Abnormal Intestinal Motility in
Gastroschisis 1
Pediatric Radiology
Kook Sang Oh, M.D., John P. Dorst, M.D.,2 Rodrigo Dominguez, M.D., and Bertram R. Girdany, M.D. The authors describe abnormal intestinal motility with a very slow transit time in eight children with gastroschisis. Other abnormal findings include malrotation, dilatation, and fixation of the loops of the bowel. INDEX TERMS: Gastrointestinal tract, abnormalities. (GI system, gastroschisis, omphalocoele, 7 [0]. 1495) • Infants, newborn, digestive system • Intestines, abnormalities • Stomach, abnormal ities
Radiology 127:457-460, May 1978
HE
Shaw (8), in his recent article "The Myth of Gastroschisis", states that gastroschisis is the result of in utero rupture of the membrane covering a hernia of the umbilical cord either during its normal phase (fifth through tenth week of fetal life) or at a later stage in embryos in which closure of the normally formed umbilical ring has not occurred. Because embryologists have thus far not given us unequiovocal evidence of "gastroschisis" in early human fetal specimens, current concepts of an embryogenesis for "gastroschisis" distinct from that of hernia of the umbilical cord should be discraded. The herniated viscera through the defect consists mainly
term gastroschisis means "belly separation"
T from the Greek words gastro (belly) and schism (sep-
aration). Gastroschisis is defined as an extraumbilical abdominal wall anomaly, in which there is a normal cord insertion with nonrotation of an abnormally short midgut, without a sac or a sac remnant (1). It occurs in one of every 6,000 live births, and about 40% of those afflicted are premature infants. Little attention has been paid to the radiological findings in gastroschisis (4, 9). In this paper we describe abnormal intestinal motility during gastrointestinal examinations at various periods after surgical repair of gastroschisis. MATERIALS AND METHODS
We studied eight patients within a period of 6 years. Each had gastrointestinal examinations with barium for evaluation of intestinal motility and morphology, and all underwent emergency surgical repair. They received intravenous hyperalimentation for a varying period of time. RESULTS
The results are summarized in TABLE I. DISCUSSION
The closing of the abdomen of the embryo is accompanied by the circumferential folding of 4 folds (3). These folds are the condensation of embryonic mesenchyma and are called the cephalic, the caudal, and the two lateral folds. Each fold has somatic and splanchnic layers. The somatic layer of the cephalic fold forms the thoracic and epigastric walls. The somatic layer of the caudal fold forms the hypogastric wall, while the somatic layers of the lateral form the two lateral walls of the abdomen. The apex of these folds forms the future umbilical ring. Failure of localized differentiation and subsequent resorption of embryonic mesenchyma in any of these folds results in a circumscribed defect.
Fig. 1. CASE I. Barium outlines the dilated duodenum and proximal jejunum. The intestinal mucosa is edematous.
From the Department of Radiology, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania. From the Department of Radiology and Radiological Science, Johns Hopkins Hospital, Baltimore, Maryland. Accepted for publication in January emt 1978. 1
2
457
458
KOOK SANG OH AND
Fig. 2. rant.
CASE II.
Fig. 3. CASE VI. show malrotation.
OTHERS
May 1978
Three examinations on three different days show fixation of the loops of the dilated small bowel in the right upper quad-
The dilated duooenum and proximal jejunum
of small and large intestine, although often the entire gastrointestinal tract from stomach to rectum is eviscerated (5, 9). In the majority of infants with gastroschisis the viscera becomes incarcerated within the abdominal wall opening during early gestation. The eviscerated mass is edematous and covered by a fibrinogelatinous membrane containing squamous cells and vernix from its in utero exposure to amniotic fluid. The matrix is often contaminated by the meconium and bacterial organisms at or just following birth. Most striking is the gross shortening and coiling of the intestine. Peristalsis is impaired (9). On the bases of appearance of the herniated bowel and the size of the peritoneal cavity, Moore (7) classifies them into two major types, antenatal and perinatal. In the antenatal type, the evisceration is presumed to have occurred during early pregnancy, with long-standing exposure to amniotic fluid, thick matted bowel covered with a gelatinous matrix, and abnormally small peritoneal cavities. However, the perinatal type has minimal serosal reaction with normal size of the peritoneal cavity and closure of the
TABLE I: SUMMARY OFRADIOLOGICAL FINDINGS CASES
II III IV V VI VII
VIII
AGE AT THE TIME OF EXAM
DILATION
FIXATION
MALROTATION
TRANSIT TIME
2 months 4 months 6 months 6 days 1 month 41 days 4 days 1 month 2 months 3 months 25 days 18 days 1 month 2 months 3 months 4 months 16 days
+ + + + + + + + + + + + + + + + +
+ + + + + + + + + + + + + + +
+
Barium in duodenum and jejunum at 5 hrs Same Same
+ +
+ + + +
+ + +
In small bowel at 48 hrs In small bowel at 6 days In jejunum at 6 hr In small bowel at 44 hrs In small and large bowel at 5 days In jejunum at 24 hr In small bowel at 5 days
In small and large bowel at 7 days In small and large bowel at 8 days
ABNORMAL INTESTINAL MOTILITY IN GASTROSCHISIS
Vol. 127
Fig. 4. fixation.
