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Incidence and impact of perioperative complications in 175 fetoscopy-guided laser coagulations of chorionic plate anastomoses in fetofetal transfusion syndrome before 26 weeks of gestation.

OBJECTIVE: The purpose of this study was to evaluate the perioperative complications in fetoscopy-guided laser therapy in fetofetal transfusion syndrome. STUDY DESIGN: Details of fetofetal transfusion syndrome cases who were delivered between 1999 and 2004 in a single center were reviewed retrospectively. RESULTS: One hundred seventy-five procedures were performed percutaneously with the use of local anesthesia. Survival at 6 months of at least 1, 1, and 2 babies was 73%, 38%, and 35%, respectively. Placental abruption and miscarriage was diagnosed in 3 and 12 cases, respectively. Premature rupture of membranes occurred in 49 cases (28%) and including 12, 29, and 46 cases that occurred before 24, 28, and 34 weeks of gestation, respectively. The entry of the trocar was transplacental in 48 cases (27%), but it was not associated with miscarriage (P = .26), premature rupture of membranes (P = .58), or placental abruption (P = .37). CONCLUSION: Fetoscopic laser treatment of fetofetal transfusion syndrome can be performed percutaneously with local anesthesia without significant maternal morbidity. Transplacental entry was not associated with premature rupture of membranes or miscarriage.

Chorion↗

Prevalence and management of late fetal complications following successful selective laser coagulation of chorionic plate anastomoses in twin-to-twin transfusion syndrome.

OBJECTIVE: This study was undertaken to report on the prevalence and management of late complications in twin-to-twin transfusion syndrome (TTTS) treated by laser therapy when both twins are alive 1 week after surgery. STUDY DESIGN: A total of 151 consecutive TTTS cases were treated by selective fetoscopic laser therapy. Cases in which both twins were alive 1 week after surgery were followed up with ultrasound and Doppler examination, including middle-cerebral artery peak systolic velocity measurement (MCA-PSV). RESULTS: In the 151 cases treated with laser, both twins were still alive 7 days after the procedure in 101 cases. Intrauterine death of 1 and both twins occurred in 7 and 1 cases, respectively. Recurrence of TTTS with the polyhydramnios-oligohydramnios sequence occurred in 14 (14%) cases. In another 13 (13%) cases, amniotic fluid remained normal in both sacs, but MCA-PSV increased above 1.5 MoM in 1 twin and decreased concomitantly below 0.8 MoM in the co-twin. This was related to anemia and polycythemia, respectively, and resulted from unidirectional feto-fetal blood transfusion, mainly from former recipients into former donors. Late complications were managed accordingly by repeat laser, amnioreduction, cord coagulation, intrauterine blood transfusion, or elective delivery. CONCLUSION: MCA-PSV Doppler measurements are useful in the follow-up of double survivors to detect and manage late complications after selective laser therapy.

Anemia↗

Foetal surgery and cleft lip and palate: current status and new perspectives.

Now-a-days, high-resolution ultrasound allows an accurate and relatively early diagnosis of congenital malformations. In a limited number of such conditions foetal surgery may be lifesaving. However, premature labour has been the major drawback for open foetal surgery. Recently, improvement of video-endoscopic technology has boosted the development of operative techniques for feto-endoscopic surgery, which has been demonstrated to be less invasive than the open approach. Main clinical application of fetoscopic procedures today is the treatment of feto-foetal transfusion syndrome. Although still in development, feto-endoscopic surgery seems to offer new hope for surgical foetal therapy not only in cases of life threatening conditions. Experimental intrauterine correction of cleft lip and palate (CLP) has been lately performed using the feto-endoscopic approach. This procedure offers two major advantages: first, scarless foetal wound healing and bone healing without callus formation, which would also allow a better/normal maxillary growth, and second, significant decrease of foetal and maternal morbidity. Herein, we report the current status of experimental and clinical foetal surgery and propose possible directions for continuing research to make intrauterine procedures safer. Furthermore, we discuss current knowledge and new perspectives of experimental foetal cleft lip and palate repair, which in the future may lead to such excellent results in the operative treatment of clefts, that less or no secondary corrections and therapies, such as orthodontic, dental, logopedic, etc. would be needed. Only if these conditions can be fulfilled, will we be able to improve substantially our therapy for the human foetus with a cleft lip and palate. In spite of all efforts, however, it must be considered that it may not ever be possible to find the optimal treatment method for this or other craniofacial malformations.

