Sudden fetal hemorrhage from umbilical cord ulcer associated with congenital intestinal atresia

There have been several reports of umbilical cord ulcer (UCU) associated with congenital intestinal atresia, many cases of which have been reported with fetal or neonatal death. Herein is described the case of a fetus with jejunal atresia complicated by acute massive intrauterine hemorrhage due to the perforation of UCU resulting in an intact survival. Although the onset of critical condition in the present case was noted due to continuous fetal heart rate (FHR) monitoring, it is necessary to establish the management of congenital intestinal atresia to prevent this life-threatening fetal hemorrhage.     

Umbilical cord ulceration and intestinal atresia

Umbilical ulceration is an extremely rare complication in the perinatal period. We encountered a case of intestinal atresia complicated by massive intrauterine hemorrhage due to the umbilical cord ulceration. This is the fifth reported case demonstrating an association between the umbilical cord ulceration and intestinal atresia.     

Umbilical cord ulcer: a serious in utero complication of intestinal atresia

We have experienced six cases of umbilical cord ulcer that resulted in life-threatening antenatal fetal haemorrhage. Fetal distress and bloody amniotic fluid that led to intrauterine and neonatal death in four patients complicated the pregnancy courses. The remaining two infants are still alive. All the infants had upper intestinal atresia below the papilla of Vater. Pathologically, umbilical arteries and surrounding Wharton's jelly were necrotic in all cases. Activated macrophages containing pigment granules were present within the ulcer bed, necrotic areas of Wharton's jelly and fetal membranes. During the same period, we encountered 38 other cases of upper intestinal atresia and the placentae were examined in 23 cases. Microscopic degeneration of Wharton's jelly was seen in three out of 23 cases. Total bile acids content in amniotic fluid was elevated in one case of umbilical cord ulcer. We speculate that the umbilical cord ulcer is closely related to in utero regurgitation of the bile. We also think that the incidence of this serious association is higher than has been reported previously.     

Umbilical cord ulceration after prenatal diagnosis of duodenal atresia with interstitial deletion of chromosome 13q: a case report

Umbilical cord ulceration complicated by massive local hemorrhage may be a lethal event in the fetus, and this ulceration has been reported to be associated with upper intestinal atresia. The diagnosis of umbilical cord bleeding is difficult. We present a case of umbilical cord ulceration, hemorrhage, and duodenal atresia which had, in addition, an interstitial deletion of chromosome 13q. A female infant weighing 1,691 g was delivered by cesarean section at 34 weeks of gestation and had resuscitation and laparotomy. Just before the cesarean section, ultrasonography showed a 'double bubble' sign and a linear shadow, suggesting fibrin in the amniotic cavity. This finding may help in the diagnosis of bleeding from the cord.     

Upper neck pouch sign in prenatal diagnosis of esophageal atresia

Introduction Prenatal diagnosis of esophageal atresia is usually based on a suspicion, with the finding of a small or absent stomach in the presence of polyhydramnios. However, this combination has a low positive predictive value, and this uncertainty in prenatal diagnosis confuses the decision making process, and causes an unnecessary parental anxiety. The upper neck pouch sign is a more reliable finding for prenatal diagnosis of esophageal atresia. Despite its usefulness there have been several cases reported until now.Case report and discussion We diagnosed a new case with congenital esophagus atresia by detecting upper neck pouch sign, and discussed the value of this finding.     

Intrauterine hemorrhage from an umbilical cord ulcer associated with fetal duodenal atresia: a case report

In the recent years, the association between congenital intestinal atresia and umbilical cord ulceration has been demonstrated; however, this complication is rarely encountered. To the best of our knowledge, only 14 cases on this association have been published previously. We encountered a case of acute intrauterine hemorrhage from an umbilical cord ulcer in a fetus with duodenal atresia that resulted in early neonatal death. There is little information available on the association between congenital intestinal atresia and umbilical cord ulceration. Greater understanding may alter the obstetric management. Although the prevention of sudden perinatal death is considered to be difficult, frequent fetal monitoring and detailed ultrasonographic examinations may facilitate the early detection of this condition, thereby enabling the rescue of the affected fetuses. Further investigation is required to establish the management protocol.     

Prenatal obstruction of the ileum diagnosed by ultrasound

A case of low atresia of the ileum, diagnosed prenatally by real-time ultrasound scanning, is presented. The ultrasound examination showed progressive distension of intestinal loops, with strong peristaltic movements. The stomach was also distended, presenting as a large cystic area in the upper left abdomen. Real-time ultrasound technique is most advantageous in the diagnosis of fetal ileus. Prenatal diagnosis of fetal intestinal obstruction is of great importance, making early and safe treatment of the newborn possible.     