CASE
III.
459
Pediatric Radiology
Barium remains in the small bowel on the sixth day after the examination. The loops of the bowel also show
abdominal defect is relatively easy. The evisceration in these cases is thought to have occurred relatively late in pregnancy. All of our eight patients had gross changes and could be classified as antenatal type. Gilbert (4) and Touloukian (9) reported that early gastrointestinal examinations revealed delayed transit time. The small bowel after one year of age was normal except for malrotation. None of their patients had episodes of intestinal obstruction later in life. The colon was redundant with normal haustral and mucosal pattern several months after surgical repair. The shortening and severe inflammation of the eviscerated intestine are apparently reversible following gastroschisis repair. In all eight of our patients who had upper gastrointestinal examinations with barium following surgical repair of gastroschisis, there were almost no visible peristaltic activities in the abnormal loops of the small bowel, which had been eviscerated through the defect and exposed to amniotic fluid in utero. We also observed occasional to-and-fro movements in the duodenal loop. The bowel was markedly dilated (Fig. 1), edematous, fixed (Fig. 2), and shortened. The dilatation and fixation could be easily seen on plain radiographs of the abdomen in all patients. Normal peristaltic activity resumes slowly if the infant does well. Malrotation of the bowel (Fig. 3) was also present in all patients as observed in other series (1, 4) and the transit time was markedly delayed, as long as several days (Fig. 4). A very slow transit time was noted, and up to six months of age in CASE I.
Whenever the plain radiographs of the abdomen showed dilatation and fixation of the bowel, the barium examination was always abnormal, i.e., abnormal plain radiographs of the abdomen precludes gastrointestinal examination with barium unless complete mechanical obstruction of the bowel or perforation is clinically suspected. Atresia of the bowel was reported to be about 10% (1, 2) but none of our patients had atresia. Because of dilatation of the loops of the bowel and a very slow transit time, the length of the small bowel could not be judged accurately from upper gastrointestinal examinations with barium. However, the shortening of the bowel was noted in all cases at the time of the initial surgical repair. PATHOGENESIS
The pathogenesis of the intestinal abnormality is unknown. In an animal experiment (6) the eviscerated intestine demonstrated interstitial edema without cellular infiltration which appears to result from contact with amniotic fluid. Potentially reversible damage to myenteric ganglion cells, and a progressive disappearance of acetylesterase and acetylcholinesterase occur as early as in the second week after the bowel is exposed to amniotic fluid. These changes cause the disordered peristalsis, and very slow transit time. The digestive dysfunction may be related to alterations in the specific enzyme systems in the mucosal cells of the exposed bowel. The degree of the
460
KOOK SANG OH AND OTHERS
abnormality in the bowel and mesentery seems to be proportional to the duration of the exposure to amniotic fluid in utero.
REFERENCES 1. Bill AH Jr: Gastroschisis in Pediatric Surgery. Mustard WT, Ravitch MM, Snyder WMJ, et al: eds. Yearbook Medical Publishers Inc., 2nd Ed., Vol. 1, P 685, 1969 2. Coupland GAE: Gastroschisis and colonic obstruction. Med J Austral 1:344-345, 1969 3. Duhamel B: Embryology of exomphalos and allied malformations. Arch Dis Child 38: 142-147, Apr 1963 4. Gilbert MG, Mencia LF, Puranik SR, et al: Management of gastroschisis and short bowel: report of 17 cases, J Ped Surg 7: 598-607, Oct-Nov, 1972 5. Hutchin P: Gastroschisis on the antenatal evisceration of the entire gastrointestinal tract. Surg 57:297-301, Feb 1965
May 1978
6. Haller JA Jr, Kehrer BH, Shaker IJ, et al: Studies of the pathophysiology of gastroschisis in fetal sheep. J Pediatr Surg 9:627-632, Oct 1974 7. Moore TC: Gastroschisis with antenatal evisceration of intestines and urinary bladder. Surg 158:263-269, Aug 1963 8. Shaw A: The myth of gastroschisis. J Pediatr Surg 10:235-244, Apr 1975 9. Touloukian RK, Spackman TJ: Gastrointestinal function and radiographic appearance following gastroschisis repair. J Pediatr Surg 6:427-434, Aug 1971
Kook Sang Oh, M.D. Dept. of Radiology Children's Hospital of Pittsburgh Pittsburgh, PA 15213
Gastroschisis 1
Pediatric Radiology
Kook Sang Oh, M.D., John P. Dorst, M.D.,2 Rodrigo Dominguez, M.D., and Bertram R. Girdany, M.D. The authors describe abnormal intestinal motility with a very slow transit time in eight children with gastroschisis. Other abnormal findings include malrotation, dilatation, and fixation of the loops of the bowel. INDEX TERMS: Gastrointestinal tract, abnormalities. (GI system, gastroschisis, omphalocoele, 7 [0]. 1495) • Infants, newborn, digestive system • Intestines, abnormalities • Stomach, abnormal ities
