Intrauterine hemorrhage from an umbilical cord ulcer associated with fetal duodenal atresia

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Slide 1 : Intrauterine hemorrhage from an umbilical cord ulcer associated with fetal duodenal atresia Nobuhiro Hidaka, M.D Department of Obstetrics and Gynecology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
Slide 2 : Abstract 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.
Slide 3 : Introduction The prognosis of intestinal atresia has improved in the recent years due to the advances in neonatal surgical techniques and perioperative management. On the other hand, the main causes of death are various combinations of infections, brain hemorrhage, and short-bowel syndrome and other associated severe anomalies. In addition to the abovementioned primary causes of death, it was recently established that the association between congenital intestinal atresia and umbilical cord ulceration might be the cause of sudden death. Yet, to the best of our knowledge, only 14 cases on this association have been published previously. Here, we present a case of intrauterine hemorrhage from an umbilical cord ulcer in a fetus with duodenal atresia that resulted in early neonatal death.
Slide 4 : Case report A 38-year-old Japanese woman, gravida 10, para 5, was presented for prenatal care at 7 weeks of gestation. She had a history of one spontaneous abortion and four artificial abortions. At 19 weeks of gestation, ultrasound examination revealed fetal anomalies including left isomerism and visceral inversion of the stomach. Genetic analysis of the amniotic fluid revealed a 46,XX karyotype. At 29 weeks of gestation, she was admitted to the hospital because of progressive polyhydramnios and an effaced uterine cervix. On hospitalization, her blood pressure was 126/74 mmHg, and no proteinuria or edema was observed. A careful ultrasound examination after hospitalization demonstrated a fetal “double bubble” sign (Figure 1); this suggested the presence of fetal duodenal atresia. The largest amniotic fluid pocket was 12 cm. We administered magnesium sulfate intravenously to achieve tocolysis. Further, we performed amnioreduction once a week and removed 800-1000 ml of amniotic fluid per puncture. A nonstress test that was performed daily showed that the fetal status was good without any deceleration or bradycardia. Her general condition was stable without any sign of pregnancy- induced hypertension.
Slide 5 : Figure 1. A typical “double bubble” sign was observed in the fetal abdomen following ultrasonographic examination at 29 weeks of gestation.
Slide 6 : However, at 35 weeks and 2 days of gestation, i.e., 5 days after the last amnioreduction, the nonstress test unexpectedly revealed continuous fetal bradycardia. At that time, the mother had no complaints of abdominal pain or genital bleeding, and her vital signs showed no remarkable change. We performed an emergent cesarean section, and a female infant was born. The amniotic fluid was blood-tinged. The birth weight of the infant was 2675 g, and the umbilical artery pH was 6.92, pO2 being 23.1 mmHg, pCO2 92.1 mmHg and base excess -16.2 mmol/l. Extremely low Apgar scores of 1 at 1 minute and 2 at 5 minutes were noted, and the infant required immediate cardiopulmonary resuscitation; therefore, she was supported with a mechanical ventilator from birth. The infant looked pale and her hemoglobin concentration was only 4.8 g/dl. The infant’s condition remained life-threatening, and her general condition as well as severe acidosis did not improve despite aggressive treatment that included the administration of epinephrine, sodium bicarbonate, and calcium gluconate. Finally, early neonatal death occurred three hours after her birth. Autopsy revealed duodenal atresia, left isomerism of the bilateral lung, and visceral inversion of stomach, spleen with polysplenia, liver, pancreas, and inferior vena cava. No intracardiac structural defect was detected. Umbilical cord ulceration without Wharton’s jerry was identified around the umbilical arteries by macroscopic (Figure 2) and microscopic histological examinations. These examinations revealed that the lumen of the umbilical artery had partially lost its wall and was exposed due to the loss of Wharton’s jelly. Based on these findings, intrauterine hemorrhage due to umbilical cord ulceration was considered to be the cause of continuous fetal bradycardia. Apart from the perforation site of the umbilical cord, Wharton’s jelly was extremely thin in a relatively large area near the placental side; this was considered to be indicative of perforation.
Slide 7 : Figure 2. Perforation site of the umbilical cord ulcer with an adherent clot (arrow)
