Cases reported "Dilatation, Pathologic"

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1/37. Premature closure of the foramen ovale associated with aortic stenosis, left ventricular dilation with thrombus, and early mortality.

    Premature foramen ovale (FO) closure has been postulated as a cause of hypoplastic left heart syndrome. We suggest that premature FO closure is also associated with left ventricular (LV) dilation and LV thrombus formation, and that FO closure in patients with aortic stenosis and LV dilation is a secondary event that occurs later in gestation than that seen with the hypoplastic left heart.
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2/37. Fetal diagnosis of lethal dysfunction of the right heart in three siblings.

    A woman, having already delivered one child, underwent fetal echocardiography during three subsequent pregnancies. All three showed enlargement and poor function of the right-sided chambers. The first was still-born, the second died as a neonate, while the third pregnancy was terminated. Pathological examination revealed the same findings in each fetus, possibly representing a variation of Uhl's anomaly, or alternatively a hitherto unrecognised cardiomyopathic process.
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keywords = pregnancy
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3/37. prenatal diagnosis of de novo distal 11q deletion associated with sonographic findings of unilateral duplex renal system, pyelectasis and orofacial clefts.

    In utero diagnosis of de novo distal 11q deletion associated with renal and orofacial malformations has not been previously described. We present a 35-year-old pregnant woman with prenatal sonographic findings of a unilateral duplex renal system, pyelectasis and orofacial clefts at 20 weeks' gestation. Both genetic amniocentesis and postnatal cytogenetic analysis revealed de novo 46,XX,del(11)(q23). After birth, the fetus manifested a dysmorphic phenotype correlated with del(11q) syndrome. Genetic marker analysis showed a paternally derived distal deletion of chromosome 11q and a breakpoint centromeric to D11S1341. The present case represents the earliest prenatal diagnosis of a duplex renal system, pyelectasis and an additional feature of orofacial clefts associated with distal 11q deletion. Prenatal sonographic detection of a duplex renal system, pyelectasis and orofacial clefts should warrant a careful assessment of fetal anatomy and prompt cytogenetic analysis looking for chromosomal aberrations.
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4/37. Cortical dysplasia with subcutaneous angioma and dilated dural venous sinuses.

    We report a rare case with dilated dural venous sinuses, cortical dysplasia, and a subcutaneous angioma in the forehead. These lesions may be derived from some factors in the certain period of gestation, during which dural venous sinuses dilate due to increased intracranial pool of blood.
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5/37. Nonobstructive dilatation of the fetal bowel in twin gestations. A possible sonographic marker for fetal compromise.

    OBJECTIVE: To assess the possible association of in-utero bowel dilatation and circulatory changes with fetal compromise. methods: A retrospective survey of all ultrasound examinations was performed at the Chaim Sheba Medical Center (n approximately 10,000) between 1995 and 1999. Cases with ultrasonographic evidence of bowel dilatation, but without evidence of obstruction, were recruited. In utero Doppler studies of umbilical, splenic, superior mesenteric arteries, and middle cerebral artery (MCA) were performed: systolic/diastolic ratio (S/D), pulsatility index (PI) and resistance index (RI) were calculated in the above arteries and compared with those of normal fetuses. Nonstress test and cord blood pH were also assessed. Neonatal medical records were procured. RESULTS: Four fetuses, all of who were products of twin gestations, showed bowel dilatation without evidence for obstruction. Three of the four fetuses were small-for-gestation-age (SGA) and of bichorionic gestation, while one was of monochorionic twin gestation, with twin transfusion syndrome. In all three SGA fetuses, Doppler studies revealed increased peripheral resistance in the umbilical artery and adaptive peripheral vasodilatation in the fetal midcerebral, splenic and superior mesenteric arteries. In all cases, an abnormal nonstress test led to prompt delivery. CONCLUSIONS: Acute fetal bowel dilatation in a twin gestation is associated with abnormal splanchnic and gut perfusion that may lead to fetal compromise.
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6/37. Rapid progression of intrapulmonary arteriovenous shunting in polysplenia syndrome associated with biliary atresia.

