Identification of essential steps in laser procedure for twin-twin transfusion syndrome using the Delphi methodology: SILICONE study.

OBJECTIVE: To determine, by expert consensus, the essential substeps of fetoscopic laser surgery (FLS) for twin-twin transfusion syndrome (TTTS) that could be used to create an authority-based curriculum for training in this procedure among fetal medicine specialists. METHODS: A Delphi survey was conducted among an international panel of experts (n = 98) in FLS. Experts rated the substeps of FLS on a five-point Likert-type scale to indicate whether they considered them to be essential, and were able to comment on each substep, using a dedicated online platform accessed by the invited tertiary care facilities that specialize in fetal therapy. Responses were returned to the panel until consensus was reached (Cronbach's α ≥ 0.80). All substeps that were rated ≥ 4 by 80% of the experts were included in the evaluation instrument. RESULTS: After the first iteration of the Delphi procedure, a response rate of 74% (73/98) was reached, and in the second and third iterations response rates of 90% (66/73) and 81% (59/73) were reached, respectively. Among a total of 81 substeps rated in the first round, 21 substeps had to be re-rated in the second round. Finally, from the initial list of substeps, 55 were agreed by experts to be essential. In the third round, the 18 categorized substeps were ranked in order of importance, with 'coagulation of all anastomoses that cross the equator' and 'determination of fetoscope insertion site' as the most important. CONCLUSIONS: A total of 55 substeps of FLS for TTTS were defined by a panel of experts to be essential in the procedure. This list is the first authority-based evidence to be used in the development of a final training model for future fetal surgeons.

Congenital lobar emphysema--the disappearing chest mass: antenatal ultrasound appearance.

Prenatal diagnosis of congenital lobar emphysema is rarely reported in the literature. This case presents a fetus with an echogenic lung diagnosed at 18 weeks' gestation, that resolved completely during the pregnancy. This case is unique in that sonographic increased echogenicity was first noted at 18 weeks' gestation and had disappeared by 29 weeks' gestation. The diagnosis was made in the postnatal period as a result of follow up of the prenatal findings. This case reinforces the importance of continuing postnatal investigations of prenatal sonographic abnormalities even when they appear to have disappeared. A discussion of the causes of congenital lobar emphysema is presented.

Observational study of effect of intrauterine transfusions on outcome of fetal hydrops after parvovirus B19 infection.

The role of intrauterine transfusion for fetal hydrops arising from maternal paravirus B19 infection is unclear. 66 cases of fetal hydrops arising from B19 infection were reported in England and Wales between June, 1992, and September, 1994. In 29 cases the fetus was dead at the time of the first abnormal ultrasound or a therapeutic abortion as performed; 12 of the 38 alive at the first abnormal scan received intrauterine transfusions and 3 of the 12 died. 26 did not receive intrauterine transfusions and 13 died. After adjustment for the severity of the hydrops as assessed by the ultrasound and for gestational age, the odds of death among those who received an intrauterine transfusion was significantly less than among those who did not (odds ratio 0.14, 95% CI 0.02-0.96). These findings suggest that intrauterine transfusion will benefit some fetuses with hydrops arising from parvovirus B19 infection.

Gastro-intestinal crises during pregnancy.

In general pregnancy is not associated with an increase in the incidence of gastro-intestinal (GI) conditions, but it is associated with an increase in the severity of these conditions. This is largely due to a delay in making the diagnosis. Delay is caused by a combination of factors. These include mimicry of symptoms and signs with pregnancy-related conditions, a change in the usual clinical presentation, reluctance to use radiological diagnostic aids and a higher threshold for performing invasive investigative procedures. However before resorting to invasive investigations, it is imperative that appropriate non-invasive investigations are performed and evaluated first. This is especially relevant to liver conditions in pregnancy which can present with abdominal pain. Optimal management of GI conditions in pregnancy requires both surgeons and obstetricians to be aware of the problems of diagnosis, communicate promptly and investigate without delay in order to make a diagnosis and avert a GI crisis. This article discusses the clinical presentation, diagnosis and management of the common GI conditions occurring in pregnancy.

Complex mosaicism associated with trisomy 9.

Fetal karyotypes can be routinely obtained by chorionic villus biopsy, amniocentesis, or fetal blood sampling. Interpretation of results and subsequent counselling can be complicated by pseudomosaicism or mosaicism confined to the placenta or other tissues. We illustrate this by reporting a case of an abnormal fetus with a total of three karyotypically different cell lines (46,XX; 47,XX, +9; and 47,XX, +del(9) (q11) in different tissues (placenta, lung, gonad, and skin).

Comprehensive fetal assessment with three ultrasonographic characteristics.

OBJECTIVES: When three ultrasonographic characteristics--umbilical artery Doppler recording, growth (abdominal circumference), and biophysical profile score--are used in combination in assessment of fetuses at risk of chronic asphyxia: (1) What are the order and time scale for the development of abnormality with each characteristic? (2) What is the short-term outcome associated with abnormalities of the three characteristics? (3) Should we amend our fetal assessment protocol? STUDY DESIGN: An audit of 103 fetuses (100 mothers) referred to a tertiary center for fetal assessment because of suspected chronic fetal asphyxia was performed with three ultrasonographic characteristics, umbilical artery Doppler recording, measurement of abdominal circumference, and documenting the biophysical profile score. RESULTS: The order of deterioration (which had a very variable time scale) was umbilical artery Doppler recording, followed by abdominal circumference and finally biophysical profile score. Normal characteristics or an abnormal umbilical artery Doppler recording alone or an abnormal abdominal circumference alone was associated with an excellent prognosis. The worst outcome was found in the 28 fetuses with abnormality of all three ultrasonographic features before delivery. CONCLUSIONS: The main suggested implications for management are avoidance of preterm delivery with normal ultrasonographic characteristics, an abnormal umbilical artery Doppler recording alone, or an abnormal abdominal circumference alone; delivery of fetuses at greater than or equal to 34 weeks with abnormal umbilical artery Doppler recording and abdominal circumference before the biophysical profile score becomes abnormal; and implementation of specific measures to prevent necrotizing enterocolitis in newborns when all three characteristics are abnormal.

Intermittent fetal tachycardia and fetal hydrops.

A case is reported where fetal hydrops was noted 10 days after an initial observation of intermittent fetal tachycardia at 31 weeks. A diagnosis of supraventricular tachycardia was made and a successful conversion to sinus rhythm was achieved with maternally administered flecainide, with subsequent resolution of the hydrops. The fetus required no further treatment in pregnancy or at follow up. The mother suffered no side effects of the treatment.