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.     

Sudden fetal death associated with both duodenal atresia and umbilical cord ulcer: a case report and review

We encountered one case of duodenal atresia complicated by massive intrauterine hemorrhage due to the perforation of an umbilical cord ulceration (UCU). UCU is an extremely rare complication in the perinatal period. Although the prenatal diagnosis of upper intestinal atresia has been established, little is known about the association between UCU and upper intestinal atresia. In this article, we report our case, review past articles, and discuss the underlying pathophysiological mechanisms of the cause of an UCU. Given the characteristic sites of upper intestinal atresia, we speculate that regurgitation of gastric or intestinal juice into the amniotic fluid could be responsible for the development of UCU. We also believe that close observation is required for patients who have upper intestinal atresia.     

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 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.     

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.     

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.     

Umbilical cord ulceration and intestinal atresia: a new association?

In three fetuses, congenital intestinal atresia was associated with linear ulcerations of the umbilical cord. In two cases, hemorrhage was seen from the cord ulcer. Both fetuses required emergency cesarean section for fetal distress and were born anemic. The third fetus was mildly hydropic, attributed to hemorrhage, and was stillborn. The mechanism of the association could not be determined. These cases suggest a risk of prenatal umbilical cord hemorrhage in infants with intestinal atresia.     

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.     

Umbilical cord knots

BACKGROUND: Umbilical cord knots may represent a hazard to the fetus, particularly as regards intrauterine death and fetal distress or asphyxia in labor. The object of this study was to analyze the impact of associated umbilical cord encirclements and cord length on fetal outcome and fetal weight deviation. METHODS: Among 22,012 births occurring in Akershus Central Hospital, there were 216 instances of umbilical cord knots. Fetal outcome, fetal weight deviation, associated umbilical cord encirclements and cord length were assessed. RESULTS: Neonates with a knotted cord are more often large-for-gestational age compared to other babies, and have longer umbilical cords. There is a 10 times higher chance of intrauterine fetal death with a knotted cord, but if this does not occur then there is no increased risk of obstetrical intervention and Apgar scores are the same as in other babies and other fetuses. CONCLUSION: There is an association between umbilical cord knots and umbilical cord encirclements. Knotting of the cord is not by itself lethal. Pregnancies with knotted cords have characteristics different from those with ordinary umbilical cord encirclements.     

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.     

Intestinal polyp of the umbilical cord

The morphology, pathogenesis, complications and differential diagnosis of an intestinal polyp of the umbilical cord are presented. The polyp were detected postnatal on the umbilical cord in an healthy male newborn. The presents of intestinal tissue upon the umbilical cord ist possible about the persistence from remnants of the ductus omphalomesentericus with prolapse and differentiation of the intestinal cells. The ductus omphalomesentericus is a tubular structure, a communication between the developing embryonic gut and the yolk sac, forming during the early embryonic life. Obliteration of the omphalomesenteric duct is usually complete by the 10(th) week of gestation. Various portions of the duct may persist, however, giving rise to polyps, fistulas or cysts of the umbilical cord with potentially dangerous clinical consequences. Other tumors of the umbilical cord are myxoma, angioma and teratoma are differential diagnosis.     

Intrauterine fetal death after multiple umbilical cord torsion—complication of a twin pregnancy following assisted reproduction

BACKGROUND: Patients requiring assisted reproductive techniques may have a higher rate of congenital malformations. Some rare complications of pregnancy might be related to such abnormalities. Torsions of the umbilical cord resulting in fetal death have previously been described exclusively in pregnancies following spontaneous conception. CASE: The case of 37 year old gravida I, para O woman with a twin pregnancy after intracytoplasmatic sperm injection and intrauterine death of one twin at approximately 30 weeks' gestation is presented. The surviving twin was delivered by cesarean section at 31 weeks after spontaneous onset of labor and recurrent fetal bradycardia. The intraoperative situs showed that the demised twin had suffered from multiple umbilical cord torsions leading to intrauterine hypoperfusion. CONCLUSION: Umbilical torsion leading to fetal death might represent a previously unrecognized complication in women requiring assisted reproductive techniques, but this problem is known to occur in pregnancies achieved by natural methods.     

Aneurysm of the umbilical vein: case report and review of literature

We report a case of aneurysm of the umbilical vein, causing fetal death at 41 weeks gestation. We conclude that these aneurysms are a complication of congenital thinning of the vessel wall and want to emphasize that in stillbirths the cause of death may only be revealed by careful placental examination, including the umbilical cord.     

A stillborn fetus with amniotic band syndrome and elevated levels of alpha-fetoprotein plus beta-human chorionic gonadotropin: a case report

Amniotic band syndrome is an uncommon, congenital fetal abnormality with multiple disfiguring and disabling manifestations. A wide spectrum of clinical deformities are encountered and range from simple ring constrictions to major craniofacial and visceral defects. We report a case of constriction amniotic bands involving upper extremities and intrauterine fetal death due to strangulation of umbilical cord. Abnormally elevated levels of alpha-fetoprotein and beta-chorionic gonadotropin were detected at 17 weeks' gestation. They were probably caused by the loss of cutaneous integrity of the fetus (alpha-fetoprotein), and by the placental attempt to counteract the fetal growth restriction and hypoxia, due to the strangulation of umbilical cord by the amniotic bands (beta-chorionic gonadotropin).     

