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Intestinal pseudoobstruction in a neonate caused by idiopathic muscular hypertrophy of the entire small intestine
Intestinal P s e u d o o b s t r u c t i o n in a N e o n a t e Caused by Idiopathic Muscular Hypertrophy of the Entire Small Intestine By I. Yamagiwa, M. Ohta, K. Obata, and M. Washio Yamagata, Japan 9 W e report a case of intestinal obstruction in a neonate caused by massive muscular hypertrophy of the entire small intestine. Furthermore, the muscularis propria consisted of three layers, in most part. The pathogenesis of this apparently unique lesion is obscure. 9 1988 by Grune & Stratton, Inc. INDEX WORDS: Idiopathic muscular hypertrophy; intestinal obstruction.
T H O U G H S E C O N D A R Y muscular hypertroA Lphy is frequently seen in the dilated segment of the intestine proximal to a stenotic portion, primary muscular hypertrophy of the alimentary tract, except the pylorus, is very rare. Spencer and Hudson 1 reported a case of idiopathic muscular hypertrophy of the esophagus, stomach, pylorus, duodenum, jejunum, and ileum, and he reviewed two cases of idiopathic muscular hypertrophy of the gastrointestinal tract in children. All three patients had hypertrophy of the esophageal and pyloric muscularis. In this report, we describe a case with intestinal obstruction caused by idiopathic muscular hypertrophy of the entire small intestine without pyloric muscular hypertrophy. CASE REPORT An infant boy was referred to Yamagata University Hospital with poor feeding and jaundice at 11 days of age. He was the product of a normal pregnancy and weighed 4,470 g at birth. He had multiple anomalies such as sclerocornea, cryptorchidism, bell shaped thorax, scoliosis, and deformity of the extremities. Poor feeding began at eight days of age. Before then, he was normally fed. Upon admission, some sausage-like masses were palpated through the abdominal wall. Vomiting with the vomit containing bile occurred at 16 days of age, and bile-stained discharge increased day by day through the nasogastric tube. Abdominal scout film and barium enema showed normal findings. The sausage-like masses were regarded not as fecal masses but as the small intestine, by palpation at the barium enema. Barium meal passed through the small intestine to the large intestine in 48 hours. Rectoanal reflex was positive, and esophageal manometry showed normal findings of the lower esophageal sphincter (length, 20.0 mm; pressure, 20.0 mmHg). There were no chromosomal abnormalities. He received total parenteral nutrition through a central venous catheter because of prolonged intestinal obstruction symptom.
From the Second Department of Surgery, Yamagata University School of Medicine, Yamagata, Japan. Address reprint requests to Iwao Yamagiwa, MD, Department of Pediatric Surgery, Niigata University Hospital, Asahimachi 1, Niigata, Japan, 951. 9 1988 by Grune & Stratton, Inc. 0022-3468/88/2309-0024503.00/0
866
Since the amount of discharge from the nasogastric tube had increased, laparotomy was performed 37 days after birth. No ascites or adhesions were found. The entire small intestine was thickened and shortened because of two or three fibrous bundles running along just like colonic tenia, and the lumen was narrowed by a thickened wall. The vascular network around the entire small intestine was extremely fine and dense, and ran irregularly (Fig 1). These changes in the small intestine were relatively slight in the proximal jejunum. The entire small intestine was 150 cm in length. No abnormal findings were seen in the stomach, duodenum, or colon. The entire mesentery appeared normal, but the mesenterial lymph nodes were swollen. There were no abnormalities in other abdominal organs. To relieve the intestinal obstruction symptoms, we resected the small intestine except for the jejunum 30 cm in length from Treitz's ligament, together with the cecum, and performed end-to-end anastomosis between the jejunum and the ascending colon. Postoperatively, the amount of discharge from the nasogastric tube decreased gradually. Thirty-six days after surgery, oral feedings could be started. One year postoperatively, the patient was able to tolerate enteral nutrition of 500 kcal/d in elemental diet, but he was troubled by short bowel syndrome rather than intestinal obstruction. Parenteral nutrition was necessary to make up for the calorie shortage.
Pathology of the Resected Intestine The wall of the small intestine showed marked thickening due to muscular hypertrophy. The muscularis propria of the small intestine was three to four times as thick as that of the cecum (Fig 2). The mucosa had no remarkable changes. Microscopic examination showed marked hypertrophy of the muscularis propria, and in addition to ordinary circular and longitudinal muscle layers, there was one more layer below the longitudinal layer through almost the entire length of the resected small intestine (Fig 3). The muscle bundles of the excessive muscle layer ran obliquely in most part, and irregularly or discontinuously in part, and they intermingled with the muscle bundles of the longitudinal muscle layer in part. The myenteric plexuses were situated between the circular and longitudinal layers in normal fashion. Ganglion cells in the myenteric and the submucosal plexuses showed no abnormal findings (Fig 4). The villi of the mucosa were slightly atrophic, and there were no abnormal findings in the submucosal layer. The appendix vermiformis was unremarkable. The myenteric plexuses of the cecum seemed more prominent and irregularly shaped than normal, but myenteric and submucosal ganglion cells were normal (Fig 4). There were no inflammatory changes in the resected intestine.