Fetal small bowel obstruction: report of two cases

Fetal small bowel obstruction is usually diagnosed by sonography in the late second or early third trimester. We report two such cases of different etiology: a case of meconium ileus due to cystic fibrosis and a case of intestinal atresia. The only sonographic finding that allowed differentiation between them was the echogenic bowel in the case of cystic fibrosis.     

Alveolar capillary dysplasia associated with duodenal atresia: ultrasonographic findings of enlarged, highly echogenic lungs and gastric dilatation in a third-trimester fetus

We report a case of alveolar capillary dysplasia, wherein duodenal atresia was diagnosed during the third trimester. A 36-year-old mother was referred to our hospital for polyhydramnios at 31 weeks' gestation. Duodenal atresia was suspected from the ultrasonographic findings, which showed gastric dilation. Other findings noted were enlarged, highly echogenic lungs, a spherical heart and an increased lung-thorax transverse area ratio. A male infant was born at 37 weeks' gestation. The findings of serial radiography of the infant's upper gastrointestinal tract were compatible with the diagnosis of duodenal atresia; however, he developed persistent pulmonary hypertension of the newborn eight hours after birth and died at five days of age. The autopsy revealed alveolar capillary dysplasia and duodenal obstruction. We propose that the detection of duodenal atresia should prompt the physician to conduct a thorough ultrasonographic examination to rule out associated anomalies, such as alveolar capillary dysplasia, which can be detected by the presence of highly echogenic lungs.     

Meckel's diverticulum induced intrauterine intussusception associated with ileal atresia complicated by meconium peritonitis.

Intrauterine intussusception with a leading point of Meckel's diverticulum is a rare cause of ileal atresia, which may cause bowel obstruction and perforation. We report such a case complicated by meconium peritonitis. The fetal ultrasonogram revealed ascites, dilated bowel loops and intra-abdominal calcification at a gestational age of 30 weeks. The patient was delivered at 37 weeks and laparotomy was performed to manage the intestinal obstruction. The operative findings showed that Meckel's diverticulum had induced intussusception associated with the ileal atresia with meconium peritonitis. The ileum was resected with end-to-end anastomosis. The postoperative course was uneventful. In this patient, ascites and intraperitoneal calcification were caused by ileal atresia, which may have been induced by intrauterine intussusception.     

Intestinal stenosis caused by perinatal intussusception in a full-term neonate

Perinatal intussusception is rare, but can be seen in both premature and full-term neonates. The symptoms and signs are similar to those seen in necrotizing enterocolitis (NEC), causing the diagnosis of intussusception to be delayed. We report a case of intussusception that presented with features suggestive of NEC in a 3.4-kg, full-term female neonate. Diagnosis of intussusception was made with a plain abdominal x-ray and abdominal ultrasound. During surgery, an ileocolic intussusception with intestinal stenosis was found. Although intestinal stenosis and atresia are commonly found in premature infants, these entities can also occur in a full-term infant. Early diagnosis is needed to reduce the high morbidity and mortality in these infants.     

Duodenal and biliary atresia associated with facial, thyroid and auditory apparatus abnormalities: a new mandibulofacial dysostosis syndrome?

We report a female child born at 36 weeks of gestation with multiple abnormalities including dysmorphic and coarse facial features with features of mandibulofacial dysostosis that include bilateral microtia with the absence of external auditory meati and Mondini dysplasia as well as, duodenal atresia, intestinal malrotation, anterior displacement of the anus, left hemiaplasia of the thyroid and biliary atresia in sibs. The associations of duodenal atresia with intrahepatic and extrahepatic biliary atresia in sibs have been reported, suggesting an autosomal recessive syndrome. However, the associated external, middle and internal ear anomalies and the thyroid malformation, however, have not been reported in this condition. To the best of our knowledge, this is a hitherto new syndrome with an unknown inheritance.     

Umbilical cord ulcer associated with fetal jejunal atresia: report of 2 cases

We report 2 cases of umbilical cord ulcer associated with fetal jejunal atresia. Both of them developed a severe intrauterine hemorrhage, followed by fetal heart rate decelerations, and underwent emergency cesarean section. Bloody amniotic fluid and umbilical cord ulcers were observed in both cases. Although both cases were successfully resuscitated, neurological impairment and renal failure developed in 1 case due to prolonged asphyxia. In a review of the literature, umbilical cord ulcer was associated only with congenital duodenal atresia or jejunal atresia, but not with ileal atresia. Although the prenatal diagnosis of duodenal or upper jejunal atresia has been established, the prenatal diagnosis of this complication has not been reported. In such cases, detailed examination of the umbilical cord by ultrasonography may be useful for the prenatal diagnosis of this disease.     

