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Megacystis-microcolon-intestinal-hypoperistalsis syndrome (neonatal hollow visceral myopathy)
INTERNATIONAL
1478
Type A: rectal mucosal biopsies in the newborn period showed proliferation of Ache-positive nerve fibers similar to those seen in aganglionosis. Type B: proliferation of AChE-positive nerve fibers was only rarely seen in both lamina propria and lamina muscularis in the newborn period. However, during or after infancy all these rectal mucosal biopsies showed increased proliferation of AChEpositive nerve fibers. Type C: rectal mucosal biopsies performed after infancy showed no proliferation of AChE-positive nerve fibers in the mucosa. An accurate diagnosis of type C can only be made by full-thickness rectal biopsy. Barium enema examination showed definitive caliber changes in seven, equivocal changes in four, and none in one. Anorectal manometry showed atypical rectosphincteric reflex in five cases and an absent reflex in seven. Management depends on severity of clinical symptoms and length of diseased intestine. The author concludes that patients with hypoganglionosis do not improve with time and require resection of the affected segment of bowel.-Prem Pun’ Abnormal Peptidergic Innervation in Internal Sphincter Achala&a. T. Fujimoto, P. Puri, and T. Miyano. Pediatr Surg Int 7:12-17,
(Januaty). 1992. The nature of innervation of the internal sphincter muscle in patients with internal sphincter achalasia was studied using antineurotransmitter antibodies (vasoactive intestinal peptide, substance P, metenkephalen, neuropeptide Y), acetylchohnesterase (Ache) enzyme histochemistry, and electronmicroscopy. Using the above methods, 14 patients with internal sphincter achalasia were compared with 5 normal controls and 4 patients with Hirschsprung’s disease (HD). It was observed that various peptide containing nerves were markedly increased in the internal sphincter in achalasia patients, but were reduced in colonic circular muscle in patients with HD. On the basis of these findings, the authors feel that the pathophysiology of internal sphincter achalasia is different from that of HD. Therefore, internal sphincter achalasia is a distinct clinical entity and should be discussed separately from HD.-George Ninan Megacystis-Microcolon-Intestinal-Hypoperistalsis Syndrome (Neonatal Hollow Visceral Myopathy). P. Puri and M. Tsuji.
ABSTRACTS
megacystis-microcolon-intestinal-hypoperistalsis syndrome (MMIHS). An esophageal examination showed normal esophageal contractions, and a positive rectoanal reflex was obtained during anorectal manometry. However, the continuous fasting manometric recording of the antrum, duodenum, and jejunum lasting a total of 12 hours showed a severe motility disturbance. During the examination a band of low-amplitude contractions was observed in the antrum only once. This was followed by small rhythmic contractions of the duodenum without propagation. No spontaneous contractions were seen in the jejunum. These findings support the hypothesis that the motility disturbance of the gastrointestinal tract might be caused by neurogenic disorders of the myenteric neural system in MMIHS.-Thomas A. Angerpointner Alimentary Tract Duplications in Infants and Children. A.B. Pint&, W Schubert, F. Szemlt?dy, et al. Eur J Pediatrc Surg 2:8-12,
(February), 1992. Thirty duplications of the alimentary tract in 28 patients are reviewed. Ages ranged from 1 day to 13 years, the majority of patients being less than 2 years. There were 6 thoracic, 20 abdominal, and 2 thoracoabdominal duplications. The children presented with distended abdomen, vomiting, bowel obstruction, and palpable abdominal masses. Plain thoracic and abdominal x-rays, ultrasonography, barium esophagogram, barium meal, and enema were the most common diagnostic procedures. Emergency intervention became necessary in 18 cases. One child died of an unrelated disease. Twenty-three duplications were cystic and 3 tubular. The surgical procedure consisted of removal of the duplication. The authors recommend that this procedure be no more radical than necessary in order to eliminate potential complaints and to prevent a short bowel syndrome. They also caution that the common blood supply shared between duplication and native bowel be carefully protected to avoid undue damage to normal bowel.-Thomas A. Angepointner lntraperitoneal Fetal Small Bowel Transplantation as Therapy for the Short Bowel Syndrome: An Animal Experimental Study. S. Kellnar and T. Rattanasouwan. Eur J Pediatr Surg
Pediatr Surg Int 7:18-22, (January), 1992.
2:13-15, (February), 1992.