Animals↗

Fetal endoscopic surgery: indications and anaesthetic management.

Fetal intervention for certain life-threatening conditions has progressed from being primarily experimental in nature to the standard of care in certain circumstances. While surgical techniques have advanced over the past few years, the anaesthetic goals for these interventions have remained the same; namely, minimizing maternal and fetal risk as well as maximizing the chances of a successful fetal intervention and optimize the conditions necessary to carry the fetus to term gestation. Fetal endoscopic techniques allow access to the fetus without the need for a hysterotomy incision, thus improving the chances of controlled post-operative tocolysis and term gestation after fetal intervention. This procedure, however, is not without associated risks to both fetus and mother. This chapter will address the fetal diseases that may benefit from fetoscopic intervention, the rationale behind why maternal and fetal anaesthesia is required, the various anaesthetics used for these cases and specific considerations of both maternal and fetal physiology that aid in the determination of the best anaesthetic technique for individual cases. Methods of intra-operative fetal monitoring and fetal resuscitation will also be discussed.

Analgesia↗

How can we diagnose and manage twin-twin transfusion syndrome?

Severe mid-trimester twin-twin transfusion syndrome (TTS) complicates about 15% of monochorionic twin pregnancies. If left untreated, the mortality is 80-100%. The pathophysiological prerequisite for the onset of TTS is unequal blood flow via arteriovenous placental anastomoses from the so-called donor to the recipient twin. This can result in hypovolemia, hypotension and oligo- or anuria in the donor, and hypervolemia, hypertension, polyuria and finally heart failure in the recipient. Leading sonographic signs of TTS include severe oligo- or anhydramnios and a small or absent bladder filling in the donor in contrast to polyhydramnios with increased bladder filling in the recipient. Patients might present with clinical symptoms due to massive polyhydramnios. In severe mid-trimester TTS, fetoscopic laser occlusion of the anastomosing vessels on the placental surface under local anaesthesia plus subsequent amniodrainage is the most promising therapeutic option at present. In acute TTS after 26 weeks of gestation, amniodrainage is the therapy of choice. All patients suspected of this high-risk condition should be referred to a specialized fetal medicine centre.

Amniotic Fluid↗

Management of other complications specific to monochorionic twin pregnancies.

Monochorionic (MC) twins have a 3-10-fold higher perinatal mortality and morbidity than dichorionic twins. This is largely attributable to their common vascular architecture and the high rate of discordant fetal growth, growth restriction and congenital abnormalities. In the event of a single intrauterine death (IUD), intertwin agonal transfusion results in up to a 38% risk of death and a 46% risk of neurological injury to the co-twin. This chapter addresses the management of complications unique to MC twins. The primary aim of management is to prevent single IUD or, if inevitable, prevent agonal transfusion occurring by vascular occlusive selective feticide. Older fetoscopic techniques have been replaced by the simpler ultrasound-guided techniques of interstitial laser and bipolar cord occlusion. Their application in twin reversed-arterial perfusion sequence has been associated with a 50% reduction of perinatal mortality in the pump twin. Moreover, prophylactic interstitial laser therapy in early pregnancy might obviate the technical and clinical difficulties in the presence of fetal decompensation in later pregnancy. Recent strategies to reduce the high perinatal mortality due to cord entanglement in antenatally diagnosed monoamniotic twins including medical amnioreduction and elective caesarean delivery at 32 weeks, are also discussed.

Chorion↗

Successful patching of iatrogenic rupture of the fetal membranes.

Rupture of the fetal membranes is a common, but potentially serious complication of invasive fetal procedures. Quintero described a technique to seal the fetal membrane defect by means of a bloodpatch, usually called 'amniopatch' in this application. The successful use in two consecutive patients with ruptured membranes after a fetoscopic intervention at respectively 17 and 22 weeks' gestational age is described, together with a literature review of published experience.

Adult↗

Pseudo-arterio-arterial anastomoses in twin-twin transfusion syndrome.

