Oligohydramnios Sequence Research Articles

The significance of clinical symptoms of subchorionic hematomas, “bleeding first”, to stratify the high-risk subgroup of very early preterm delivery

ObjectiveTo investigate the factors that stratify high-risk cases among subchorionic hematomas (SCHs) patients with persistent vaginal bleeding in early pregnancy. Materials and methodsA total of 56 patients who required hospitalization for SCH with vaginal bleeding in early pregnancy were classified into two groups: 1) no hematoma by ultrasonography when vaginal bleeding occurred, and then hematoma was observed by ultrasonography “bleeding to hematoma (BH group, n = 15)” and 2) no vaginal bleeding when hematoma was observed by routine ultrasonography, and then vaginal bleeding occurred later “hematoma to bleeding (HB group, n = 41)”. Retrospective cohort study was performed and maternal and neonatal outcomes were evaluated. ResultsThe duration of SCHs and/or vaginal bleeding was significantly longer in the BH group than in the HB group (mean: 60.8 days [BH group] vs. 33.3 days [HB group], p = 0.015). BH group patients delivered earlier than HB group patients significantly (mean: 27.3 weeks [BH group] vs. 35.6 weeks [HB group], p = 0.0028). The frequency of chronic abruption and oligohydramnios sequence (CAOS) was significantly higher in the BH group than in the HB group (3/15; 20.0% [BH group] vs. 0/41; 0.0% [HB group], p = 0.016). The frequency of sever fetal distress (Apgar score <4 points) was significantly higher in the BH group than in the HB group (4/15; 26.7% [BH group] vs. 0/41; 0.0% [HB group], p = 0.0037). The levels of factor XIII were relatively lower in the BH group than in the HB group (mean: 54.8% (n = 4) [BH group] vs. 76.1% (n = 7) [HB group], p = 0.077). ConclusionThe order of the symptoms, bleeding first, is an important feature that reflects the subsequent prolonged duration of SCHs/vaginal bleeding, resulting in very early preterm delivery. Continuous hemorrhage consumes coagulation factor XIII, which further worsen the hemostasis.

Renal oligohydramnios and Potter sequence with cystic kidney disease

For the first time in 1946 E.L. Potter (1901–1993) described the characteristic appearance of stillborns and deceased newborns with bilateral renal agenesis. Due to the further observations Potter distinguished the syndrome (Q60.6) – a set of characteristic external signs that are formed due to the extreme degree of oligohydramnios and intrauterine compression of the fetus. Classical Potter syndrome is diagnosed by the disfunction of both kidneys in the fetus (for example, bilateral agenesis), which leads to death. The term «Potter sequence» or oligohydramnios sequence with diverse causes has received the wide clinical use. The term «renal oligohydramnios» (ROH) is used to describe oligohydramnios resulting from a decrease or absence of fetal kidney function. The authors state that renal oligohydramnios and Potter sequence often develop in the fetus with cystic kidney disease with the formation of cysts in the parenchyma of both kidneys (autosomal recessive polycystic kidney disease, autosomal dominant polycystic kidney disease, glomerulocystic kidney disease associated with HNF1ß/TCF2 gene mutations, renal-coloboma syndrome, cystic renal hypoplasia, cystic renal dysplasia with mutations of the CEP55 gene).

Histological distribution pattern of hemosiderin deposition on the chorionic plate and fetal membrane of diffuse chorioamniotic hemosiderosis related to chronic abruption oligohydramnios sequence