In this review of the literature on megacystis-microcolonintestinal-hypoperistalsis syndrome (MMIHS), the authors have analyzed data from 59 cases of MMIHS reported to date. There were 46 females and 13 males. The clinical presentation is characterized by abdominal distension, bile-stained vomiting, and absent or decreased bowel sounds. Radiologically or at operation the most frequent finding is a massively distended urinary bladder and microcolon. Most investigators now agree that ganglion cells and nerve fibers in the myenteric and submucous plexus in this syndrome are normal in number and appearance. Vacuolar degeneration in the smooth muscle cells with abundant amount of connective tissue between muscle cells have been found on electronmicroscopy by some investigators. The prognosis in this syndrome is poor: 87% morality in 59 reported cases. Six of the 8 survivors are dependent on total parenteral nutrition. The higher incidence in siblings, together with consanguinity in three sets of parents of children affected with the syndrome, suggests an autosomal recessive pattern of inheritance.--George Ninan
Morphological integrity of fetal rat intestinal transplants and in vitro evidence of both the digestive and resorptive function in the transplanted bowel segments have already been demonstrated by the authors. In another experimental approach an attempt was made to assess intestinal function in vivo. Fetal intestinal transplants were placed into host animals and allowed to mature for 4 weeks. Then the whole small bowel was resected from the ligament of Treitz to the ileocecal valve. The matured fetal intestinal transplant was then interposed so that the function of the small intestine was taken over by the transplanted fetal bowel. The interposed fetal bowel transplant prevented weight loss and provided normal weight gain. This shows that in the rat model fetal small bowel, previously transplanted into a host, can be a functioning substitute for normal small bowel.-Thomas A. Angelpointner
Manometric Evaluation of Gastrointestinal Motility in a Case of Megacystis-Microcolon-Intestinal-Hypoperistalsis Syndrome (MMIHS). T. Shone, S. Suita, T. Taguchi, etal. Eur J Pediatr
Surg 2:52-55, (February), 1992. The authors report on a manometric study of the esophagus, stomach, duodenum. jejunum, and anorectum in a child with
A Reassessment of Surgical Techniques for Neomucosal Growth. A. Bianchi, M. Lendon, and I.D. Ward. Pediatr Surg Int
7:41-46, (January), 1992. The techniques of Binnington, Gaton, and Thomason for growing neomucosa over colonic serosal patches and those of Norton, Nothigen, Watson for growing new mucosa over vascularized mucosally denuded colonic muscle were evaluated in eight pigs. This study revealed little, if any, neomucosal growth along the colonic serosal surface or in mucosally denuded colonic muscle. No mucosal growth was obtained even when the colonic serosa and the
1478
Type A: rectal mucosal biopsies in the newborn period showed proliferation of Ache-positive nerve fibers similar to those seen in aganglionosis. Type B: proliferation of AChE-positive nerve fibers was only rarely seen in both lamina propria and lamina muscularis in the newborn period. However, during or after infancy all these rectal mucosal biopsies showed increased proliferation of AChEpositive nerve fibers. Type C: rectal mucosal biopsies performed after infancy showed no proliferation of AChE-positive nerve fibers in the mucosa. An accurate diagnosis of type C can only be made by full-thickness rectal biopsy. Barium enema examination showed definitive caliber changes in seven, equivocal changes in four, and none in one. Anorectal manometry showed atypical rectosphincteric reflex in five cases and an absent reflex in seven. Management depends on severity of clinical symptoms and length of diseased intestine. The author concludes that patients with hypoganglionosis do not improve with time and require resection of the affected segment of bowel.-Prem Pun’ Abnormal Peptidergic Innervation in Internal Sphincter Achala&a. T. Fujimoto, P. Puri, and T. Miyano. Pediatr Surg Int 7:12-17,
(Januaty). 1992. The nature of innervation of the internal sphincter muscle in patients with internal sphincter achalasia was studied using antineurotransmitter antibodies (vasoactive intestinal peptide, substance P, metenkephalen, neuropeptide Y), acetylchohnesterase (Ache) enzyme histochemistry, and electronmicroscopy. Using the above methods, 14 patients with internal sphincter achalasia were compared with 5 normal controls and 4 patients with Hirschsprung’s disease (HD). It was observed that various peptide containing nerves were markedly increased in the internal sphincter in achalasia patients, but were reduced in colonic circular muscle in patients with HD. On the basis of these findings, the authors feel that the pathophysiology of internal sphincter achalasia is different from that of HD. Therefore, internal sphincter achalasia is a distinct clinical entity and should be discussed separately from HD.-George Ninan Megacystis-Microcolon-Intestinal-Hypoperistalsis Syndrome (Neonatal Hollow Visceral Myopathy). P. Puri and M. Tsuji.