OBJECTIVE: It has recently been claimed that fetoscopic recognition of a haemodynamic equator within an arterio-arterial anastomosis (AAA) suggests minimal net intertwin flow. This was based on blood from one fetus being dark and from the other bright red, the boundary between them reciprocating with the fetal heart beats. However, bright red indicates that the blood had passed through a cotyledon and been freshly oxygenated, which should be impossible in an AAA. We applied a computer model of chorionic vessels to determine a configuration that reproduced this phenomenon. METHODS: A previously published TTTS model was extended to provide placental detail in a segment containing four cotyledons of each placenta supplied by three generations of placental arteries and veins. RESULTS: Reciprocating flow is not unique to AAAs. It also occurs in the chorionic arteries of any cotyledon deprived of its venous outflow, in a similar manner to that in which reverse end-diastolic flow occurs in umbilical arteries when whole placental resistance is high. If venous return from the common chorionic vein in the recipient (draining the venous end of an AVA) is blocked as might happen after laser, there can be bidirectional flow from one umbilical artery insertion, through two cotyledons to the other insertion. We define this phenomenon as a pseudo-AAA (PAAA). The inclusion of two cotyledons in this path means that its resistance cannot match the low flow resistance of a true AAA, and transmission of the contralateral pulsatile pattern is absorbed in the cotyledons. Thus, PAAA Doppler patterns differ from true AAA patterns in that two sets of systolic peaks, one forward and one reverse, can be discerned in true AAAs but only one in PAAAs. CONCLUSIONS: We demonstrate how venous occlusion of an arterio-venous anastomosis may produce a pseudo-AAA colour equator at endoscopy. However, visual observation of reciprocating flow is not sufficient to define a vessel as a true AAA which instead requires ultrasonical identification of two systolic patterns.

Adult↗

Assessment of feto-fetal transfusion flow through placental arterio-venous anastomoses in a unique case of twin-to-twin transfusion syndrome.

In vivo measurements of blood flow through arterio-venous anastomoses in monochorionic twin placentas have recently been attempted with Doppler ultrasound, but the accuracy is questionable. We present a case of twin-to-twin transfusion syndrome treated with fetoscopic laser surgery. The ex-recipient subsequently became severely anaemic and was treated with an intrauterine blood transfusion at 29 weeks' gestation. After birth, a placental injection study identified residual unidirectional arterio-venous anastomoses from the ex-recipient to the ex-donor without arterio-venous anastomoses in the opposite direction. Prospective measurements of decreasing haemoglobin levels between the intrauterine transfusion and birth allowed us to assess the net blood flow through the anastomoses as 27.9mL/24h. This finding may also explain the inaccuracy of Doppler flow measurements, as such low flow velocities cannot possibly be detected with current Doppler techniques.

Adult↗

Experimental fetal transesophageal and intracardiac echocardiography utilizing intravascular ultrasound technology.

Fetal transesophageal and intracardiac echocardiography by utilizing ultrasound technology permits accurate definition of cardiac anatomy in fetal sheep. Because fetal transesophageal echocardiography is less invasive than intracardiac echocardiography, it has the potential to serve as a monitoring tool for currently developed open and fetoscopic fetal cardiac interventions.

Animals↗

The relationship of fetal plasma protein concentration and hemoglobin level to the development of hydrops in rhesus isoimmunization.

Fetoscopic samples of pure fetal blood were obtained at 18 to 25 weeks' gestation from seven hydropic and 10 nonhydropic fetuses affected by rhesus isoimmunization, and the fetal plasma albumin, plasma total protein, and hemoglobin concentrations were determined. All fetuses with sonographic evidence of hydrops had a hemoglobin of 3.8 gm/dl or less, whereas all but one of those without hydrops had a hemoglobin greater than 4.0 gm/dl. The plasma total protein was less than 2 standard deviations below the mean of the normal range in all hydropic fetuses and in six of 10 of those without hydrops. Hypoalbuminemia was found in six of the seven hydropic fetuses and in two of the nonhydropic fetuses. Ascitic fluid total protein and albumin concentrations were also determined in four hydropic fetuses, and the values in three were found to be more than 50% of the corresponding plasma levels.

Albumins↗

Endoscopic laser surgery versus serial amniocenteses in the treatment of severe twin-twin transfusion syndrome.