IntroductionChronic abruption oligohydramnios sequence (CAOS) is histologically characterized by diffuse chorioamniotic hemosiderosis (DCH). However, the criteria for the histological evaluation of the extent of CAOS-related hemosiderin deposition (HD) of the membranes and the difference in HD between the chorionic plate (CP) and fetal membrane (FM) are not well studied. This case control study compared the degree and distribution pattern of HD on CP and FM to present the histological features of DCH and the criteria for histological evaluation. MethodsFrom the medical records of Kyoto University Hospital (2010–2019), we selected 20 CAOS cases that were clinically diagnosed by Elliot's criteria. Twenty non-CAOS cases matched to the CAOS group by gestational age were selected as controls. We compared the clinical data and pathological features in the two groups. We performed iron staining in all the cases and analyzed HD in CP and FM according to the histological score (H-Score: 0–12), which was determined as the density (0–3) multiplied by the extent of staining (0–4). ResultsHD was found in 100% (20/20) of CAOS and 15% (3/20) of control cases. In both the FM and CP, CAOS cases showed a significantly higher HS than control cases (CAOS, HS = 4–12; Control, HS = 0–1, p < 0.0001). Three CAOS patients presented HD alone in the CP. The HS of the CP was significantly higher than that of the FM (p = 0.0003). DiscussionCAOS presented DCH with HS ≥ 4. This study showed that the CP might be more suitable for evaluating DCH than the FM.

Successful treatment of severe arterial hypotension and anuria in a preterm infant with renal tubular dysgenesis\u2013 a case report

BackgroundOligohydramnios sequence can be caused by renal tubular dysgenesis (RTD), a rare condition resulting in pulmonary and renal morbidity. Besides typical features of Potter-sequence, the infants present with severe arterial hypotension and anuria as main symptoms. Establishing an adequate arterial blood pressure and sufficient renal perfusion is crucial for the survival of these infants.Case presentationWe describe a male preterm infant of 34 + 0 weeks of gestation. Prenatally oligohydramnios of unknown cause was detected. After uneventful delivery and good adaptation the infant developed respiratory distress due to a spontaneous right-sided pneumothorax and required thoracocentesis and placement of a chest tube; he showed no major respiratory concerns thereafter and needed only minimal ventilatory support. Echocardiography revealed no abnormalities, especially no pulmonary hypertension. However, he suffered from severe arterial hypotension and anuria refractory to catecholamine therapy (dobutamine, epinephrine and noradrenaline). After 36 h of life, vasopressin therapy was initiated resulting in an almost immediate stabilization of arterial blood pressure and subsequent onset of diuresis. Therapy with vasopressin was necessary for three weeks to maintain adequate arterial blood pressure levels and diuresis. Sepsis and adrenal insufficiency were ruled out as inflammation markers, microbiological tests and cortisol level were normal. At two weeks of age, our patient developed electrolyte disturbances which were successfully treated with fludrocortisone. He did not need renal replacement therapy. Genetic analyses revealed a novel compound hyterozygous mutation of RTD. Now 17 months of age, the patient is in clinically stable condition with treatment of fludrocortisone and sodium bicarbonate. He suffers from stage 2 chronic kidney disease; blood pressure, motor and cognitive development are normal.ConclusionsRTD is a rare cause of oligohydramnios sequence. Next to pulmonary hypoplasia, severe arterial hypotension is responsible for poor survival. We present the only second surviving infant with RTD, who did not require renal replacement therapy during the neonatal period. It can be speculated whether the use of vasopressin prevents renal replacement therapy as vasopressin increases urinary output by improving renal blood flow.

Chronic Abruption Oligohydramnios Sequence at 16 Weeks Pregnancy with Compensated Severe Anemia

Chronic Abruption Oligohydramnios Sequence at 16 Weeks Pregnancy with Compensated Severe Anemia

Oligohydramnios sequence revisited in relationship to arthrogryposis, with distinctive skin changes.

Thirty cases of arthrogryposis associated with longstanding oligohydramnios were identified among 2,500 cases of arthrogryposis (1.2%) and were reviewed for clinical features and natural history. None had renal agenesis or renal disease. Twenty-two had a history of known rupture of membranes. Only 50% had pulmonary hypoplasia at birth and only two died (7%). Sixty percent (18/30) seemed to have their multiple congenital contractures (MCC) primarily on the basis of compression related to the longstanding oligohydramnios and responded well to physical therapy. On average they did not have intrauterine growth restriction. "Potter" facies and remarkable skin changes were present in all. An excess of males was observed in spite of the lack of genitourinary anomalies.