Intrauterine umbilical cord hemorrhage with associated jejunal atresia captured by real-time ultrasound

The presence of unexplained umbilical cord ulceration and hemorrhage has been sporadically reported in fetuses with antenatally suggested intestinal atresia. This case report illustrates a patient with spontaneous intrauterine umbilical cord hemorrhage, captured by real-time ultrasonography, in the setting of jejunal atresia with volvulus of the distal jejunal segment.     

Pregnancy following repair of a congenital atresia of the uterine cervix and upper vagina

Summary A case of congenital atresia of the uterine cervix combined with atresia of the upper vagina and without any other anomalies is reported. Direct anastomosis of cervix and vagina resulted in a restoration of reproductive function. The patient conceived 6 years afterward and delivered a healthy boy by cesarean section at 31 weeks of gestation. Therapeutic reconstructing options for this kind of genital tract malformation with respect to reproductive function are discussed.     

Gastroschisis complicated by midgut atresia, absorption of bowel, and closure of the abdominal wall defect

We present a case of gastroschisis that was associated with progressive resorption of the extra-abdominal bowel loops and dilation of intra-abdominal bowel loops. After preterm delivery at 32 weeks, a small paraumbilical remnant was present. There was complete atresia of most of the jejunum, ileum, cecum, and the proximal half of the transverse colon. At laparotomy, the jejunum was anastomosed to the transverse colon. The neonate developed short gut syndrome and eventually received a liver and intestinal transplant.     

Role of intraluminal bowel echogenicity on prenatal ultrasounds to determine the anatomical level of intestinal atresia

Objective: To evaluate the correlation between different degrees of bowel intraluminal echogenicity showed by prenatal ultrasounds and the anatomic level of intestinal atresia.

Methods: We report three cases of intestinal atresia at different intestinal levels verified during the neonatal surgery with specific ultrasonographic prenatal features. Intensity of sonolucency was analyzed using the image-processing program ImageJ for quantitative measurements based on the gray-scale intensity values.

Results: A total of three cases are reported, a jejunal, an ileal and a colonic atresia. All cases showed intestinal dilatation. Both, jejunal and ileal atresia, showed two degrees of hypoechoic intestinal content, while colonic atresia showed hyperechogenic content dilated loop at prenatal ultrasound scan.

Conclusions: We propose the use of prenatal ultrasounds echogenicity of intestinal dilated loop fluid content to help in determining the level of obstruction in bowel atresia. These are initial results, to be confirmed by a multicentric research with more cases.     

Fetal hemorrhage associated with congenital intestinal atresia

We encountered two cases of severe intrauterine hemorrhage associated with congenital intestinal atresia. In both cases, the first sign that necessitated immediate clinical management was the abnormal fetal heart rate patterns, represented by prolonged bradycardias and late decelerations. This occurred immediately after the onset of labor. An emergency cesarean section was performed on both patients and, despite being born with severe anemia, the condition of the infants was excellent. We found that continuous fetal heart rate monitoring should be performed if polyhydramniosis suspected, when the fetus is diagnosed with congenital intestinal atresia and when changes in intrauterine pressure are clinically expected, such as at the onset of labor or after amniocentesis.     

Fetal laryngeal stenosis/atresia and congenital high airway obstructive syndrome (CHAOS): a case report.

Congenital high airway obstructive syndrome (CHAOS) is a rare but fatal disease with predictably characteristic features including stenotic or atretic upper airway, hyperplastic lungs, elevated diaphragm, massive fetal ascites and fetal hydrops. Diagnosis of CHAOS by ultrasound scan is possible and clinically important since advanced intrauterine surgery to correct the defect is possible. We report a case of fetus of CHAOS with massive ascites, pulmonary hyperplasia and laryngeal stenosis/atresia. We feel that it is important to recognize the entity both by ultrasound scan and by the pathologist so that some cases can be corrected by intrauterine fetal surgery.     

Jejunal atresia with 'apple peel' deformity. A report of eight survivors

Jejunal atresia with an "apple peel" deformity is one of the most severe forms of intestinal atresia. Until quite recently, neonatal mortality rates as a result of this anomaly were high. Early diagnosis, appropriate surgery, and prolonged parenteral nutrition have led to a spectacular improvement of the survival rates. We are reporting eight cases of apple peel atresia--all eight patients survived. The pathophysiology, diagnostic aspects, surgical approaches, and postoperative management are discussed.