Correlation between the presence of liver herniation and perinatal outcome in prenatally diagnosed fetal omphalocele Ppt Powerpoint Presentation slides Nobuhiro Hidaka Medical Slideshow Fetal Surgery for Prenatally Diagnosed Malformations

Correlation between the presence of liver herniation and perinatal outcome in prenatally diagnosed fetal omphalocele

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Slide 1 :

Correlation between the presence of liver herniation and perinatal outcome in prenatally diagnosed fetal omphalocele Department of Obstetrics and Gynecology, Kyushu University Nobuhiro Hidaka

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Abstract Objectives: The purpose of this study was to investigate the association between the presence of liver herniation and perinatal course in prenatally diagnosed fetal omphalocele, and to discuss the clinical significance of liver herniation as a predictive marker. Methods: A retrospective review was conducted of fetuses prenatally diagnosed with omphalocele at Kyushu University Hospital between 1990 and 2006. Results: Thirty-three fetal omphaloceles were diagnosed at 12-38 weeks of gestation. The chromosomal status of 29 of 33 fetuses was determined; 18 of these were found to have omphalocele with extracorporeal liver, while 11 had intracorporeal liver. The rate of chromosomal abnormalities in the extracorporeal group was significantly lower (11%, 2/18) than in the intracorporeal group (55%, 6/11) (p = 0.028). In chromosomally normal cases, four with extracorporeal liver resulted in early neonatal death while none with intracorporeal liver died. Five of the 21 chromosomally normal fetuses showed an abnormal volume of amniotic fluid. All five of these cases had extracorporeal liver and two of them resulted in neonatal death. Conclusions: Fetuses with extracorporeal livers had a lower rate of chromosomal abnormalities than members of the intracorporeal liver group. However, in chromosomally normal cases, it appeared that extracorporeal livers might be associated with more life-threatening anomalies, amniotic fluid volume abnormalities, and a higher rate of mortality than intracorporeal livers. Upon diagnosis of fetal omphalocele, a careful search for liver location should be conducted before parents are counseled on the expected perinatal course of the fetus.

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Introduction 1 The omphalocele is a defect in the ventral abdominal wall characterized by an absence of abdominal muscles, fascia, and skin, which are covered by a membrane consisting of peritoneum and amnion. The spectrum of severity of omphalocele can vary from a small umbilical hernia to a large abdominal wall defect with extrusion of all abdominal viscera. The etiology has been speculated by some previous investigators; however, there is room for further discussion on the specific cause for its occurrence.

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Introduction 2 Large defects (i.e., extracorporeal liver), is speculated to be caused by the arrested development of the lateral folds and failure of body wall closure in the third or fourth week of gestation. (Dimmick JE et al., Developmental pathology of the embryo and fetus. Philadelphia, Lippincott, 1992, pp 527-529) Small defects (i.e., intracorporeal liver), may be a failure of the intestine to return to the abdominal cavity through the umbilicus, a developmental malformation occurring at approximately 12 weeks of gestation. (Brandt ML. Avery’s diseases of the newborn. Saunders, Philadelphia, 1998, pp 979-994) Considering the difference of the speculated etiology, small and large omphaloceles may be essentially different disorders.

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Objectives Some knowledge of the clinical characteristics of fetal omphalocele has been gathered from previous investigations; however, further efforts to classify fetal omphalocele based on liver location would surely facilitate more appropriate parental counseling and possibly improve patient care. The aims of this study are to investigate the association between liver herniation and perinatal course in prenatally diagnosed fetal omphalocele, and to discuss the clinical significance of liver herniation as a predictive marker.

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Method 1 Study population: The study population included all fetuses with a prenatal diagnosis of omphalocele at Kyushu University Hospital from 1990 to 2006. Data were collected retrospectively by reviewing maternal and neonatal medical records.