Radiology 127:457-460, May 1978
HE
Shaw (8), in his recent article "The Myth of Gastroschisis", states that gastroschisis is the result of in utero rupture of the membrane covering a hernia of the umbilical cord either during its normal phase (fifth through tenth week of fetal life) or at a later stage in embryos in which closure of the normally formed umbilical ring has not occurred. Because embryologists have thus far not given us unequiovocal evidence of "gastroschisis" in early human fetal specimens, current concepts of an embryogenesis for "gastroschisis" distinct from that of hernia of the umbilical cord should be discraded. The herniated viscera through the defect consists mainly
term gastroschisis means "belly separation"
T from the Greek words gastro (belly) and schism (sep-
aration). Gastroschisis is defined as an extraumbilical abdominal wall anomaly, in which there is a normal cord insertion with nonrotation of an abnormally short midgut, without a sac or a sac remnant (1). It occurs in one of every 6,000 live births, and about 40% of those afflicted are premature infants. Little attention has been paid to the radiological findings in gastroschisis (4, 9). In this paper we describe abnormal intestinal motility during gastrointestinal examinations at various periods after surgical repair of gastroschisis. MATERIALS AND METHODS
We studied eight patients within a period of 6 years. Each had gastrointestinal examinations with barium for evaluation of intestinal motility and morphology, and all underwent emergency surgical repair. They received intravenous hyperalimentation for a varying period of time. RESULTS
The results are summarized in TABLE I. DISCUSSION
The closing of the abdomen of the embryo is accompanied by the circumferential folding of 4 folds (3). These folds are the condensation of embryonic mesenchyma and are called the cephalic, the caudal, and the two lateral folds. Each fold has somatic and splanchnic layers. The somatic layer of the cephalic fold forms the thoracic and epigastric walls. The somatic layer of the caudal fold forms the hypogastric wall, while the somatic layers of the lateral form the two lateral walls of the abdomen. The apex of these folds forms the future umbilical ring. Failure of localized differentiation and subsequent resorption of embryonic mesenchyma in any of these folds results in a circumscribed defect.
Fig. 1. CASE I. Barium outlines the dilated duodenum and proximal jejunum. The intestinal mucosa is edematous.
From the Department of Radiology, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania. From the Department of Radiology and Radiological Science, Johns Hopkins Hospital, Baltimore, Maryland. Accepted for publication in January emt 1978. 1
2
457
458
KOOK SANG OH AND
Fig. 2. rant.
CASE II.
Fig. 3. CASE VI. show malrotation.
OTHERS
May 1978
Three examinations on three different days show fixation of the loops of the dilated small bowel in the right upper quad-
The dilated duooenum and proximal jejunum
of small and large intestine, although often the entire gastrointestinal tract from stomach to rectum is eviscerated (5, 9). In the majority of infants with gastroschisis the viscera becomes incarcerated within the abdominal wall opening during early gestation. The eviscerated mass is edematous and covered by a fibrinogelatinous membrane containing squamous cells and vernix from its in utero exposure to amniotic fluid. The matrix is often contaminated by the meconium and bacterial organisms at or just following birth. Most striking is the gross shortening and coiling of the intestine. Peristalsis is impaired (9). On the bases of appearance of the herniated bowel and the size of the peritoneal cavity, Moore (7) classifies them into two major types, antenatal and perinatal. In the antenatal type, the evisceration is presumed to have occurred during early pregnancy, with long-standing exposure to amniotic fluid, thick matted bowel covered with a gelatinous matrix, and abnormally small peritoneal cavities. However, the perinatal type has minimal serosal reaction with normal size of the peritoneal cavity and closure of the
TABLE I: SUMMARY OFRADIOLOGICAL FINDINGS CASES
II III IV V VI VII
VIII
AGE AT THE TIME OF EXAM
DILATION
FIXATION
MALROTATION
TRANSIT TIME
2 months 4 months 6 months 6 days 1 month 41 days 4 days 1 month 2 months 3 months 25 days 18 days 1 month 2 months 3 months 4 months 16 days
+ + + + + + + + + + + + + + + + +
+ + + + + + + + + + + + + + +
+
Barium in duodenum and jejunum at 5 hrs Same Same
+ +
+ + + +
+ + +
In small bowel at 48 hrs In small bowel at 6 days In jejunum at 6 hr In small bowel at 44 hrs In small and large bowel at 5 days In jejunum at 24 hr In small bowel at 5 days
In small and large bowel at 7 days In small and large bowel at 8 days
ABNORMAL INTESTINAL MOTILITY IN GASTROSCHISIS
Vol. 127
Fig. 4. fixation.
