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Lower esophageal sphincter in hypertrophic pyloric stenosis
ABSTRACTS
675
ALIMENTARY TRACT Gastrointestinal Manifestations of Cystic Fibrosis: A Review Roger IV. Park and Richard J. Grand. Gastroentcrol
81:1143-1161, (December), 1981. Cystic fibrosis in the most common, lethal, genetic defect of white populations. It is inherited as an autosomal recessive and it is estimated to occur in I in 2000 live births. Chronic obstructive pulmonary disease is eventually present in all cases and accounts for almost all of the mortality associated with cystic fibrosis beyond the neonatal period. Gastrointestinal symptoms occur in 85% to 90% of patients and this article reviews those symptoms. Cystic fibrosis is the most common cause o f exocrine pancreatic insutficiency i n infants and children in the developed nations. Eighty-five to ninety percent of patients with cystic fibrosis have evidence of pancreatic exocrine dysfunction. Fat and protein maldigestion and fecal losses are the primary manifestations of pancreatic involvement. Endocrine dysfunction (glucose intolerance) has been found in approximately 30% of the patients with cystic fibrosis and clinically significant diabetes mellitus occurs in 1%-2% of the patients. Three pancreatic preparations are generally used: Cotazyme, Viokase, or Pancrease. Although enzyme activities vary considerably from one product to another they are all roughly equivalent in their clinical effectiveness. Delivery of low levels of lipase remains a problem with all preparations. Meconium ileus is a presenting symptom in 10%-15% of infants with cystic fibrosis. As many as half of the cases of meconium ileus are complicated by volvulus, atresia, and/or meconium peritonitis. For this reason, a sweat test should be done in all infants with jejunal and ileal atresia, volvulus, meconium ileus, or meconium peritonitis. Sweat tests are likely to be positive in 30% of patients with meconium peritonitis and 15% to 20% of patients with atresias. Nonoperative relief of uncomplicated meconium ileus can be achieved with Gastrografin or Mucomyst enemas. Complicated meconium ileus requires surgery. Infants with cystic fibrosis and meconium ileus who survived beyond 6 mo of age have the same prognosis as any patient with cystic fibrosis. "Meconium ileus equivalent" or distal intestinal obstruction syndrome is estimated to occur in about 10% of patients with cystic fibrosis and it may be the presenting symptom of the disease. This problem can usually be managed medically with Mucomyst or Gastrografin enemas and/or by instilling Mucomyst through an intestinal tube. Intussusception is a complication of the meconium ileus equivalent that occurred in approximately 1% of a large group of patients with cystic fibrosis. Intussuception can be the presenting symptom of cystic fibrosis and cystic fibrosis is the major cause of the intussusception beyond infancy. Rectal prolapse is common in cystic fibrosis being reported in 22% of 386 patients having a long-term follow-up. The prolapse is usually recurrent, but medical management is almost always successful. The etiology may be secondary to voluminous stools, frequent bowel movements, varying degrees of malnutrition, and increased abdominal pressure secondary to distended bowel, coughing, or pulmonary hyperinflation. Cystic fibrosis commonly affects-the liver and reports of abnormalities vary from 20% to 50% of cases studied, although only 5% of the patients develop cirrhosis and only 2% progress to clinically apparent or important liver disease. Fatty liver remains one of the most common hepatic
