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At least 217 records · Page 12Linked to original sources

Prenatal diagnosis of harlequin ichthyosis.

We report the successful prenatal diagnosis of ichthyosis in the fetus of a woman whose previous liveborn child was affected with "harlequin ichthyosis". The fetal diagnosis was established through analysis of ultrasonographically guided fetoscopic skin biopsies. These biopsies showed premature hyperkeratosis, most marked around hair follicles and sweat ducts, and forming plugs of hyperkeratotic debris. These observations were in distinct contrast to those in control fetuses, whose epidermis consists of squamous epithelium only a few cells in depth with minimal keratinization.

Adult↗

Fetoscopy in prenatal diagnosis of the Majewski and the Saldino-Noonan types of the Short Rib-Polydactyly syndromes.

Fetoscopy was performed in three pregnancies at risk for the Majewski syndrome and in one pregnancy at risk for the Saldino-Noonan syndrome of the fetus. One case of Majewski syndrome and two normal fetuses were correctly diagnosed. In the remaining case the amniotic fluid was blood-stained and the fetus could not be visualized. Patients previously carrying a fetus with a Short Rib-Polydactyly syndrome of the Majewski or Saldino-Noonan types are at a high risk of recurrence (25%) and should be offered prenatal diagnosis in subsequent pregnancies. Polydactyly is consistently present in these syndromes, and is easily seen through the fetoscope. Fetoscopy offers a safe and instant diagnosis as early as 15-16 weeks of gestation.

Adult↗

In-utero intravascular transfusion of the fetus for the management of severe Rhesus isoimmunization--a reappraisal.

Ten fetuses, severely affected by Rhesus (D) haemolytic disease, received one to three intravascular blood transfusions at between 18 and 30 weeks gestation, with the use of fetoscopically guided needles into one of the umbilical cord vessels. Although the technique was successfully accomplished in all cases, the fetal response to the procedure was varied. Only two fetuses survived beyond the neonatal period, and one child subsequently died principally because of the problems resulting from premature delivery. The reason for the low rate of survival has been explored and the continued use of the method described is now questioned.

Blood Transfusion, Intrauterine↗

Antenatal fetal blood sampling for the management of alloimmunized pregnancies: effect upon maternal anti-D potency levels.

Increase in maternal anti-D concentrations after intrauterine investigation has been studied retrospectively in 95 rhesus (D) alloimmunized pregnancies; 48 were managed by fetal blood sampling (FBS) procedures (using fetoscopy or ultrasound-guided needle sampling) and 47 using amniocentesis. In those pregnancies where the fetus was rhesus (D) positive, the frequency of procedure-related increases (greater than 50%) in maternal anti-D potency was estimated following single procedures and found to be similar for the two methods of FBS employed (28%) and for amniocentesis (31%). The proportion of pregnancies showing an increase in anti-D potency was higher after ultrasound-guided needle sampling (75%) than after fetoscopic FBS (40%) and after amniocentesis (44%).

Amniocentesis↗

A randomized trial of electronic fetal monitoring in preterm labor: mothers' views.

To determine if perceptions of preterm labor and birth differed between women who were monitored by electronic fetal monitoring (EFM) or by periodic auscultation, 135 subjects were randomly assigned to one of two treatment groups on admission to a tertiary perinatal care setting. The first group received external monitoring by continuous Doppler and tocodynamometer when membranes were intact, and with an internal fetal scalp electrode and pressure catheter once membranes were ruptured. The second group received periodic monitoring with a DeLee fetoscope or amplified Doppler. All women were cared for on a one-to-one basis by expert study nurses. Subjects completed a questionnaire about their labor experience during their postpartum hospital stay. There was no statistically significant difference between the two groups on the study measures [T2(7,81) = 13.65; F = 1.82; P greater than 0.05]. Forty-four percent of the variance in women's global evaluation of labor was explained by their perceptions of nursing support. These findings suggest that mothers' perceptions of their preterm labor are less influenced by the technologic interventions used than by the supportive care received from nurses.

Adolescent↗

Fetal haematology in rhesus isoimmunisation.

Haematological studies were carried out in pure fetal blood samples obtained fetoscopically in 29 rhesus isoimmunised pregnancies at 18-24 weeks' gestation, and the values were compared with those obtained in 62 normal control pregnancies. Fetal reticulocytosis or erythroblastaemia was seen only in association with a haemoglobin concentration of 4 g/dl or less. Ten of the 14 fetuses with a haemoglobin concentration below 4 g/dl showed ultrasonographic evidence of hydrops.