Adverse perinatal and neonatal outcomes in patients with chronic abruption-oligohydramnios sequence.

Chronic abruption-oligohydramnios sequence (CAOS) is a clinical condition with lasting vaginal bleeding and oligohydramnios because of chronic placental abruption, which seems to cause preterm labor and neonatal chronic lung disease (CLD). This prompted us to explore the correlation between perinatal/neonatal outcomes and CAOS. Patients with suspected risk of abortion with recurrent vaginal bleeding were divided into CAOS and non-CAOS groups, and we compared the perinatal/neonatal outcomes between these two groups. To examine the impact of chorioamnionitis (CAM) on the prognosis of CAOS, we also compared outcomes between the CAOS group and gestational-age-matched preterm labor due to CAM (CAM group). In the CAOS and non-CAOS groups, initial vaginal bleeding was seen at the first trimester. However, its duration was significantly longer in the CAOS group. Additionally, neonatal birthweight was lower, and small-for-gestational-age (SGA) incidence was higher in the CAOS group. CLD was observed in most infants from CAOS patients. In the comparison between CAOS and CAM groups, birthweight was lower and SGA incidence was higher in the CAOS group. Moreover, CLD incidence and neonatal mortality were significantly higher, despite the lower incidence of severe CAM in the CAOS group. Finally, multivariate analysis demonstrated that duration of bleeding was a significant predictive factor for CAOS. Our observations demonstrated that patients with CAOS were a high-risk group for poor perinatal/neonatal outcomes. Moreover, episodes of recurrent and prolonged uterine bleeding were predictive factors for CAOS. During the first trimester, prolonged bleeding is an important sign as one symptom of CAOS.

Decrease of the Incidence of Renin-Angiotensin-System Inhibitor Induced Oligohydramnios-Sequence in Germany in 2011

to assess whether the incidence of angiotensin II-receptor type 1 antagonist (AT1-antagonist)– or ACE-inhibitor induced cases of oligohydramnios sequence (OHS) in 2011 was reduced after intensive alerts as to the causal association between AT1-antagonist /ACE-inhibitor and OHS in the German medical literature. 3 sources of information were used: A nationwide active surveillance of OHS in German paediatric hospitals (ESPED); Embryotox, (Berlin Institute for Clinical Teratology and Drug Risk Assessment in Pregnancy) and screening of pubmed (AT1-antagonist/ACE-inhibitor induced OHS). 45 cases of OHS were identified, no case due to maternal AT1-antagonist/ACE-inhibitor treatment. Causes for OHS were: premature rupture of membranes (PPROM) (n = 28), congenital anomalies of fetal kidneys and urinary tract(CAKUT (n = 15), placental insufficiency (n = 1),unknown cause (n = 1). Mortality until discharge was 37.8 % (32.1 % PPROM, 57.1 % CAKUT). Embryotox identified 3 exposures to AT1-antagonists in pregnancy, no case was associated with OHS. The pubmed search did not identify any case of OHS related to AT1-antagonist/ACE-inhibitor in pregnancy in Germany in 2011. Treatment of pregnant women with ACE inhibitors or AT1-antagonists still occurs but no cases of AT1-antagonist- or ACE-inhibitor induced OHS were reported in 2011 in Germany most likely due to repeated published alerts underlining the importance of consequent education. OHS remains a serious condition with high mortality despite modern intensive care.