Body stalk anomaly--a case report on a rare congenital defect

INTRODUCTION: A body stalk anomaly is a rare congenital defect. The anomaly is one of the anterior abdominal wall defects. It is characterized by severe clefts of the abdominal wall with absence of or very small, umbilical cord. CASE REPORT: We report on a patient in the 21st week of gestational age with the ultrasonographic findings of multiple fetal anomalies including a singular umbilical artery and an ahydramnion. She was sent to hospital for termination of the pregnancy. The karyotype was normal (46 XX). Ultrasonographic evaluation of the fetal anterior abdominal wall was impossible due to ahydramnion. Clinical examination showed an omphalocoele with herniation of the stomach, the small intestine, the liver and the spleen. A very short umbilical cord was observed which was attached to the omphalocoele. The lower limbs were deformed. Furthermore, anal atresia with an extroverted coele was found. Autopsy confirmed the clinical diagnosis of a body stalk anomaly. DISCUSSION: It is important to distinguish a body stalk anomaly from other types of anterior abdominal wall defects because of their prognosis and an appropriate medical management. A body stalk anomaly is a sporadic defect and not associated with chromosomal abnormalities. This congenital defect is invariably lethal.     

Single umbilical artery and umbilical cord torsion leading to fetal death. A case report

BACKGROUND: Intrauterine fetal death is a complication that cannot often be predicted by standard obstetric management. Cord accident may be responsible for about 5% of cases. Umbilical cord torsion is an extremely rare cause of intrauterine fetal death. CASE: An 18-year-old, nulliparous woman presented with a complaint of decreased fetal movement at 38 weeks' gestation. Intrauterine fetal death was diagnosed on ultrasound. The pathologic examination revealed umbilical cord torsion and confirmed a single umbilical artery that was diagnosed on ultrasound. CONCLUSION: Umbilical cord torsion that leads to intrauterine fetal death is extremely rare. A pregnancy with a single umbilical artery may need fetal monitoring during the third trimester.     

Hemangioma of the umbilical cord: stenotic change of the umbilical vessels

We report a rare case of an umbilical cord hemangioma diagnosed by ultrasound at 16 weeks of gestation. The umbilical cord consisted of a hemangioma nodule and pseudocysts near the placental insertion, a large gelatin-like swelling adjacent to the nodule on its fetal side, and a short normal part extending to the navel. At 17 weeks of gestation, this condition resulted in the intrauterine death of the fetus. Microscopically, there were communications between the capillary of the hemangioma and the umbilical vessels, verifying the origin of the tumor. Moreover, the umbilical vein and one of the arteries changed stenotically due to the intravascular proliferation of the hemangioma. These findings indicate the possibility of a pathological association between the umbilical cord hemangioma and fetal demise due to impaired umbilical circulation.     

Antenatal sonographic detection of single umbilical artery.

The absence of one umbilical artery of single umbilical artery (SUA) is one of the most common congenital malformations in man. This vascular anomaly of the umbilical cord is frequently associated with other congenital malformations as well as some adverse perinatal events such as intrauterine growth retardation (IUGR), premature delivery, and increased perinatal mortality. Five cases of SUA detected prenatally by ultrasound are reported here in detail, including the first reported case in a twin gestation. None of the 5 affected infants had associated anomalies, but 2 cases of intrauterine growth retardation (IUGR) and 1 stillborn infant were noted in this series. An umbilical vein/umbilical artery ratio less than 2 was invariably found in all cases, making this observation another useful sonographic characteristic to use in the antenatal detection of SUA. Since the umbilical cord can be easily seen prenatally by ultrasound, and SUA is recognized as an important index for detecting congenital malformations, examination of the umbilical cord for the absence of one umbilical artery is an extremely valuable tool in prenatal diagnosis. The prenatal detection of SUA demands an extensive search for associated anomalies and a close surveillance of fetal well-being, since these fetuses have a high risk of fetal death or IUGR. Sonologists and sonographers should be aware of the possibility of SUA, especially in those cases associated with congenital malformations or IUGR.     

Monoamniotic twins: an update on antenatal diagnosis and treatment.

OBJECTIVE: Monoamniotic twins are very uncommon and are characterized by a high perinatal mortality rate. Cord entanglement, prematurity, congenital anomalies and twin to twin transfusion are reportedly the main causes of death, which usually occurs before 24 weeks' gestation. The aim of this study was to review the newly developed methods for diagnosis and treatment and suggest a reasonable approach to the management of these rare cases. METHOD: We reviewed the English-language literature in the past 15 years through Medline search and subsequent examination of individual publications. RESULTS: In the past two decades, newer technologies such as advanced ultrasonography and color flow Doppler studies have enabled early diagnosis of this condition and its complications. Furthermore, new treatment modalities such as fetoscopy with laser coagulation of vascular anastomoses and treatment with non-steroidal anti-inflammatory drugs such as sulindac, to reduce amniotic fluid volume, may have contributed to a better outcome. Also, occlusion of one umbilical cord (fetal reduction) at an early stage has been suggested to prevent late complications. CONCLUSION: Cumulative experience suggests that the majority of cases can be diagnosed reliably at an early gestational age. Treatment with medical amnioreduction, surgical amnioreduction or fetal reduction in selected cases may be offered before 24 weeks' gestation. Later, intensive antepartum fetal surveillance should probably be offered until 32 weeks, at which point elective preterm delivery may be considered to prevent possible fetal death.     

Uterovaginal anastomosis for the management of congenital atresia of the uterine cervix

Congenital atresia of the cervix is a rare mullerian anomaly. Hysterectomy has been advocated as the management of choice in the early days as the reproductive performance is thought to be low despite successful neo-canal creation. In recent years, conservative surgery is being recommended more frequently in patients with congenital cervical atresia and with total or partial vaginal aplasia and is shown to have a better reproductive performance. The treatment strategy should be tailored to relieve retrograde menstrual symptoms and restore fertility. Here, we report a young girl with congenital cervical atresia with upper vaginal atresia managed with uterovaginal anastomosis and review the management options and reproductive performance in such cases.