DISCUSSION
There are many mechanisms that may produce an intestinal obstruction in neonates. Besides well-known etiologies, there have been some reports of intestinal obstruction due to rare causes, such as neuronal intestinal dysplasia, 2 megacystis-microcolon-intestinal hypoperistalsis syndrome? congenital short intestine, 4 circular-muscle defect in the small bowel, 5 and so on. In the case reported herein, intestinal obstruction was Journal of Pediatric Surgery, Vo123, No 9 (September),1988: pp 866-869
MUSCULAR HYPERTROPHY OF THE SMALL INTESTINE
867
Fig 1. T w o to t h r e e fibrous bundles run along t h e entire small intestine just like colonic tenia. Consequently, t h e small intestine becomes shortened and thickened, and t h e thickened wall narrows t h e lumen. Vascular n e t w o r k around t h e small i n t e s t i n e is fine and dense, and runs irregularly. Note unique appearance of t h e small intestine in contrast w i t h t h e sigmoid colon (arrow) preserving normal appearance.
.o
Fig 2. Ileocecal valve. Note the thickened muscle layer of t h e terminal ileum in contrast w i t h normal thickness of t h e muscle layer of t h e cecum (H&E, original magnification x 1.8).
Fig 3. Ileum. Muscularis propria consists of three muscle layers, the circular, longitudinal, and extra-oblique muscle layers. Myenteric plexuses are situated between the circular and longitudinal muscle layers. The intestine is free from inflammation or degenerative changes (H&E, original magnification •
Fig 4. The myenteric plexuses of (A) the ileum and (B) the cecum have normally distributed ganglion cells (H&E, original magnification x 50).
MUSCULAR HYPERTROPHY OF THE SMALL INTESTINE
869
caused by muscular hypertrophy in the entire small intestine. Spencer and Hudson ~ reported a 3-year-old child with idiopathic muscular hypertrophy of the esophagus, stomach, pylorus, duodenum, jejunum, and ileum. The patient's principal s y m p t o m was vomiting starting when he was 3 months old. Two other cases of idiopathic muscular hypertrophy of the gastrointestinal tract in children had been reported previously. 6'7 All three children had hypertrophy of the esophageal and pyloric muscles, but one had involvement of the ileocecal valve, 7 and other two had additional involvement of the d u o d e n u m and the jejunum. ~'6 In our case, although the esophageal involvement was uncertain, there was no pyloric muscular hypertrophy. In addition, muscular hypertrophy was observed in the circular muscle in Spencer's case, but in our case there was one more layer in addition to the ordinary circular and longitudinal muscle layer. Therefore, this case m a y be classified as a different entity from the other three. W e believe that the muscular hypertrophy in this case m a y be primary change for the following reasons:
(1) there were no obstructive lesions in the colon, (2) some sausage-like masses were palpated through the abdominal wall even after relief of intestinal obstruction, (3) there were no adhesions or inflammatory changes in the abdominal cavity, and (4) there were no degenerative or inflammatory changes in the intestine. Massive resection of the intestine relieved intestinal obstruction, but the patient was then plagued by short bowel syndrome. It m a y be preferable that we should have preserved more length of the small intestine. ADDENDUM
After submission of this manuscript, the patient died of hepatic failure at 14 months of age. A n autopsy showed no muscular hypertrophy of the esophagus. ACKNOWLEDGMENT
The authors thank Dr Hidenobu Watanabe, Professor of First Department of Pathology, Niigata University School of Medicine, for his advice on histologic findings and helpful comments.
