Fetal midgut herniation into the umbilical cord: improved definition of ventral abdominal anomaly with the use of transvaginal sonography

Abstract

The most common anomalies of the fetal ventral abdominal wall include omphalocele and gastroschisis. Umbilical cord hernia is another abdominal wall defect that is poorly defined and usually mistakenly considered as a small omphalocele. The present report describes the sonographic features and clinical significance of four cases of umbilical cord hernia identified transvaginally in the early second trimester of pregnancy. These cases seemed to present a different entity from that of simple omphalocele. The transvaginal sonographic approach provided a clear image of the midgut protruding into the umbilical cord, precise localization of cord insertion in the region of the umbilical ring. Doppler flow evaluation of the umbilical vessels and their relation to the protruding mass. Serial sonographic observations revealed a stable umbilical cord mass in three fetuses, and an enlarging mass in one. Normal karyotype was determined and no associated malformations were detected prenatally. However, in one case that underwent immediate correction of the hernia after delivery at term, the neonate was subsequently found to have pulmonic stenosis and severe neonatal seizures developed at 4 months of age. In two cases, pregnancy was terminated due to parental request. The fourth fetus was delivered vaginally at term and catastrophic division of the umbilical cord containing a loop of small intestine was avoided only by the diligent observation of the midwife. We suggest that umbilical cord hernia is a distinct anomaly originating at a different stage of embryogenesis, thereby having a unique clinical significance, unlike simple omphalocele. Umbilical cord hernia should therefore be defined and considered as a separate entity.

Mouse mutant embryos overexpressing IGF-II exhibit phenotypic features of the Beckwith–Wiedemann and Simpson–Golabi–Behmel syndromes

“…At E15.5, the intestine begins to return to the peritoneal cavity, and the body wall closes by E16.5 (only the umbilical cord and its contents remain in this region ;Pleeging 1977;Kaufman 1992). A similar physiological umbilical hernia is transiently present in the human embryo (see Duhamel 1963;Achiron et al 1995;VermeijKeers et al 1996). By the 10th to 12th gestational week, however, the gut returns to the abdominal cavity and the apices of cephalic, caudal and lateral folds forming the coelom come together at the umbilical ring.…”

“…By the 10th to 12th gestational week, however, the gut returns to the abdominal cavity and the apices of cephalic, caudal and lateral folds forming the coelom come together at the umbilical ring. Failure of the intestine to return to the coelom results in an umbilical hernia (Achiron et al 1995). In this ventral wall defect, the folds develop normally, the umbilical ring is intact, the protrusion is covered with skin, and the herniation does not include any organ other than midgut present inside the umbilical cord.…”

“…In this ventral wall defect, the folds develop normally, the umbilical ring is intact, the protrusion is covered with skin, and the herniation does not include any organ other than midgut present inside the umbilical cord. An omphalocele (exomphalos), on the other hand, results from a failure of the lateral folds to progress (Duhamel 1963;Achiron et al 1995). Thus, the ventral wall defect is large and an umbilical ring is not formed.…”

Evaluation of First-Trimester Physiological Midgut Herniation Using Three-Dimensional Ultrasound

“…Failure may result in a (pathological) congenital omphalocele. Since this does not explain the possible herniation of other organs such as the liver, Achiron et al [11] differentiated between an omphalocele caused by failure to form the primitive umbilical ring and a failed return of the midgut from the umbilical cord. This would also explain the spontaneous resolution of small omphaloceles.…”

Prenatal Diagnosis of a Fetal Abdominal Eventration: A Rare Congenital Abdominal Wall Defect

“…The two most common abnormalities are omphalocele and laparoschisis [1,2] . Intrafunicular hernia is less common, and corresponds to the midgut protruding into the umbilical cord through a patent umbilical ring without parietal defect [3,4] . and visible peristaltism ( fig.…”