Birth injury after cesarean section at 24 weeks of gestation: a large scalp laceration

Cesarean section (CS), compared with vaginal delivery, has a lower incidence of neonatal birth injury. Previously, we reported a femur fracture after term CS in this journal [1]. In a pregnant woman with chronic abruption oligohydramnios sequence, CS was performed at 24 weeks under general anesthesia due to continued oligohydramnios and elevated C-reactive protein level (4.5 mg/dL). The obstetrician had 20 years of experience. Being in head presentation, lower segment transverse incision was made with surgical knife: the incision was stopped before entering the uterine cavity to avoid direct injury to the fetal scalp. The uterine cavity was entered with a finger and the incision was extended laterally by both index fingers (Fig. 1a). Then the doctor recognized something ‘‘white’’ just beneath the fingers. In retrospect, it was the skull bone: the scalp skin may have been lacerated by the fingers, sliding off from the underlying skull bone (Fig. 1b). This was well reconstructed. Bleeding from the laceration was slight, requiring no blood transfusion. Extra-or-subdural hematoma or intra-ventricular hemorrhage was absent. The infant, weighing 544 g with Apgar score 4/7 (1/5 min), died the next day due to prematurity. Autopsy was not performed. The scalp laceration itself was not considered to have influenced the outcome. Another similar experience: a scalp laceration in an infant after 23 weeks’ CS. Oligohydramnios due to premature rupture of the membranes and signs of intrauterine infection required CS. The fetal head was deeply engaged and thus the hand was put deeply into the uterine cavity, pulling out the fetal head with the fingers. In retrospect, the scalp, being pushed and possibly scratched by the fingers, was lacerated at the parietal area. The infant died next day due to prematurity. The laceration was not considered to have influenced the outcome. There is a close similarity between the two patients: preterm CS, oligohydramnios, and intrauterine infections. A previous report [2] also showed that ruptured membranes and thus oligohydramnios were the risk factors of fetal scalp laceration, in which, however, infants suffering laceration were almost term infants (average of 38.67 weeks), with the earliest and the lightest being 29 weeks and 825 g of birth weight, respectively. Its context was that obstetricians should avoid direct injury to the fetal scalp with the knife or scalpel [2]. The present significance is different: pushing or scratching the scalp skin with fingers was the considered culprit. Skin and the underlying skull bone, especially at the parietal area, are more loosely connected than skin and other body parts such as the extremities or the trunk. This is more exaggerated in preterm infants. Intra-uterine infection, weakening the skin, may also have caused scalp laceration. Oligohydramnios, pushing the fetal head tightly to the uterine wall, obliged the obstetrician to put fingers into the narrow space between the fetal head and the uterine wall, which may also have caused this injury. The CS procedure was performed gently, considering the infant’s prematurity; however, a much gentler maneuver S. Matsubara (&) R. Usui Department of Obstetrics and Gynecology, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi 329-0498, Japan e-mail: matsushi@jichi.ac.jp

Prenatal Management of the Fetus with Lethal Malformation: From a Study of Oligohydramnios Sequence

47 cases of oligohydramnios sequence (OS) diagnosed at Kanagawa Children's Medical Center from 1992 to 2008 were studied retrospectively. Early termination of pregnancy was chosen in 9 cases, observed natural labor was chosen in 23 cases, and 18 cases resulted in natural deliveries. Fetal demise occurred during labor in 44.4% of terminated cases, while it occurred in 5.6% of observed cases. Preterm labors and breech presentations occurred most frequently. Most infants died within 18 hours after their births. There were 3 familial cases. These results provide important information for planning the perinatal care when fetuses are diagnosed with OS.

The use of high frequency jet ventilation to treat suspected pulmonary hypoplasia

We report two cases of term male infants with suspected pulmonary hypoplasia, who could not be managed successfully with conventional mechanical ventilation. Both responded to rescue support with high frequency jet ventilation (HFJV) using an optimal positive end expiratory pressure strategy. One infant had the oligohydramnios sequence secondary to posterior urethral valves. The second infant was diagnosed prenatally with a severe right-sided congenital diaphragmatic hernia. We summarize the literature on the use of HFJV to support infants with severe pulmonary dysfunction. Unique air flow dynamics of HFJV may enable better gas exchange with less barotrauma, less volutrauma and ventilation at lower mean airway pressure. Our experience and the available literature suggest that HFJV appears to offer a unique advantage over conventional ventilation in such infants.