ABSTRACTS
megacystis-microcolon-intestinal-hypoperistalsis syndrome (MMIHS). An esophageal examination showed normal esophageal contractions, and a positive rectoanal reflex was obtained during anorectal manometry. However, the continuous fasting manometric recording of the antrum, duodenum, and jejunum lasting a total of 12 hours showed a severe motility disturbance. During the examination a band of low-amplitude contractions was observed in the antrum only once. This was followed by small rhythmic contractions of the duodenum without propagation. No spontaneous contractions were seen in the jejunum. These findings support the hypothesis that the motility disturbance of the gastrointestinal tract might be caused by neurogenic disorders of the myenteric neural system in MMIHS.-Thomas A. Angerpointner Alimentary Tract Duplications in Infants and Children. A.B. Pint&, W Schubert, F. Szemlt?dy, et al. Eur J Pediatrc Surg 2:8-12,
(February), 1992. Thirty duplications of the alimentary tract in 28 patients are reviewed. Ages ranged from 1 day to 13 years, the majority of patients being less than 2 years. There were 6 thoracic, 20 abdominal, and 2 thoracoabdominal duplications. The children presented with distended abdomen, vomiting, bowel obstruction, and palpable abdominal masses. Plain thoracic and abdominal x-rays, ultrasonography, barium esophagogram, barium meal, and enema were the most common diagnostic procedures. Emergency intervention became necessary in 18 cases. One child died of an unrelated disease. Twenty-three duplications were cystic and 3 tubular. The surgical procedure consisted of removal of the duplication. The authors recommend that this procedure be no more radical than necessary in order to eliminate potential complaints and to prevent a short bowel syndrome. They also caution that the common blood supply shared between duplication and native bowel be carefully protected to avoid undue damage to normal bowel.-Thomas A. Angepointner lntraperitoneal Fetal Small Bowel Transplantation as Therapy for the Short Bowel Syndrome: An Animal Experimental Study. S. Kellnar and T. Rattanasouwan. Eur J Pediatr Surg
Pediatr Surg Int 7:18-22, (January), 1992.
2:13-15, (February), 1992.
In this review of the literature on megacystis-microcolonintestinal-hypoperistalsis syndrome (MMIHS), the authors have analyzed data from 59 cases of MMIHS reported to date. There were 46 females and 13 males. The clinical presentation is characterized by abdominal distension, bile-stained vomiting, and absent or decreased bowel sounds. Radiologically or at operation the most frequent finding is a massively distended urinary bladder and microcolon. Most investigators now agree that ganglion cells and nerve fibers in the myenteric and submucous plexus in this syndrome are normal in number and appearance. Vacuolar degeneration in the smooth muscle cells with abundant amount of connective tissue between muscle cells have been found on electronmicroscopy by some investigators. The prognosis in this syndrome is poor: 87% morality in 59 reported cases. Six of the 8 survivors are dependent on total parenteral nutrition. The higher incidence in siblings, together with consanguinity in three sets of parents of children affected with the syndrome, suggests an autosomal recessive pattern of inheritance.--George Ninan
Morphological integrity of fetal rat intestinal transplants and in vitro evidence of both the digestive and resorptive function in the transplanted bowel segments have already been demonstrated by the authors. In another experimental approach an attempt was made to assess intestinal function in vivo. Fetal intestinal transplants were placed into host animals and allowed to mature for 4 weeks. Then the whole small bowel was resected from the ligament of Treitz to the ileocecal valve. The matured fetal intestinal transplant was then interposed so that the function of the small intestine was taken over by the transplanted fetal bowel. The interposed fetal bowel transplant prevented weight loss and provided normal weight gain. This shows that in the rat model fetal small bowel, previously transplanted into a host, can be a functioning substitute for normal small bowel.-Thomas A. Angelpointner
Manometric Evaluation of Gastrointestinal Motility in a Case of Megacystis-Microcolon-Intestinal-Hypoperistalsis Syndrome (MMIHS). T. Shone, S. Suita, T. Taguchi, etal. Eur J Pediatr
Surg 2:52-55, (February), 1992. The authors report on a manometric study of the esophagus, stomach, duodenum. jejunum, and anorectum in a child with
A Reassessment of Surgical Techniques for Neomucosal Growth. A. Bianchi, M. Lendon, and I.D. Ward. Pediatr Surg Int
7:41-46, (January), 1992. The techniques of Binnington, Gaton, and Thomason for growing neomucosa over colonic serosal patches and those of Norton, Nothigen, Watson for growing new mucosa over vascularized mucosally denuded colonic muscle were evaluated in eight pigs. This study revealed little, if any, neomucosal growth along the colonic serosal surface or in mucosally denuded colonic muscle. No mucosal growth was obtained even when the colonic serosa and the