abnormalities encountered in cystic fibrosis. Hepatic fibrosis and eventually biliary cirrhosis may develop. The most common clinical abnormality indicative of hepatic involvement and cystic fibrosis is an elevated serum alkaline phosphatase. Portasystemic shunts may be required in patients with portal hypertension; about half of the reported procedures were performed because of bleeding varices and the remainder were done because of hypersplenism. Some authors recommend prophylactic shunt surgery in patients with portal hypertension at an early age because the progressive pulmonary disease would make surgery at a later date more complicated. This approach is not recommended by these authors. The gallbladder and cystic duct are abnormal in approximately one third of patients with cystic fibrosis. Microgallbladders are commonly reported, characteristically containing thick, colorless "white bile." Functional and anatomic abnormalities, however, are not usually associated with symptoms. Nutritional deficiencies are common in patients with cystic fibrosis secondary to malabsorption, anorexia, or increase requirements imposed by the chronic lung disease. Growth patterns may be normal until about the age of 9 yr when slow growth becomes the rule, corresponding to increased severity of pulmonary disease. Protein requirements are increased because of increased fecal losses. This is especially true in infants. In older children there may be minimal protein loss from the gut, but some patients with liver disease may show decreased synthesis of protein. However, by far the most common and significant cause for hypoalbuminemia in older patients is an increase plasma volume associated with right ventricular failure. Steatorrhea is associated with malabsorption of the fat soluble vitamins A, D, E, and K. An increased intake of a multiple vitamin preparation is customarily recommended. Rickets is surprisingly uncommon is pa'tients with cystic fibrosis so that vitamin D supplementation beyond that provided by multiple vitamin preparations has generally not been recommended. Deficiencies in water soluble vitamins has not been a problem in patients with cystic fibrosis.--Richard R. Ricketts Eosinophilic Esophagitis. D. Picus and P. H. Frank. A JR 136:1001-1003, (May), 1981. A 16-yr-old male presented with progressive dysphagia and a history of penicillin allergy. Laboratory results included a peripheral eosinophilia (16%). An esophagram revealed a stricture and nodularity at the junction of the upper and middle thirds. Endoscopic biopsy revealed a heavy eosinophil infiltration. Clinical symptoms responded to oral steroids and at 3 wk the esophagus appeared normal except for a postinflammatory stricture that responded well to serial dilatations. The differential diagnosis includes caustic peptic or radiation esophagitis, primary and metastatic tumors, tuberculosis, and Crohn's disease. Steroids usually control the symptoms.--Randall W. Powell Lower Esophageal Sphincter in Hypertrophic Py|oric Stanosis. S. Take, A. Kimura, S. Nishida, et al. J Jpn Soc Pediatr
Surg 18:123-127, (February), 1982. Intraluminal pressures of lower esophageal sphincter (LESP) were measured in 16 infants with hypertrophic pyloric stenosis, 6 of whom showed gastro-esophageal reflux
676
(GER), and 10 age-matched controls. LESP in controls was 17.70 mm Hg and increased to 29.30 mm Hg after administration 47/kg of Tetra-Gastrin. LESP in infants with both hypertrophic pyloric stenosis and GER was 8.50 • 2.43 mm Hg and significantly lower than in controls. LESP in these patients increased to 15.35 _+ 4.67 mm Hg after pyloromyotomy. Administration of Tetra-Gastrin resulted in increase of LESP, but increase of LESP was limited to 2.17 mm Hg before operation. However, there was marked increase of LESP (21.17 • 4.26 mm Hg) by administration of TetraGastrin in these patients after pyloromyotomy. LESP in infants with hypertrophic pyloric stenosis but no GER was 17.60 mm Hg. LESP in these patients after pyloromyotomy was 17.10 _+ 5.13 mm Hg. There was no significant difference of LESP between controls and these patients. Increase of LESP after administration of Tetra-Gastrin in these patients before operation was significantly lower than controls, but there was no difference in increase of LESP by administration between controls and these patients postoperatively. The authors concluded that response of LESP to administration of Tetra-Gastrin was limited in the patients with hypertrophic pyloric stcnosis, but the response was normalized by pyloromyotomy.--H. Suzuki Esophageal Manometry in Hypertrophic Pyloric Stenosis. M. Honzumi, S. Amano, H. Saijo, et al. Mie Med J 31:135-138, (September), 1981.