Syndrome transfuseur-transfusé : physiopathologie, diagnostic et traitement chirurgical

Multiple pregnancies represent 2% of all pregnancies but account for 20% of admissions to neonatal intensive care units. The outcome of multiple pregnancies is mainly dependent on chorionicity. Most perinatal complications are 3 to 12 times more prevalent in monochorionic pregnancies. The increased mortality and morbidity in this setting are mainly related to vascular anastomoses on the chorionic plate, joining the two fetal circulations. Intrauterine death of a monochorionic twin leads to exsanguination of the survivor, with fatal outcome in 20 to 30% of cases and a similar rate of severe ischemic complications. The most severe and acute complication is the twin-to-twin transfusion syndrome (TTTS). The main clinical manifestations of TTTS are the polyuric polyhydramnios--oliguric oligohydramnios sequence in the recipient and donor twin, respectively. TTTS is associated with a perinatal mortality rate of around 90%, and neurological sequelae are present in 20-40% of survivors born at around 25 weeks. Serial amnioreduction was long the only fetal therapy for TTTS, improving the survival rate to around 50% for at least one twin delivered at around 28 weeks, and reducing the risk of sequelae to around 20% among survivors. We have developed an intrauterine fetoscopic surgical treatment for TTTS. A 2-mm endoscope and a diode laser fiber are introduced percutaneously, under local anesthesia, through a single 3-mm trocard Coagulation of feto-fetal anastomoses on the chorionic plate leads to the survival of at least one twin in around 80% of cases at 33 weeks. Fewer than 10% of survivors have sequelae, mainly related to prematurity. We conducted a randomized controlled trial that confirmed the superiority of laser therapy over amnioreduction. The long-term outlook of these infants is good, with up to 6 years of follow-up.

OP10.02: The CHOP cardiovascular score: a method for quantification of cardiovascular abnormality in the twin–twin transfusion syndrome

Objectives: Umbilical vein volume flow (UVVF), calculated as velocity (cm/s) times transversal section area at the straight portion of the intra-abdominal umbilical vein (cm2), is an interesting parameter to study in twin–twin transfusion syndrome (TTTS). Recipients were found to have 2 to 3 times higher UVVF than donors, but this parameter was not studied in normal monochorionic pregnancies. Methods: Comparison of recipient to donor (rec/don) UVVF ratio and bigger-to-smaller UVVF ratio in non-TTTS monochorionic twin pregnancies (control group). The method of dividing the same parameter from each pair of twins aimed to emulate other factors such as gestational age and maternal condition, which change between pregnancies. The control group consisted of monochorionic twin pregnancies with normal umbilical artery Doppler and normal amniotic fluid (2 to 8 cm of MVP), between 18 and 26 weeks. Results: Eight-four TTTS and 24 normal monochorionic twin pregnancies were compared. The median weight discordance was 1.3 (rec/don) and 1.2 respectively (big/small, Wilcoxon P < 0.7). The UVVF ratio between recipients and donors (UVVF R/D) was greater than that of the bigger to smaller UVVF ratio from normal monochorionic twins (medians 2.2 and 1.3, Wilcoxon P < 0.001). Conclusions: This study shows that UVVF R/D imbalance occurs in cases with the polyhydramnios and oligohydramnios sequence found in TTTS, and that normal monchorionic pregnancies lack this imbalance. The difference is not due to fetal weight discordance as it was shown that normal monochorionic twins had similar discordance. The parameter has still not proven to be useful in screening for early diagnosis by this methodology, but it describes that UVVF imbalance between twins does not exist in normal monochorionic pregnancies. OP10.02 The CHOP cardiovascular score: a method for quantification of cardiovascular abnormality in the twin–twin transfusion syndrome