OBJECTIVE: Severe twin-twin transfusion can be treated by either serial amniocenteses with removal of large volumes of amniotic fluid or by endoscopic laser coagulation of the communicating vessels. We investigated the benefit of laser surgery in comparison to serial amniocenteses in terms of pregnancy outcome. STUDY DESIGN: The data used in this comparative study were collected from 116 patients with severe twin-twin transfusion syndrome between 17 and 25 weeks' gestation. The patients were grouped according to the treatment center. The first group comprised 73 patients (median gestational age 20.7 weeks) treated in Hamburg by fetoscopic laser coagulation of the vascular placental anastomoses between January 1995 and May 1997. The second group comprised 43 patients (median gestational age 20.4 weeks), fulfilling identical diagnostic criteria and treated in Bonn by serial amniocenteses between 1992 and 1996. RESULTS: The overall fetal survival rate was not significantly different (61%, 89/146, vs 51%, 44/86; P =.239). There was a significantly higher proportion of pregnancies with >/=1 survivor in the laser-treated group (79%, 58/73, vs 60%, 26/43; P =.033). The number of cases with spontaneous intrauterine fetal death of both fetuses was significantly lower in the laser-treated group (3%, 2/73, vs 19%, 8/43; P =.003). The incidence of abnormal ultrasonographic findings in the brain was significantly lower among surviving neonates after laser surgery than after amniocenteses (6%, 5/89, vs 18%, 8/44; P =. 03). For pregnancies with >/=1 live-born baby, a significantly longer interval between first intervention and delivery (median 90 vs 72 days, P =.022) leading to a significantly higher gestational age at delivery (median 33.7 vs 30.7 weeks, P =.018) was observed for the laser-treated group. The birth weights of the donor fetuses were significantly higher in the laser-treated group (median 1750 vs 1145 g, P =.034), and a trend toward higher birth weight was also found for recipient fetuses (median 2000 vs 1560 g, P =.076). CONCLUSIONS: These findings indicate that endoscopic laser coagulation of placental vascular anastomoses offers a more effective alternative to serial amniocenteses as a treatment of severe second-trimester twin-twin transfusion syndrome.

Amniocentesis↗

Congenital pyloric atresia and epidermolysis bullosa letalis in premature siblings.

Epidermolysis bullosa letalis has been associated with congenital pyloric atresia in an autosomal recessive syndrome. This report documents the second occurrence in premature siblings. Our review indicates that it is a lethal condition with death due to the septic complications of epidermolysis bullosa letalis. Surgical management of pyloric atresia should be withheld until the severity of the epidermolysis is known. Skin biopsy to document the type of epidermolysis bullosa is imperative as only the letalis form is documented to be lethal. The risk for subsequent siblings is 25% and parents should undergo genetic counseling. Prenatal diagnosis of epidermolysis bullosa letalis can be made by fetoscopic skin biopsy. We suggest nonoperative management of pyloric atresia unless the skin disease is responsive to treatment.

Epidermolysis Bullosa↗

Fetendo-clip: a fetal endoscopic tracheal clip procedure in a human fetus.

Fetal tracheal occlusion accelerates lung growth and corrects the often fatal pulmonary hypoplasia seen in fetuses with congenital diaphragmatic hernia. Fetoscopy presents a unique opportunity to glimpse into the world of the fetus, but its use, until recently, has been limited to diagnostic and simple procedures. Using fetoscopic techniques ("Fetendo"), we now report successful tracheal occlusion in a 30-week-old fetus for the treatment of congenital diaphragmatic hernia.

Adult↗

Fetal carotid blood flow during videofetoscopy.

