetal anemia. RESULTS: Women with a previous Kell-positive child had a lower gestational age at the first intrauterine transfusion compared with those without a previous Kell-positive child (P=.01). However, in two of 29 pregnancies in the latter group, severe fetal anemia requiring transfusion was detected before 20 weeks of gestation. In neither group were maternal Kell antibody titers significantly correlated with gestational age at first intrauterine transfusion (P=.62 and P=.72, respectively). In all but two pregnancies (1:2 and 1:4, respectively), antibody titers were at least 1:32 before the first intrauterine transfusion. CONCLUSION: For timely detection of all cases of severe fetal anemia, Kell-alloimmunized pregnancies with a Kell-positive fetus and titers greater than or equal to 1:2 should be closely monitored from 16 to 17 weeks of gestation onward. LEVEL OF EVIDENCE: II

Twenty-Five Years of Fetoscopic Laser Coagulation in Twin-Twin Transfusion Syndrome: A Systematic Review

Objective: The aim of this study was to assess the perinatal outcome of pregnancies with twin-twin transfusion syndrome (TTTS) treated with laser therapy over the past 25 years, and in relation to different techniques used in this time period. Methods: A systematic review of studies reporting on perinatal outcome according to the Meta-Analysis of Observational Studies in Epidemiology (MOOSE) guidelines was conducted. The MEDLINE, Embase and Cochrane Library databases were systematically searched. Comparisons were made in respect to time period and laser technique and Quintero stages. Results: In total, 34 studies reporting on 3,868 monochorionic twin pregnancies were included. The mean survival of both twins increased from 35 to 65% (p = 0.012) and for at least one twin from 70 to 88% (p = 0.009) over the past 25 years. Mean gestational age at birth remained stable over the years at 32 weeks gestation. Also, we showed a significantly improved perinatal survival with the evolution of the laser technique from non-selective to selective, selective sequential and the Solomon technique (p = 0.010). Discussion: Since the introduction of laser therapy for TTTS more than two decades ago, perinatal survival improved significantly. Improved outcome is probably associated with several factors, including evolution of the laser technique, learning curve effect, better referral and improved early neonatal care.

Perinatal outcome after selective feticide in monochorionic twin pregnancies

To evaluate the incidence and risk factors of adverse perinatal outcome in complicated monochorionic twin pregnancies treated with selective feticide.