The extreme Spectrum of Pallister-Hall syndrome

Pallister-Hall syndrome can result from GLI 3 gene mutation and is characterized by hypothalamic hamartoblastoma, hypopituitarism, imperforate anus, and postaxial polydactyly. It has a wide spectrum of severity, from perinatal lethal to minimally symptomatic. Familial cases with different expressiveness were reported before. We herein report a 22-week terminated fetus, which was a product of a GIPO 16-year-old healthy mother. Initial ultrasound at 20 weeks gestation revealed severe oligohydramnios and a small fetus. Subsequent ultrasound revealed absent stomach, dysplastic kidneys, short limbs, distal dysplasia, abnormal facies and the possibility of Roberts syndrome was discussed. The patient was counseled and pregnancy termination was sought. Cytogenetic studies revealed a normal 46,XY male karyotype and negative study for centromere analysis. Autopsy revealed a normally grown 22-23 week male fetus with hypothalamic hamartoma, polydactyly, imperforate anus and multiple other congenital anomalies. He had small, wide-spaced eyes, bilateral choanal atresia, soft tissue mass of the philtrum, small mouth with soft tissue fusion between alveolar ridges, lips and tongue, abnormal palate with possible midline clefting, micrognathia and simple lowset ears. There were short upper extremity digits with hypoplastic nail bed and bilateral partial duplication and fusion of the fourth digits. The feel were markedly everted with small medially placed great toes. The third, fourth and fifth digits are all short with poor nail bed. All digits had prominent syndactyly. There was anal atresia with blind rectosigmoid colon emptying into the bladder region without rectovesicular fistula. There was bilateral renal hypoplastic dysplasia with disorganization of the cortex. Brain examination revealed hypothalamic hamartoblastoma, 1.0 × 1.5 × 1.0 cm. There was absent cerebellar vermis, absent corpus callosum, probable absent olfactory grooves and tracts and sella turica wilh no pituitary gland present. Small adrenal glands with disorganization of the adrenal cortex were also noted. Cardiac exam revealed malrotation of the great vessels and cardiomegaly. Pulmonary hypoplasia and oligohydramnios sequence was noted with flexion conlractures of the joints. Our patient represents an extreme spectrum of PHS and emphasizes the importance of recognition of fetal syndrome and documented autopsy for genetic counseling.

Limb deformations in oligohydramnios sequence: Effects of gestational age and duration of oligohydramnios

Limb deformations in oligohydramnios sequence: Effects of gestational age and duration of oligohydramnios

Limb deformations in oligohydramnios sequence: Effects of gestational age and duration of oligohydramnios

In order to study the pathogenesis of prenatal deformities, we reviewed maternal histories, delivery records, pathology reports, radiographs, and photographs of 90 fetuses with prenatally documented oligohydramnios at gestational ages from 14 weeks to term. The causes of oligohydramnios included premature rupture of membranes (44 cases), fetal renal insufficiency (25 cases), idiopathic (15 cases), and twin-twin transfusion (6 cases). The fetuses were grouped according to gestational age at delivery and duration of oligohydramnios. Sixty-three fetuses (70%) had documented contractures. As expected, contractures were more frequent with earlier onset and longer duration of oligohydramnios. During the 2nd trimester, the frequency of contractures in fetuses with oligohydramnios was 77% compared to 52% in the 3rd trimester (chi(2) = 5.33, 1 df, P =.02). Considering all gestational ages together, 57% of fetuses had contractures after less than 2 weeks of oligohydramnios compared to 81% of fetuses with a longer duration of oligohydramnios (chi2 = 6.23, 1 df, P <.02). The type of contracture varied with gestational age. Clubfoot was the most frequent at all ages, but hand contractures such as camptodactyly were common only in the 2nd trimester while the broad flat hand originally described in Potter sequence was found almost exclusively in the fetuses with oligohydramnios in the 3rd trimester. Of the 63 fetuses with oligohydramnios and contractures, 25 (40%) had either additional malformations or family history that could explain contractures independent of oligohydramnios.