REFERENCES
1. Spencer R, Hudson TL: Idiopathic muscular hypertrophy of the gastrointestinal tract in a child. Surgery 50:678-686, 1961 2. Scharli AF, Meier-Ruge W: Localized and disseminated forms of neuronal intestinal dysplasia mimicking Hirschsprung's disease. J Pediatr Surg 16:164-170, 1981 3. Amoury RA, Fellows RA, Goodwin CD, et al: Megacystismicrocolon-intestinal hypoperistalsis syndrome: A cause of intestinal obstruction in the newborn period. J Pediatr Surg 12:1063-1065, 1977 4. Yutani C, Miyaji T: Congenital short intestine: A case report and review of the literature. Arch Pathol 96:81-82, 1973
5. Humphry A, Mancer K, Stephens CA: Obstructive circularmuscle defect in the small bowel in a one-year-old child. J Pediatr Surg 15:197-199, 1980 6. Guthrie KJ: Idiopathic muscular hypertrophy of oesophagus, pylorus, duodenum and jejunum in a young girl. Arch Dis Child 20:176-178, 1945 7. Pritchard E, Hillier WT: Specimens from a case of hypertrophic pyloric stenosis with associated hypertrophies. Proc R Soc Med 13:33, 1920
T H O U G H S E C O N D A R Y muscular hypertroA Lphy is frequently seen in the dilated segment of the intestine proximal to a stenotic portion, primary muscular hypertrophy of the alimentary tract, except the pylorus, is very rare. Spencer and Hudson 1 reported a case of idiopathic muscular hypertrophy of the esophagus, stomach, pylorus, duodenum, jejunum, and ileum, and he reviewed two cases of idiopathic muscular hypertrophy of the gastrointestinal tract in children. All three patients had hypertrophy of the esophageal and pyloric muscularis. In this report, we describe a case with intestinal obstruction caused by idiopathic muscular hypertrophy of the entire small intestine without pyloric muscular hypertrophy. CASE REPORT An infant boy was referred to Yamagata University Hospital with poor feeding and jaundice at 11 days of age. He was the product of a normal pregnancy and weighed 4,470 g at birth. He had multiple anomalies such as sclerocornea, cryptorchidism, bell shaped thorax, scoliosis, and deformity of the extremities. Poor feeding began at eight days of age. Before then, he was normally fed. Upon admission, some sausage-like masses were palpated through the abdominal wall. Vomiting with the vomit containing bile occurred at 16 days of age, and bile-stained discharge increased day by day through the nasogastric tube. Abdominal scout film and barium enema showed normal findings. The sausage-like masses were regarded not as fecal masses but as the small intestine, by palpation at the barium enema. Barium meal passed through the small intestine to the large intestine in 48 hours. Rectoanal reflex was positive, and esophageal manometry showed normal findings of the lower esophageal sphincter (length, 20.0 mm; pressure, 20.0 mmHg). There were no chromosomal abnormalities. He received total parenteral nutrition through a central venous catheter because of prolonged intestinal obstruction symptom.
From the Second Department of Surgery, Yamagata University School of Medicine, Yamagata, Japan. Address reprint requests to Iwao Yamagiwa, MD, Department of Pediatric Surgery, Niigata University Hospital, Asahimachi 1, Niigata, Japan, 951. 9 1988 by Grune & Stratton, Inc. 0022-3468/88/2309-0024503.00/0
866
Since the amount of discharge from the nasogastric tube had increased, laparotomy was performed 37 days after birth. No ascites or adhesions were found. The entire small intestine was thickened and shortened because of two or three fibrous bundles running along just like colonic tenia, and the lumen was narrowed by a thickened wall. The vascular network around the entire small intestine was extremely fine and dense, and ran irregularly (Fig 1). These changes in the small intestine were relatively slight in the proximal jejunum. The entire small intestine was 150 cm in length. No abnormal findings were seen in the stomach, duodenum, or colon. The entire mesentery appeared normal, but the mesenterial lymph nodes were swollen. There were no abnormalities in other abdominal organs. To relieve the intestinal obstruction symptoms, we resected the small intestine except for the jejunum 30 cm in length from Treitz's ligament, together with the cecum, and performed end-to-end anastomosis between the jejunum and the ascending colon. Postoperatively, the amount of discharge from the nasogastric tube decreased gradually. Thirty-six days after surgery, oral feedings could be started. One year postoperatively, the patient was able to tolerate enteral nutrition of 500 kcal/d in elemental diet, but he was troubled by short bowel syndrome rather than intestinal obstruction. Parenteral nutrition was necessary to make up for the calorie shortage.
Pathology of the Resected Intestine The wall of the small intestine showed marked thickening due to muscular hypertrophy. The muscularis propria of the small intestine was three to four times as thick as that of the cecum (Fig 2). The mucosa had no remarkable changes. Microscopic examination showed marked hypertrophy of the muscularis propria, and in addition to ordinary circular and longitudinal muscle layers, there was one more layer below the longitudinal layer through almost the entire length of the resected small intestine (Fig 3). The muscle bundles of the excessive muscle layer ran obliquely in most part, and irregularly or discontinuously in part, and they intermingled with the muscle bundles of the longitudinal muscle layer in part. The myenteric plexuses were situated between the circular and longitudinal layers in normal fashion. Ganglion cells in the myenteric and the submucosal plexuses showed no abnormal findings (Fig 4). The villi of the mucosa were slightly atrophic, and there were no abnormal findings in the submucosal layer. The appendix vermiformis was unremarkable. The myenteric plexuses of the cecum seemed more prominent and irregularly shaped than normal, but myenteric and submucosal ganglion cells were normal (Fig 4). There were no inflammatory changes in the resected intestine.