CASE
III.
459
Pediatric Radiology
Barium remains in the small bowel on the sixth day after the examination. The loops of the bowel also show
abdominal defect is relatively easy. The evisceration in these cases is thought to have occurred relatively late in pregnancy. All of our eight patients had gross changes and could be classified as antenatal type. Gilbert (4) and Touloukian (9) reported that early gastrointestinal examinations revealed delayed transit time. The small bowel after one year of age was normal except for malrotation. None of their patients had episodes of intestinal obstruction later in life. The colon was redundant with normal haustral and mucosal pattern several months after surgical repair. The shortening and severe inflammation of the eviscerated intestine are apparently reversible following gastroschisis repair. In all eight of our patients who had upper gastrointestinal examinations with barium following surgical repair of gastroschisis, there were almost no visible peristaltic activities in the abnormal loops of the small bowel, which had been eviscerated through the defect and exposed to amniotic fluid in utero. We also observed occasional to-and-fro movements in the duodenal loop. The bowel was markedly dilated (Fig. 1), edematous, fixed (Fig. 2), and shortened. The dilatation and fixation could be easily seen on plain radiographs of the abdomen in all patients. Normal peristaltic activity resumes slowly if the infant does well. Malrotation of the bowel (Fig. 3) was also present in all patients as observed in other series (1, 4) and the transit time was markedly delayed, as long as several days (Fig. 4). A very slow transit time was noted, and up to six months of age in CASE I.
Whenever the plain radiographs of the abdomen showed dilatation and fixation of the bowel, the barium examination was always abnormal, i.e., abnormal plain radiographs of the abdomen precludes gastrointestinal examination with barium unless complete mechanical obstruction of the bowel or perforation is clinically suspected. Atresia of the bowel was reported to be about 10% (1, 2) but none of our patients had atresia. Because of dilatation of the loops of the bowel and a very slow transit time, the length of the small bowel could not be judged accurately from upper gastrointestinal examinations with barium. However, the shortening of the bowel was noted in all cases at the time of the initial surgical repair. PATHOGENESIS
The pathogenesis of the intestinal abnormality is unknown. In an animal experiment (6) the eviscerated intestine demonstrated interstitial edema without cellular infiltration which appears to result from contact with amniotic fluid. Potentially reversible damage to myenteric ganglion cells, and a progressive disappearance of acetylesterase and acetylcholinesterase occur as early as in the second week after the bowel is exposed to amniotic fluid. These changes cause the disordered peristalsis, and very slow transit time. The digestive dysfunction may be related to alterations in the specific enzyme systems in the mucosal cells of the exposed bowel. The degree of the
460
KOOK SANG OH AND OTHERS
abnormality in the bowel and mesentery seems to be proportional to the duration of the exposure to amniotic fluid in utero.
REFERENCES 1. Bill AH Jr: Gastroschisis in Pediatric Surgery. Mustard WT, Ravitch MM, Snyder WMJ, et al: eds. Yearbook Medical Publishers Inc., 2nd Ed., Vol. 1, P 685, 1969 2. Coupland GAE: Gastroschisis and colonic obstruction. Med J Austral 1:344-345, 1969 3. Duhamel B: Embryology of exomphalos and allied malformations. Arch Dis Child 38: 142-147, Apr 1963 4. Gilbert MG, Mencia LF, Puranik SR, et al: Management of gastroschisis and short bowel: report of 17 cases, J Ped Surg 7: 598-607, Oct-Nov, 1972 5. Hutchin P: Gastroschisis on the antenatal evisceration of the entire gastrointestinal tract. Surg 57:297-301, Feb 1965
May 1978
6. Haller JA Jr, Kehrer BH, Shaker IJ, et al: Studies of the pathophysiology of gastroschisis in fetal sheep. J Pediatr Surg 9:627-632, Oct 1974 7. Moore TC: Gastroschisis with antenatal evisceration of intestines and urinary bladder. Surg 158:263-269, Aug 1963 8. Shaw A: The myth of gastroschisis. J Pediatr Surg 10:235-244, Apr 1975 9. Touloukian RK, Spackman TJ: Gastrointestinal function and radiographic appearance following gastroschisis repair. J Pediatr Surg 6:427-434, Aug 1971
Kook Sang Oh, M.D. Dept. of Radiology Children's Hospital of Pittsburgh Pittsburgh, PA 15213