Erythroblasts↗

Temporary iatrogenic fetal tricuspid valve atresia in a case of twin to twin transfusion syndrome.

This report describes the sequence of events in the development and subsequent spontaneous resolution of functional tricuspid valve atresia in the donor fetus in a case of twin to twin transfusion syndrome. Fetoscopic laser coagulation of the placental anastomoses was performed at 20 weeks' gestation. Subsequently, there was evidence of increased placental vascular resistance in the donor twin and major impairment of right ventricular function with no forward flow through the tricuspid valve. During the next four weeks, however, there was spontaneous and complete recovery of ventricular function and resolution of the functional tricuspid valve atresia. These findings suggest that alterations in fetal haemodynamics may result in structural cardiac abnormality and may be the precursors of some forms of congenital heart disease.

Echocardiography, Doppler, Color↗

Calcium homeostasis in second trimester fetuses.

The concentrations of ionised calcium ions (Ca++), total calcium, parathyroid hormone, pH, total protein, albumin, sodium, and potassium were measured in paired fetal and maternal blood from pregnancies at 15 to 24 weeks' gestation. Pure fetal blood samples were obtained fetoscopically. The concentrations of fetal ionised calcium ions (n = 26); mean (SD) 1.33 (0.12) mmol/l (5.32 (0.48) mg/100 ml) and those of parathyroid hormone (n = 9); 68 (19) pmol/l (58 (16) micrograms/100 ml) were significantly higher than those of the mothers: 1.18 (0.09) mmol/l (4.7 (0.4) mg/100 ml), and 40 pmol/l (less than 34 micrograms/100 ml), respectively. There was no difference between measured fetal and maternal total calcium, pH, and electrolytes. The fetal total protein and albumin concentrations increased with gestation but were always lower than the equivalent maternal values. The calculated total calcium was 0.23-0.45 mmol/l (0.9-1.8 mg/100 ml) higher in the fetal than in maternal blood from the same pregnancy. There were no fetal arteriovenous differences in ionised calcium ions despite higher venous pH.

Blood Proteins↗

Prenatal diagnosis of bullous ichthyosiform erythroderma: detection of tonofilament clumps in fetal epidermal and amniotic fluid cells.

The prenatal diagnosis of bullous ichthyosiform erythroderma (BIE) has been achieved at 20 weeks' gestation by electron microscopic identification of a pathognomonic cytoskeletal abnormality within fetal epidermal cells obtained by fetoscopic skin biopsy. The same abnormality was also observed in skin derived amniotic fluid cells. The question whether amniocentesis might be used instead of fetoscopy for future prenatal detection of BIE is discussed.

Actin Cytoskeleton↗

Fetoscopy in the pregnant rabbit at midgestation.

OBJECTIVE: To develop a small animal model for fetoscopy. METHODS: In 12 time-dated pregnant rabbits at 22 days' gestational age (term 32 days) one amniotic sac in each uterine horn (n = 24) was used for a fetoscopic procedure. After laparotomy, a 2- to 3-mm microsurgical myometrial incision was made to expose the chorionic and amniotic membrane. Under microscopic control, a 2-mm needle was inserted into the amniotic sac. Through this a 1.2-mm endoscope was passed to carry out fetoscopy during maximally 10 min, using 5-10 ml saline amnioinfusion. Mean outcome measurements were ability to visualize the placenta, umbilical cord and the different fetal elements during fetoscopy, as well as fetal survival and weight at second-look operation at 30 days. The untreated amniotic sacs served as negative controls. RESULTS: In all cases, fetoscopy could be carried out successfully, and all fetuses survived till delivery without significant influence on fetal birth weight. CONCLUSION: The midgestational rabbit can be used to perform fetoscopy.

Amnion↗

Continuous monitoring of fetal pH, pO(2) and pCO(2) using a fiberoptic multiparameter sensor in animal models reproducing in utero conditions.

