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      Perinatal Survival of a Fetus with Intestinal Volvulus and Intussusception: A Case Report and Review of the Literature

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          Abstract

          Fetal intestinal volvulus is a rare life-threatening condition. Late diagnosis of volvulus contributes to high rate of morbidity and mortality. It has variable degrees of presentation and survival. Intrauterine volvulus may be complicated by intestinal atresia due to ischemic necrosis. To our knowledge, there are three reported cases of term fetal demise. We report a case of fetal intestinal volvulus with perinatal survival of the largest term infant described with this complication to date. The volvulus was associated with type 3A jejunal atresia and intestinal pathology was noted on prenatal ultrasound. The infant was born via urgent cesarean delivery at 37 6/7 weeks of gestation and underwent emergent exploratory laparotomy with resection of small bowel and primary end-to-end anastomosis. Intrauterine intestinal volvulus may be suspected on prenatal ultrasound but only definitively diagnosed postnatally. Signs of fetal distress and volvulus are rarely associated with reports of survival in the term fetus. We review reported cases of prenatally suspected volvulus in infants documented to survive past the neonatal period. As fetal volvulus and most intestinal atresias/stenoses manifest during the third trimester, we recommend that the limited fetal anatomical survey during growth ultrasounds at 32 to 36 weeks routinely include an assessment of the fetal bowel.

          Most cited references22

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          Midgut volvulus causing fetal demise in utero.

          Intestinal malrotation has an incidence of 1 per 6000 live births. The most serious consequence of malrotation is volvulus. Midgut volvulus is a rare condition in which the small bowel and proximal colon twist around the superior mesenteric artery, leading to a high-grade proximal bowel obstruction and vascular compromise of the intestine, thereby leading to infarction of the involved intestine. Midgut volvulus rarely occurs antenatally and is usually not lethal in utero. There are only 7 cases of intrauterine fetal demise caused by midgut volvulus reported in the literature. We report a case of intrauterine fetal demise at 38 weeks of gestation, due to cardiovascular failure and shock from midgut volvulus. Non-specific abnormalities, including ascites and dilated bowel, had been seen on the antenatal ultrasound from the 15th week of gestation. In addition to the volvulus, the fetus had intestinal atresia and arthrogryposis.
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            Definitive diagnosis of intestinal volvulus in utero.

            Midgut volvulus with or without intestinal malrotation can occur in fetal life. Several reports have described congenital midgut volvulus showing non-specific sonographic findings of intestinal obstruction and perforation in utero. None of the previously reported cases, however, were definitively diagnosed as midgut volvulus by fetal sonography. We report two cases both exhibiting the sonographic 'whirlpool' sign, in utero. Color Doppler interrogation provided a clue to the viability of the involved intestinal segment.
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              Intrauterine midgut volvulus without malrotation: diagnosis from the 'coffee bean sign'.

              Fetal midgut volvulus is quite rare, and most cases are associated with abnormalities of intestinal rotation or fixation. We report a case of midgut volvulus without malrotation, associated with a meconium pellet, during the gestation period. This 2.79 kg, 33-wk infant was born via a spontaneous vaginal delivery caused by preterm labor. Prenatal ultrasound showed dilated bowel loops with the appearance of a 'coffee bean sign'. This patient had an unusual presentation with a distended abdomen showing skin discoloration. An emergency laparotomy revealed a midgut volvulus and a twisted small bowel, caused by complicated meconium ileus. Such nonspecific prenatal radiological signs and a low index of suspicion of a volvulus during gestation might delay appropriate surgical management and result in ischemic necrosis of the bowel. Preterm labor, specific prenatal sonographic findings (for example, the coffee bean sign) and bluish discoloration of the abdominal wall could suggest intrauterine midgut volvulus requiring prompt surgical intervention.
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                Author and article information

                Journal
                AJP Rep
                AJP Rep
                AJP Reports
                Thieme Medical Publishers (333 Seventh Avenue, New York, NY 10001, USA. )
                2157-6998
                2157-7005
                11 July 2013
                October 2013
                : 3
                : 2
                : 107-112
                Affiliations
                [1 ]Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas
                [2 ]Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas
                [3 ]Department of Surgery, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas
                [* ]Esohe Ohuoba and Gary Fruhman contributed equally to this manuscript.
                Author notes
                Address for correspondence Nikolaos Zacharias, MD, FACOG Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology Baylor College of Medicine, One Baylor Plaza, HoustonTX 77030 nmzach@ 123456yahoo.com
                Article
                13r0012
                10.1055/s-0033-1349367
                3799706
                24147247
                9963e223-a972-480c-be15-086121d44b06
                © Thieme Medical Publishers
                History
                : 20 March 2013
                : 05 June 2013
                Categories
                Article

                fetal intestinal volvulus,perinatal mortality,intussusception,intrauterine volvulus

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