Limb deformations in oligohydramnios sequence: effects of gestational age and duration of oligohydramnios

Oligohydramnios, regardless of cause, results in a sequence of anomalies including characteristic facies, contractures and lung hypoplasia, but the timing and duration of of oligohydramnios required to produce different features of the sequence, especially limb defects, are not well defined. Other causes of restricted fetal movement such as fetal neuromuscular disorders also result in limb contractures and lung hypoplasia. Because such neuromuscular disorders often cause polyhydramnios which in turn predisposes to premature rupture of membranes, such fetuses may present with ruptured membranes and oligohydramnios. Additional data regarding the type and incidence of limb contractures in fetuses with differing onset and duration of oligohydramnios is needed to provide accurate counseling regarding the underlying cause and recurrence risks.We have reviewed maternal histories, delivery records, pathology reports, radiographs and photographs of 90 fetuses with gestational ages from 14 weeks to term with >24 hrs documented oligohydramnios. The causes of oligohydramnios included premature rupture of membranes (44 cases), fetal renal insufficiency (25 cases), idiopathic (15 cases) and twin-twin transfusion (6 cases). The fetuses were grouped according to gestational age at delivery and duration of oligohydramnios. As expected, contractures were more frequent with earlier onset and longer duration of oligohydramnios. During the second trimester, the frequency of contractures in fetuses with oligohydramnios was 77% compared to 52% in the third trimester(p=.02). Considering all gestational ages together, 57% of fetuses had contractures after less than 2 weeks of oligohydramnios compared to 81% of fetuses with a longer duration of oligohydramnios (p<.02). The type of contracture varied with gestational age. Club foot was the most frequent at all ages, but hand contractures such as camptodactyly were common only in the second trimester while the broad flat hand originally described in Potter sequence was found almost exclusively in the third trimester. Of the 59 fetuses with oligohydramnios and contractures, 25 (42%) had either additional malformations or family history that could explain contractures independent of oligohydramnios.Oligohydramnios sequence is common in the second trimester, but presents differently compared to the third trimester and is more easily confused with other birth defects. Fetuses with oligohydramnios and contractures require a detailed examination and review of history before the contractures can be attributed to oligohydramnios sequence.

ADDITIONAL DIAGNOSES IN MULTIPLE PREGNANCIES WITH POLYHYDRAMNIOS / OLIGOHYDRAMNIOS SEQUENCE AND PRESUMED TWIN TRANSFUSION SYNDROME 861

Objective: To report additional diagnoses in presumed twin transfusion syndrome (TTS) presenting with polyhydramnios / oligohydramnios(poly/oli) sequence treated with amnioreduction.

PERINATAL SURVIVAL AND MORBIDITY IN MULTIPLE PREGNANCIES WITH POLYHYDRAMNIOS/ OLIGOHYDRAMNIOS SEQUENCE TREATED WITH AMNIOREDUCTION 860

Objective: To determine survival and morbidity in twin transfusion syndrome presenting with polyhydramnios / oligohydramnios(poly/oli) sequence managed by amnioreduction.

VACTERL association, epidemiologic definition and delineation

This study departed from a preconceived definition of VACTERL, including more than one of these six anomalies in the same infant:; V (vertebral anomalies), A (anal atresia), C (congenital heart disease), TE (tracheoesophageal fistula or esophageal atresia), R (reno-urinary anomalies), and L (radial limb defect). Under this definition, 524 infants were ascertained by ECLAMC from almost 3,000,000 births examined from 1967 through 1990. Observed association rates among VACTERL components as well as between VACTERL and other defects were compared against randomly expected values obtained from 10,084 multiply malformed infants (casuistic method) from the same birth sample. Conclusions were: 1) Cardiac defects are not a part of VACTERL. 2) Single umbilical artery, ambiguous genitalia, abdominal wall defects, diaphragmatic hernia, and anomalies that are secondary to VACTERL components (intestinal and respiratory anomalies, and oligohydramnios sequence defects) are frequent enough to be considered an “extension” of VACTERL, and cardiac defects should be included in this category. 3) Neural tube defects are negatively associated with VACTERL which could not be explained by selection bias or any other operational artifact. High embryonic lethality or mutually exclusive pathogenetic mechanisms could be suitable explanations. 4) Results were not clear enough to determine whether VACTERL should be defined by at least two or three component defects. © 1996 Wiley-Liss, Inc.