DISCUSSION
There are many mechanisms that may produce an intestinal obstruction in neonates. Besides well-known etiologies, there have been some reports of intestinal obstruction due to rare causes, such as neuronal intestinal dysplasia, 2 megacystis-microcolon-intestinal hypoperistalsis syndrome? congenital short intestine, 4 circular-muscle defect in the small bowel, 5 and so on. In the case reported herein, intestinal obstruction was Journal of Pediatric Surgery, Vo123, No 9 (September),1988: pp 866-869
MUSCULAR HYPERTROPHY OF THE SMALL INTESTINE
867
Fig 1. T w o to t h r e e fibrous bundles run along t h e entire small intestine just like colonic tenia. Consequently, t h e small intestine becomes shortened and thickened, and t h e thickened wall narrows t h e lumen. Vascular n e t w o r k around t h e small i n t e s t i n e is fine and dense, and runs irregularly. Note unique appearance of t h e small intestine in contrast w i t h t h e sigmoid colon (arrow) preserving normal appearance.
.o
Fig 2. Ileocecal valve. Note the thickened muscle layer of t h e terminal ileum in contrast w i t h normal thickness of t h e muscle layer of t h e cecum (H&E, original magnification x 1.8).
Fig 3. Ileum. Muscularis propria consists of three muscle layers, the circular, longitudinal, and extra-oblique muscle layers. Myenteric plexuses are situated between the circular and longitudinal muscle layers. The intestine is free from inflammation or degenerative changes (H&E, original magnification •
Fig 4. The myenteric plexuses of (A) the ileum and (B) the cecum have normally distributed ganglion cells (H&E, original magnification x 50).
MUSCULAR HYPERTROPHY OF THE SMALL INTESTINE
869
caused by muscular hypertrophy in the entire small intestine. Spencer and Hudson ~ reported a 3-year-old child with idiopathic muscular hypertrophy of the esophagus, stomach, pylorus, duodenum, jejunum, and ileum. The patient's principal s y m p t o m was vomiting starting when he was 3 months old. Two other cases of idiopathic muscular hypertrophy of the gastrointestinal tract in children had been reported previously. 6'7 All three children had hypertrophy of the esophageal and pyloric muscles, but one had involvement of the ileocecal valve, 7 and other two had additional involvement of the d u o d e n u m and the jejunum. ~'6 In our case, although the esophageal involvement was uncertain, there was no pyloric muscular hypertrophy. In addition, muscular hypertrophy was observed in the circular muscle in Spencer's case, but in our case there was one more layer in addition to the ordinary circular and longitudinal muscle layer. Therefore, this case m a y be classified as a different entity from the other three. W e believe that the muscular hypertrophy in this case m a y be primary change for the following reasons:
(1) there were no obstructive lesions in the colon, (2) some sausage-like masses were palpated through the abdominal wall even after relief of intestinal obstruction, (3) there were no adhesions or inflammatory changes in the abdominal cavity, and (4) there were no degenerative or inflammatory changes in the intestine. Massive resection of the intestine relieved intestinal obstruction, but the patient was then plagued by short bowel syndrome. It m a y be preferable that we should have preserved more length of the small intestine. ADDENDUM
After submission of this manuscript, the patient died of hepatic failure at 14 months of age. A n autopsy showed no muscular hypertrophy of the esophagus. ACKNOWLEDGMENT
The authors thank Dr Hidenobu Watanabe, Professor of First Department of Pathology, Niigata University School of Medicine, for his advice on histologic findings and helpful comments.
REFERENCES
1. Spencer R, Hudson TL: Idiopathic muscular hypertrophy of the gastrointestinal tract in a child. Surgery 50:678-686, 1961 2. Scharli AF, Meier-Ruge W: Localized and disseminated forms of neuronal intestinal dysplasia mimicking Hirschsprung's disease. J Pediatr Surg 16:164-170, 1981 3. Amoury RA, Fellows RA, Goodwin CD, et al: Megacystismicrocolon-intestinal hypoperistalsis syndrome: A cause of intestinal obstruction in the newborn period. J Pediatr Surg 12:1063-1065, 1977 4. Yutani C, Miyaji T: Congenital short intestine: A case report and review of the literature. Arch Pathol 96:81-82, 1973
5. Humphry A, Mancer K, Stephens CA: Obstructive circularmuscle defect in the small bowel in a one-year-old child. J Pediatr Surg 15:197-199, 1980 6. Guthrie KJ: Idiopathic muscular hypertrophy of oesophagus, pylorus, duodenum and jejunum in a young girl. Arch Dis Child 20:176-178, 1945 7. Pritchard E, Hillier WT: Specimens from a case of hypertrophic pyloric stenosis with associated hypertrophies. Proc R Soc Med 13:33, 1920