OBJECTIVES: To evaluate the feasibility and accuracy of continuous fetal monitoring with a multiparameter intravascular sensor (MPIS) in animal models mimicking the fetal metabolic status. METHODS: First, the accuracy of the MPIS in hypoxic conditions was tested in adult rabbits (n = 6). The carotid artery (n = 4) or vein (n = 2) was catheterized with a 20-gauge cannula, housing a 500-microm Neotrend((R)) fiberoptic sensor for pCO(2), pO(2), pH and temperature. Fiberoptic readings were compared with arterial blood-gas (ABG) analyses. Secondly, the feasibility of continuous fetal fiberoptic sensing was tested during experimental fetoscopic surgery in lambs (n = 4). An identical sensor was introduced in a chorionic artery and readings were compared to ABG analyses of fetal blood samples. RESULTS: The overall bias and precision in the first experiment (147 measurement pairs) were -4.2 and 10.9 mm Hg for pO(2), +1.6 and 8.2 mm Hg for pCO(2) and -0.015 and 0.031 for pH, respectively. In the sheep experiments, continuous readings for all parameters were only available during 50% of the operation time, mainly due to disturbances induced by movement, contact with vessel wall and interference by endoscopic light. 20 sample pairs were obtained resulting in an overall bias and precision of -3.9 and 4.3 mm Hg for pO(2), -0.74 and 3.68 mm Hg for pCO(2) and -0.0032 and 0. 02 for pH. CONCLUSIONS: Fiberoptic microsensors are potentially useful for monitoring acid-base status in the low pO(2) range present in fetal life.

Animals↗

Endoscopic ligation of umbilical cord at 19 week's gestation in monoamniotic monochorionic twins discordant for hypoplastic left heart syndrome.

We report the first attempt of reduction of monoamniotic twins, discordant for hypoplastic left heart syndrome, using a new fetoscopic technique. Employing sonographic guidance and endoscopic visualization, cord ligation was accomplished, but significant cord entanglement, not previously appreciated, resulted in the ligation of the umbilical cord of the normal fetus. Cord entanglement may frustrate endoscopic techniques in monoamniotic twins.

Adult↗

Epidural versus general anesthesia for twin-twin transfusion syndrome requiring fetal surgery.

OBJECTIVES: This paper describes our experiences with epidural and general anesthesia for selective fetoscopic laser photocoagulation (SFLP) of placental vascular anastomoses in patients with severe twin-twin transfusion syndrome (TTTS). METHODS: Retrospective review of 29 anesthetic and surgical operative records of 28 women undergoing SFLP at The Children's Hospital of Philadelphia from July 1996 to June 2001. RESULTS: Patients with anterior placentas were more likely to receive a general anesthetic. Patients receiving an epidural anesthetic received significantly more intravenous (i.v.) crystalloid but less i.v. fentanyl than those receiving a general or combined technique. CONCLUSIONS: The advantages of general anesthesia include increased ability of the patient to tolerate extreme positions and the surgical manipulation of the uterus (required in the technically more difficult cases), greater uterine relaxation, decreased use of i.v. fluids, but increased fentanyl requirements. Epidural anesthesia avoids the risks of failed tracheal intubation in pregnant women but may not reduce fetal responses to surgical stimuli unless the mother receives supplemental intravenous drugs. However, this may increase the risk of respiratory depression. The choice of anesthetic technique for SFLP in patients with severe TTTS must be made after careful consideration of maternal and fetal factors.

Adjuvants, Anesthesia↗

Antenatal necrotic injury in severe twin-to-twin transfusion syndrome. A case and review.

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.

Adult↗

Effects on the maternofetal unit of the rabbit model after substitution of the amniotic fluid with perfluorocarbons.

OBJECTIVES: Exchanging amniotic fluid (AF) with perfluorocarbon (PFC) may serve as a medium for fetoscopic surgery. This study evaluates the distribution and physiologic effects of intraamniotic PFC as a medium for fetoscopy. METHODS: Fetuses of 17 pregnant rabbits underwent either exchange of the AF with PFC, electrolyte solution (ES), or control. The quality of vision during fetoscopy was assessed in AF and PFC. After 6 h, we determined the distribution of PFC in the maternofetal unit. RESULTS: Quality of vision during fetoscopy was better in PFC than with AF. There was no difference in fetal survival between the study groups. PFC was demonstrated on X-ray in the pharynx of 4 fetuses, and the esophagus in 1. CONCLUSIONS: PFC provided an ideal medium for fetoscopy without fetal compromise.

Amniotic Fluid↗

An experience of laser surgery for feto-fetal transfusion syndrome complicated with unexpected feto-fetal hemorrhage in a case of monochorionic triamniotic triplets.