Esophageal manometry was carried out in 15 infants with hypertrophic pyloric stenosis before and after pyloromyotomy. Six normal infants were also studied as age-matched controls. High intragastric pressure of 26_+ 7, 1 8 4 2 cm HzO was characteristic of infants with hypertrophic pyloric stenosis. The intragastric pressure decreased within 1 wk after the surgery. Pressure of the lower esophageal sphincter (LES) in these infants was low (28 -+ 13, 10--63 cm H20) in comparison with controls (37 • 4, 24--47 cm H20), but the difference was not significant. No increase of pressure of the LES was noted within 1 wk after the operation. The pressure difference between the LES and the stomach was negative in 9 of 15 infants with pyloric stenosis, whereas this pressure difference was positive in all the postoperative infants l wk after the surgery. The authors suggest that the incompetence of the LES in hypertrophic pyloric stenosis was secondary to the gastric outlet obstruction.--H. Suzuki Hiatus Hernia. K. lkeda, S. Sumitomo, and Y. Hayashida.
Surg Diag Treat [Tokyo] 24:431-438, (April), 1982. The results of follow-up studies in 7 patients with hiatus hernia who underwent Nissen's fundoplication were reported in 6 males and 1 female. The ages at the time of the operation ranged from 1 mo to 4 yr and 6 too. The ages at the time of follow-up ranged from 7 yr and 6 mo to 14 yr and 3 too. None of these patients complained of dysphagia, heart burn, or respiratory symptoms during follow-up. Physical development was satisfactory in 4 of 7 patients. On gastro-esophagograms, reflux was not observed and anatomical correction of hernia was satisfactory. Esophageal manometry was carried out in 6 cases. Manometric length of lower esophageal sphincter ranged from 1.9 to 3.2 cm (mean: 2.6 • 0.6 cm) and
ABSTRACTS
intraluminal pressure of lower esophageal sphincter ranged from 15.9 to 26.4 cm H20 (mean: 20.2 _+4.1 cm H20). There was no significant difference of length and pressure of lower esophageal sphincter between the postoperative patients and age-matched controls. Peristalsis of the esophagus and concomitant relaxation of lower esophageal sphincter were normally observed in all the studied postoperative patients,--H. Suzuki Gastric Duplication. K. Kato, H. Yamaraoto, K. OzakL et aL Shujutsu (Operation), 35:1496-1499, (December), 1981.
Gastric duplication seen in a 12-day-old female baby was reported. She presented with vomiting and abdominal mass. At the operation, a 3 x 4 x 5 em duplication cyst was found in the antral portion of the stomach. Distal gastrectomy and gastroduodenostomy were carried out. Postoperative course was uneventful. The authors collected 14 cases of gastric duplication seen in pediatric age group from the Japanese literature. Five were males and 9 were females. Presenting symptoms were vomiting in 12, abdominal pain in 3, abdominal mass in 7, rectal bleeding in 1, and anorexia in 1 case. Correct diagnosis was made in only 1 case preoperatively. Treatments were gastrectomy in 4, exstirpation of the cyst in 8, and none in 2 cases. There were no deaths.--H. S u z u k i Sonography of Hypertrophic Pyloric Stenosis. S. Strauss, Y. Itzchak, A. Manor, et aL AJR 136:1057-1058, (June), 1981.
Twenty infants (14-49 days) underwent abdominal sonography because of suspected pyloric stenosis. Sixteen infants had ventral-dorsal diameters of the pylorus of 1.5 cm or greater and 15 had the diagnosis confirmed at surgery. One infant with a 2.4-cm diameter pylorus improved with conservative treatment. Four infants with pyloric diameters less than 1.5 cm improved without surgery. The hypertrophied pylorus presents as an anechoic mass with a central collection of echoes representing the opposing surfaces of the narrowed pyloric canal.--Randall 14I.Powell Henoch-Schonlein Syndrome in Chldren: Gastrointestinal Manifestations. C. M. Glasier, M. J. Siegel, W. H. McAlister, et al. A JR 136:1081-1085, (June), 1981.