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Method 2 Management policy: After the initial diagnosis was made, repetitive serial transabdominal ultrasonographic examinations, including detailed evaluations of the herniated organs, were performed. Women found to have fetal omphalocele were usually offered an amniocentesis to determine the fetal karyotype. Antenatal steroids were administered to women at high risk of preterm delivery before 34 weeks gestation in order to induce pulmonary maturation. Cesarean delivery was routinely performed (except in cases of trisomy 13 and trisomy 18), usually electively, at 37-38 weeks of gestation if there were no pregnancy complications.

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Method 3 Definition of terms: Intrauterine growth restriction (IUGR) was defined as a birth weight below the 10th percentile for the given gestational age. Preterm delivery was defined as delivery before 37 complete weeks of gestation. Polyhydramnios was sonographically defined as an amniotic fluid pocket of more than 8 cm, whereas oligohydramnios was defined as an amniotic fluid pocket of less than 2 cm. Examination after birth included echocardiography; however, neonates with patent ductus arteriosus or patent foramen ovale were not considered to have cardiac disease in this study. Statistics: Results were expressed as the mean ± standard deviation. Data were analyzed using Student’s t test and Fisher’s exact test, and differences between groups were considered significant at p < 0.05.

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Result 1 Perinatal data of 33 cases with prenatally diagnosed omphalocele * excluding TOP cases IUGR: intrauterine growth restriction IUFD: intrauterine fetal death TOP: termination of pregnancy

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Result 2 Correlation of the presence of liver herniation and chromosomal abnormality in fetuses with omphalocele P=0.028 (trisomy 13, n = 2; trisomy 18, n = 4) (trisomy 18) Number of patients

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Result 3 Clinical characteristics and perinatal outcome of chromosomally normal patients with omphalocele stratified by extracorporeal liver * excluding TOP cases ART: assisted reproductive technology CTG: cardiotocogram IUGR: intrauterine growth restriction PPHN: persistent pulmonary hypertension of the newborn TOP: termination of pregnancy

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Result 4 Surgical procedures in chromosomally normal omphaloceles * excluding TOP cases

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Result 5 List of associated anomalies in chromosomally normal fetuses with omphalocele Case Associated anomalies 1 * Anencephaly,Acrania 2 Meningocele 3 Bladder exstrophy, Meningocele, Anal atresia (OEIS) 4 ? Diaphragmatic hernia, Asternia, CHD, 5 ? Cantrell, CHD(truncus, VSD) 6 Scoliosis, Hypoplastic right leg, Diaphragmatic eventration, SUA 7 Scoliosis, Finger anchylosis, Short neck 8 ? Scoliosis, Finger defect, SUA 9 Syndactylus, Cryptorchism 10 SUA 11 ? none 12 * none 13 none 14 none 15 none 16 none 17 Beckwith-Wiedemann syndrome 18 CHD (DORV), Low sets ears, Buried penis, Cryptorchism 19 Ileal atresia 20 none 21 none Extra- group Intra- group CHD: congenital heart disease, VSD: ventricular septum defect, SUA: single umbilical artery, DORV: double outlet right ventricle *TOP ?neonatal death

Slide 14 :

Conclusions ·The existence of an intracorporeal liver is correlated with the existence of chromosomal abnormalities. ·In chromosomally normal cases, extracorporeal liver status and/or amniotic fluid volume abnormalities may have value as indicators for poor prognosis. ·The incidence of associated anomalies was similar in both groups; however, the extracorporeal group tended to be complicated with more severe anomalies that had a higher potential for lethality for the affected babies. ·Cases for whom a surgical procedure could not be performed because of severe respiratory distress or acute clinical course ultimately ended in neonatal death. However, when respiratory status and general condition were sufficiently sustainable for an operative procedure, the mortality rate after surgery was low. ·Upon diagnosis of fetal omphalocele, a careful search for liver location should be conducted before parents are counseled on the expected perinatal course of the fetus.

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	n-hidaka@kpa.biglobe.ne.jp 1 Years ago. Category: Obstetrics and Gynecology

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