Slide 8 : Outcome of 15 cases showing the association between umbilical cord ulceration and fetal intestinal atresia
Slide 9 : Comment 1 The pathway of the development of umbilical cord ulceration still remains unclear. Bendon et al., who first reported about the case of umbilical cord ulcer with fetal intestinal atresia in 1991, speculated that an abnormal vascular reactivity or an epithelial abnormality was the initial cause; but no later report could demonstrate this hypothesis to date. Ohyama et al. had assumed that the toxicity of gastric or intestinal contents, including bile regurgitated into the amniotic fluid, was the initiating cause. In all the 15 cases reported thus far, including our case, the site of atresia was limited to the duodenum or the jejunum. Esophageal atresia or ileal atresia had not been noted. This fact may elucidate Ohyama’s opinion. We believe that further studies regarding the analysis of amniotic fluid, including pH, ?OD450, and measurements of bilirubin, bile acids, amylase concentration and so on, will clarify the pathway of umbilical cord ulceration in the future.
Slide 10 : Comment 2 In 2002, Brantberg et al. reported an increased risk of sudden and unexpected intrauterine death of fetuses with duodenal obstruction. Ohyama et al. reported 44 cases of fetuses with intestinal atresia, six of which showed hemorrhage from umbilical cord ulcer. Although only 15 cases have been reported to date, it is possible that the true incidence of this complication is even higher than that estimated currently. Greater understanding of this association is required. Although in this case we could not rescue the affected infant, 6 other cases of survival have been reported. Close observations and frequent fetal monitoring may prevent sudden fetal demise. However, it is possibly the overmanagement to hospitalize all patients in whom fetuses have been diagnosed with intestinal atresia. In future, it will be important to establish some methods that will facilitate the identification of the high-risk cases. For example, the detailed ultrasonographic quantitative examination of Wharton’s jelly may be useful. In our case, only one bleeding spot was detected; however, Wharton’s jelly was extremely thin in the other parts. Ulceration occurred over a relatively extensive area. The quantitative evaluation of Wharton’s jelly by an ultrasound examination has been reported to be possible, and currently it is possible to obtain a nomogram of the Wharton’s jelly area. This may be applied to the in utero diagnosis of umbilical cord ulcers. Further investigation with sufficient accumulation of data from similar cases is required to establish the management protocol.
Slide 11 : Bendon RW, Tyson RW, Baldwin VJ, Cashner KA, Mimouni F, Miodovnik M (1991) Umbilical cord ulceration and intestinal atresia: A new association? American Journal of Obstetrics & Gynecology 164:582-586 Khong TY, Ford WDA, Haan EA (1994) Umbilical cord ulceration in association with intestinal atresia in a child with detection 13q and Hirschsprung’s disease. Archives of Disease in Childhood Fetal & Nenatal Edition 71:F212-213 Khurana A, Huettner PC, Cole FS (1995) Umbilical cord ulceration as a cause of hypoxic-ischemic encephalopathy: report of a case and review of the literature. Journal of Perinatology 15:423-425 Yamanaka M, Ohyama M, Koresawa M, Kawataki M, Ohsaki I, Tanaka Y (1996) Umbilical cord ulceration and intestinal atresia. European Journal of Obstetrics & Gynecology and Reproductive Biology 70: 209-212 Ohyama M, Itani Y, Yamanaka M, Imaizumi K, Nishi T, Ijiri R, Tanaka Y (2000) Umbilical cord ulcer: a serious in utero complication of intestinal atresia. Placenta 21:432-435 Shimizu S, Kawagishi R, Wada K, Ishida E, Shimoya K, Murata Y (2003) Fetal hemorrhage associated with congenital intestinal atresia. Journal of Obstetrics & Gynecology Research 29:312-316 Anami A, Morokuma S, Tsukimori K, Kondo H, Satoh S, Nozaki M, Sueishi K, Nakano H (2006) Sudden fetal death associated with both duodenal atresia and umbilical cord ulcer, a case and review. American Journal of Perinatology 23:183-188 Brantberg A, Blaas HG, Salvesen KA, Haugen SE, Mollerlokken G, Eik-Nes SH (2002) Fetal duodenal obstructions: increased risk of prenatal sudden death. Ultrasound in Obstetrics and Gynecology 20:439-446 Ghezzi F, Raio L, Dinaro E, Franchi M, Balestreri D, D’addario V (2001) Nomogram of Wharton’s jelly as depicted in the sonographic cross section of the umbilical cord. Ultrasound in Obstetrics and Gynecology 18:121-125 Weissman A, Jakobi P, Bronshtein M, Goldstein I (1994) Sonographic measurements of the umbilical cord and vessels during normal pregnancies. Journal of Ultrasound in Medicine 13:11-14 References

 


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