Twenty-two children ranging in age from 20 mo to 11 yr (mean 5.5 yr) admitted over a 10-yr period to form the basis for this report. Only two patients demonstrated all four characteristic features of Henoch-Schonlein purpura, i.e., abdominal pain, rash, arthralgia, and hematuria. Six patients demonstrated the first 3 features, while 9 patients had only abdominal pain and rash. Three patients developed or presented with intussusception with 2 requiring surgical resection for resolution. Fifteen patients underwent UGI series with SBFT with the most common finding being mucosal abnormalities of the small bowel. The patients with distal ileal involvement had findings similar to Crohn's disease. The differential diagnosis of the small bowel findings include regional enteritis, lymphoma, and submucosal bleeding. The intussusceptions are frequently ileoileal (approximately 50%) and not reduceable by barium enema.--Randall W. Powell
675
ALIMENTARY TRACT Gastrointestinal Manifestations of Cystic Fibrosis: A Review Roger IV. Park and Richard J. Grand. Gastroentcrol
81:1143-1161, (December), 1981. Cystic fibrosis in the most common, lethal, genetic defect of white populations. It is inherited as an autosomal recessive and it is estimated to occur in I in 2000 live births. Chronic obstructive pulmonary disease is eventually present in all cases and accounts for almost all of the mortality associated with cystic fibrosis beyond the neonatal period. Gastrointestinal symptoms occur in 85% to 90% of patients and this article reviews those symptoms. Cystic fibrosis is the most common cause o f exocrine pancreatic insutficiency i n infants and children in the developed nations. Eighty-five to ninety percent of patients with cystic fibrosis have evidence of pancreatic exocrine dysfunction. Fat and protein maldigestion and fecal losses are the primary manifestations of pancreatic involvement. Endocrine dysfunction (glucose intolerance) has been found in approximately 30% of the patients with cystic fibrosis and clinically significant diabetes mellitus occurs in 1%-2% of the patients. Three pancreatic preparations are generally used: Cotazyme, Viokase, or Pancrease. Although enzyme activities vary considerably from one product to another they are all roughly equivalent in their clinical effectiveness. Delivery of low levels of lipase remains a problem with all preparations. Meconium ileus is a presenting symptom in 10%-15% of infants with cystic fibrosis. As many as half of the cases of meconium ileus are complicated by volvulus, atresia, and/or meconium peritonitis. For this reason, a sweat test should be done in all infants with jejunal and ileal atresia, volvulus, meconium ileus, or meconium peritonitis. Sweat tests are likely to be positive in 30% of patients with meconium peritonitis and 15% to 20% of patients with atresias. Nonoperative relief of uncomplicated meconium ileus can be achieved with Gastrografin or Mucomyst enemas. Complicated meconium ileus requires surgery. Infants with cystic fibrosis and meconium ileus who survived beyond 6 mo of age have the same prognosis as any patient with cystic fibrosis. "Meconium ileus equivalent" or distal intestinal obstruction syndrome is estimated to occur in about 10% of patients with cystic fibrosis and it may be the presenting symptom of the disease. This problem can usually be managed medically with Mucomyst or Gastrografin enemas and/or by instilling Mucomyst through an intestinal tube. Intussusception is a complication of the meconium ileus equivalent that occurred in approximately 1% of a large group of patients with cystic fibrosis. Intussuception can be the presenting symptom of cystic fibrosis and cystic fibrosis is the major cause of the intussusception beyond infancy. Rectal prolapse is common in cystic fibrosis being reported in 22% of 386 patients having a long-term follow-up. The prolapse is usually recurrent, but medical management is almost always successful. The etiology may be secondary to voluminous stools, frequent bowel movements, varying degrees of malnutrition, and increased abdominal pressure secondary to distended bowel, coughing, or pulmonary hyperinflation. Cystic fibrosis commonly affects-the liver and reports of abnormalities vary from 20% to 50% of cases studied, although only 5% of the patients develop cirrhosis and only 2% progress to clinically apparent or important liver disease. Fatty liver remains one of the most common hepatic