BACKGROUND/PURPOSE: Intracranial bleeding has been reported as one of the complications of both open and minimally invasive fetal surgery and putatively attributed to intraoperative fluctuations of carotid blood flow. The aim of this study was to look at fetal carotid blood flow and its relationship with umbilical blood flow, blood pressure, oxygen delivery, and acid-base status in the fetus at various intraamniotic pressures with both liquid and gas media during fetoscopic surgery. METHODS: Six 115- to 130-day-gestation ewes underwent continuous invasive systemic blood pressure monitoring in the descending aorta. A hysterotomy was performed. A 6-mm ultrasonic blood flow probe was placed around the common umbilical artery at its origin from the fetal aorta. This was followed by placement of a double-lumen, 4F catheter in the fetal descending aorta through a femoral artery. A 4-mm ultrasonic blood flow probe was then placed around the fetal left common carotid artery. A pressure-monitoring, multiperforated catheter was placed inside the amniotic cavity. The fetus was repositioned inside the uterus, which was then closed. The abdominal wall was closed loosely. No further manipulation was performed for 1 hour. Intraamniotic pressure was raised from 0 to 30 mm Hg at 5-mm Hg intervals by infusing either warmed saline or medical air. Common umbilical artery and left carotid artery blood flows, blood pressure, blood gases, bicarbonate, sodium, and hematocrit were recorded in all fetuses at each 5-mm Hg interval. Maternal systemic blood pressure, O2 saturation, and temperature were kept constant. RESULTS: Carotid blood flow remained stable within the intra-amniotic pressure range studied (0 to 30 mm Hg), despite the significant drop in common umbilical artery blood flow uniformly observed above 20 mm Hg when saline was infused and above 15 mm Hg when air was infused. There was fetal hypoxemia and hypercarbia concomitant with decreased common umbilical artery blood flow (however, without fetal acidosis, because of compensatory elevation of bicarbonate). Within the intraamniotic pressure range studied, fetal aortic blood pressure, sodium, and hematocrit did not vary significantly, even when there was decreased umbilical blood flow. CONCLUSIONS: Fetal carotid blood flow is protected, possibly autoregulated, remaining stable even after umbilical blood flow decreases as a consequence of elevated intrauterine pressures up to 30 mm Hg during videofetoscopy. These data suggests that perioperative intracranial bleeding related to videofetoscopic surgery is caused by factors other than fluctuations of cerebral blood flow.

Amniotic Fluid↗

Congenital diaphragmatic hernia: advances in prenatal therapy.

Despite intensive clinical and experimental efforts, mortality from CDH remains high. More than two decades of research in multiple centers has led to a better understanding of the pathophysiology, prognosis, and treatment options for fetuses that have CDH. It now appears that fetuses that have prenatally diagnosed CDH can be stratified into high- and low-risk groups based upon sonographic parameters. Fetuses that do not have liver herniation into the chest that have a favorable LHR have an excellent chance of survival with postnatal therapy. Prenatal diagnosis allows the time and place of delivery to be planned in advance so these infants can be treated in a tertiary care nursery that has maximal medical and surgical therapy. Fetuses that have liver herniation into the chest and an unfavorable LHR have a grim prognosis. These fetuses might benefit from in utero intervention. There is no role for open fetal repair of the diaphragmatic detect; however, fetoscopic temporary tracheal occlusion might improve lung growth and development and might decrease morbidity and mortality in these infants. The FETENDO strategy appears to work, and for the first time it offers hope to the fetus that has high-risk CDH, but its efficacy must be proven in a proper randomized, controlled trial.

Animals↗

Early prenatal diagnosis of neural-tube defects by ultrasound-guided fetoscopy.

In three fetuses with neural-tube defects (N.T.D.s) the lesions were clearly seen by fetoscopy in the second trimester. In a fourth fetus, in which the diagnosis of N.T.D. was suspected because of raised amniotic-fluid alpha-fetoprotein, spina bifida was excluded by fetoscopic examination and a normal baby was delivered at term. Ultrasound-guided fetoscopy has a place in the diagnosis of N.T.D.s when the results of other investigations are conflicting or inconclusive, and may be useful in assessing the severity of a lesion.

Amniocentesis↗

Dual diagnosis of prenatal haemophilia A by measurement of fetal factor VIIIC and VIIIC antigen (VIIICAg).

Procoagulant factor VIII (VIIIC) and procoagulant factor VIII antigen (VIIICAg) were measured in 20 samples of pure fetal blood obtained by fetoscopy at mid-term from non-haemophilic fetuses. VIIIC levels ranged from 25 to 89 U/dl (mean 43.5) and VIIICAg from 11 to 43 U/dl (mean 22.3). Samples from 39 consecutive fetuses at risk of haemophilia A gave results indicating haemophilia in 11, and these pregnancies were terminated at the parents' request. Of the remaining 28, 18 have resulted in normal births, 8 still await delivery, and 2 pregnancies were terminated for non-haematological reasons. The results confirm that pure fetal blood can be aspirated fetoscopically for plasma assays of both VIIIC and VIIICAg. Dual measurement enhances the reliability of predicting or excluding haemophilia A, and makes prenatal diagnosis a dependable option for known or potential carriers seeking this information.

Antigens↗