Feto-fetal transfusion syndrome (FFTS) in monochorionic triplets is a rare clinical entity which may share the principal adverse perinatal outcomes of twin-twin transfusion syndrome. Recently, favorable prognoses regarding morbidity and mortality in twins after selective laser photocoagulation of placental communicating vessels (SLPCV) have been described. But descriptions of this procedure in monochorionic triplet cases are limited. This is the case report of an experience of SLPCV applied to monochorionic triamniotic triplets with FFTS. Triplet A had polyhydramnios, while absent end-diastolic flow in the umbilical artery of triplet B, the triplet with anhydramnios, was persistent. Triplet C looked normal, vertical amniotic pocket and Doppler studies yielding normal results. At the beginning of the SLPCV procedure, feto-fetal hemorrhage, subsequent to the fetal death of the donor triplet, occurred. Both surviving cofetuses showed persistent bradycardia; and 1 fetus died while the other recovered from the fetal bradycardia. All vascular anastomoses between the 3 triplets could be identified and obliterated, requiring two trocars. Our fetoscopic observation revealed feto-fetal hemorrhage after demise of monochorionic triplet, which led to injury of other fetuses due to hypovolemia in an instant. In conclusion, SLPCV might be a valid option for FFTS in triplet cases, while further experience is required in order to evaluate the risks and benefits of this procedure in triplet cases.

Adult↗

Markedly elevated alpha-fetoprotein and positive acetylcholinesterase in amniotic fluid from a pregnancy affected with dystrophic epidermolysis bullosa.

Prenatal diagnosis of dystrophic epidermolysis bullosa (DEB) has been achieved in the past by fetal skin sampling. However, this invasive procedure is associated with a relatively high rate of pregnancy loss. We present a consanguineous Arab family ascertained by 2 affected offspring to be at risk for DEB. In a previous gestation, fetoscopic skin sampling for prenatal diagnosis yielded a false-positive result. In the index pregnancy, abnormally elevated amniotic fluid alpha-fetoprotein (13.7 MOM) and positive acetylcholinesterase were highly suggestive of an affected fetus. Fetal skin biopsy was declined. At term, the patient delivered a male infant with DEB that expired on the 3rd day of life. It is apparent from our experience and from review of the literature that in some genodermatoses, markedly elevated alpha-fetoprotein and positive acetylcholinesterase in amniotic fluid are highly suggestive of an affected fetus and may obviate the need for fetal skin sampling in the prenatal diagnosis of these disorders.

Acetylcholinesterase↗

Treatment of the twin-twin transfusion syndrome: initial experience using laser-induced interstitial thermotherapy.

This paper describes our initial experience with laser-induced interstitial thermotherapy (LITT) for the treatment of the twin-twin transfusion syndrome (TTTS). This procedure was utilized in four pregnancies-three monochorionic twin pregnancies and one triplet pregnancy (20-26 weeks of gestation)-with severe TTTS with fetal dropsy, polyhydramnion of the acceptor, and anhydramnion of the donor. In vitro examinations of placental tissue had shown that laser coagulation can be monitored by sonography, hence we used this method for the first time in these four pregnancies. Blood vessels connecting the two umbilical cords were determined prior to the treatment using a new ultrasound color technique which is highly sensitive and capable of representing slow blood flow velocities. A 1.2 mm thick puncture needle was then directed to the shunt under on-line ultrasound control. All patients had an anterior wall placenta. The laser fiber was inserted via this thin needle. A coagulation time of 2-3 min was necessary at 3 W. In the one twin pregnancy the intrauterine fetal death of the smaller child occurred 10 weeks after LITT, the other child survived and is healthy. A cesarian section was necessary in another twin pregnancy 1 week after LITT due to the intrauterine death of the smaller child. In the third twin pregnancy, the donor, who had already had distinct bradycardia prior to the treatment, died immediately after LITT. The intrauterine fetal death of the donor in the triplet pregnancy occurred 3 days after LITT once the volume of amniotic fluid had basically returned to normal. The tragic intrauterine death of the uninvolved child occurred 13 weeks later as a result of umbilical cord strangulation, the surviving child is healthy. All four pregnancies were severe and advanced cases of TTTS with a very poor prognosis, leaving us with no other alternative to the described method of treatment. The instruments we used are a lot thinner than those utilized for fetoscopic laser treatment to date. Furthermore, it is not necessary to penetrate the amniotic sac in patients with an anterior wall placenta; intraplacental vessels can be coagulated, and the laser energy required for LITT is also much lower. In our opinion these advantages justify the utilization of LITT under more promising conditions than those described above.

Adult↗