    This report describes a patient with biliary atresia (BA) associated with polysplenia syndrome who showed a rapid progression of intrapulmonary arteriovenous shunting (IPS), resulting in a fatal outcome. Intrauterine ultrasonography at 36 weeks of gestation revealed fetal abnormalities, including situs inversus, absent retrohepatic inferior vena cava, and azygous connection. She was diagnosed postnatally as BA because of persistent acholic stool and neonatal jaundice. She underwent hepatic portoenterostomy at age 158 days. The gallbladder and the hepatic ducts were hypoplastic, and the common bile duct was absent. Magnetic resonance image and operative findings also identified polysplenia and an absent portal trunk. Liver histology showed cirrhotic changes and bile duct proliferation. Postoperatively, she achieved good bile secretion, with gradual decrease of total bilirubin. However, she had repeated febrile episodes, and computerized tomography at age 7 months showed multiple liver cysts. Thereafter, she presented with exertional dyspnea. Contrast-enhanced echocardiography showed IPS with a degree of 2/III at age 8 months and 3/III at 10 months. (99m)technetium-labeled macroaggregated albumin ((99m)Tc-MAA) scintigraphy revealed a shunt ratio of 25.5% at 9 months and 39.7% at 10 months. Percutaneous transhepatic drainage of the bile cysts was performed without success. Sludged bile was obtained. However, respiratory distress rapidly progressed, and she died at age 11 months. In the present patient, the association of polysplenia syndrome and absent portal vein with BA, as well as liver cirrhosis, seemed to be contributing factors to rapid progression of IPS in early life.
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7/37. prenatal diagnosis of esophageal atresia with the pouch sign.

    A 19-year-old primipara was referred to our center for a routine scan at 33 4 weeks' gestation. A visible but small stomach bubble (18 mm) was detected in the fetal abdomen, associated with a dilation of the esophagus with a tapering distal part. Direct visualization of filling and emptying of the proximal esophagus suggested the presence of obstruction. A cystic pouch in the region of the esophagus was observed to be full and empty in accordance with fetal swallowing. No other fetal anomalies were detected. Fetal biometric measurements were on the 10th percentile and polyhydramnios was detected. esophageal atresia type I was suspected. Fetal karyotyping was declined by the parents, no therapy was given and the pregnancy continued until week 37 when a female baby was delivered following spontaneous labor. esophageal atresia type I was radiographically confirmed. Bougienage was used for 8 weeks and esophageal anastomosis was successfully performed when the infant was 11 months old. No other anomalies were found and the child is currently in excellent health. The present case shows that the upper neck pouch sign may be a delayed manifestation present only in certain types of esophageal atresia. However, when it occurs it should prompt careful fetal examination as it is an important step in the diagnosis of esophageal atresia.
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ranking = 1.0822173617676
keywords = gestation, pregnancy
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8/37. Megacystis microcolon intestinal hypoperistalsis syndrome with bilateral duplicated systems.

    A 29-year-old multigravida woman presented for her second prenatal ultrasound evaluation at 30 weeks of gestation. The study showed a female fetus, bilateral duplicated systems with severe hydronephrosis in the upper pole moieties and a massively distended bladder. Initial interpretation suggested ectopic/obstructing bilateral ureteroceles. To evaluate these findings further, a prenatal magnetic resonance imaging scan was obtained, documenting the absence of ureteroceles. The presumptive diagnosis of megacystis microcolon intestinal hypoperistalsis syndrome was made. After birth, contrast enema confirmed the presence of microcolon. This appears to be the first reported case of megacystis microcolon intestinal hypoperistalsis syndrome with bilateral duplex systems evaluated with prenatal magnetic resonance imaging.
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9/37. placenta previa percreta with urinary bladder and ureter invasion.

    A 26-year-old woman, with one previous cesarean delivery and two uterine curettage due to incomplete abortion, was admitted to the labor ward with the diagnosis of partial placenta previa at 35 weeks of gestation. Repeat cesarean section was performed due to profuse vaginal bleeding. placenta previa percreta invading the bladder trigone was confirmed with cystotomy. As bilateral hypogastric artery ligation and supracervical hysterectomy performed were not successful in stopping the profuse bleeding, the abdomen was packed with laparotomy pads. dilatation of the left ureter was noticed on the second postoperative day. Relaparotomy was performed to remove the pads, and placental invasion of the distal left ureter was noticed. Ureteroneocystostomy was performed. The postoperative course was uneventful, and the double-J-catheter was removed two months later.
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ranking = 1
keywords = gestation
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10/37. Diminutive fetal left ventricle at mid-gestation associated with persistent left superior vena cava and coronary sinus dilatation.

    In a fetus with a small left ventricle diagnosed at mid-gestation, a persistent left superior vena cava connected to a dilated coronary sinus was detected. Although the size of the mitral annulus appeared to be normal, opening of the mitral valves was restricted in diastole. Echocardiographic follow-up showed no significant growth of the left ventricle and termination of pregnancy was carried out at 31 weeks at the parents' request. In addition to the prenatal findings, postmortem examination revealed a small mitral annulus with abnormal insertion of the mitral valve chordae tendineae. We hypothesized that abnormal venous return to a dilated coronary sinus may have led to mitral valve dysfunction and hypoplasia. On the other hand the left ventricular hypoplasia we observed may have involved a global abnormality of the left-sided cardiac structures.
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ranking = 5.0822173617676
keywords = gestation, pregnancy
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