abnormalities encountered in cystic fibrosis. Hepatic fibrosis and eventually biliary cirrhosis may develop. The most common clinical abnormality indicative of hepatic involvement and cystic fibrosis is an elevated serum alkaline phosphatase. Portasystemic shunts may be required in patients with portal hypertension; about half of the reported procedures were performed because of bleeding varices and the remainder were done because of hypersplenism. Some authors recommend prophylactic shunt surgery in patients with portal hypertension at an early age because the progressive pulmonary disease would make surgery at a later date more complicated. This approach is not recommended by these authors. The gallbladder and cystic duct are abnormal in approximately one third of patients with cystic fibrosis. Microgallbladders are commonly reported, characteristically containing thick, colorless "white bile." Functional and anatomic abnormalities, however, are not usually associated with symptoms. Nutritional deficiencies are common in patients with cystic fibrosis secondary to malabsorption, anorexia, or increase requirements imposed by the chronic lung disease. Growth patterns may be normal until about the age of 9 yr when slow growth becomes the rule, corresponding to increased severity of pulmonary disease. Protein requirements are increased because of increased fecal losses. This is especially true in infants. In older children there may be minimal protein loss from the gut, but some patients with liver disease may show decreased synthesis of protein. However, by far the most common and significant cause for hypoalbuminemia in older patients is an increase plasma volume associated with right ventricular failure. Steatorrhea is associated with malabsorption of the fat soluble vitamins A, D, E, and K. An increased intake of a multiple vitamin preparation is customarily recommended. Rickets is surprisingly uncommon is pa'tients with cystic fibrosis so that vitamin D supplementation beyond that provided by multiple vitamin preparations has generally not been recommended. Deficiencies in water soluble vitamins has not been a problem in patients with cystic fibrosis.--Richard R. Ricketts Eosinophilic Esophagitis. D. Picus and P. H. Frank. A JR 136:1001-1003, (May), 1981. A 16-yr-old male presented with progressive dysphagia and a history of penicillin allergy. Laboratory results included a peripheral eosinophilia (16%). An esophagram revealed a stricture and nodularity at the junction of the upper and middle thirds. Endoscopic biopsy revealed a heavy eosinophil infiltration. Clinical symptoms responded to oral steroids and at 3 wk the esophagus appeared normal except for a postinflammatory stricture that responded well to serial dilatations. The differential diagnosis includes caustic peptic or radiation esophagitis, primary and metastatic tumors, tuberculosis, and Crohn's disease. Steroids usually control the symptoms.--Randall W. Powell Lower Esophageal Sphincter in Hypertrophic Py|oric Stanosis. S. Take, A. Kimura, S. Nishida, et al. J Jpn Soc Pediatr
Surg 18:123-127, (February), 1982. Intraluminal pressures of lower esophageal sphincter (LESP) were measured in 16 infants with hypertrophic pyloric stenosis, 6 of whom showed gastro-esophageal reflux
676
(GER), and 10 age-matched controls. LESP in controls was 17.70 mm Hg and increased to 29.30 mm Hg after administration 47/kg of Tetra-Gastrin. LESP in infants with both hypertrophic pyloric stenosis and GER was 8.50 • 2.43 mm Hg and significantly lower than in controls. LESP in these patients increased to 15.35 _+ 4.67 mm Hg after pyloromyotomy. Administration of Tetra-Gastrin resulted in increase of LESP, but increase of LESP was limited to 2.17 mm Hg before operation. However, there was marked increase of LESP (21.17 • 4.26 mm Hg) by administration of TetraGastrin in these patients after pyloromyotomy. LESP in infants with hypertrophic pyloric stenosis but no GER was 17.60 mm Hg. LESP in these patients after pyloromyotomy was 17.10 _+ 5.13 mm Hg. There was no significant difference of LESP between controls and these patients. Increase of LESP after administration of Tetra-Gastrin in these patients before operation was significantly lower than controls, but there was no difference in increase of LESP by administration between controls and these patients postoperatively. The authors concluded that response of LESP to administration of Tetra-Gastrin was limited in the patients with hypertrophic pyloric stcnosis, but the response was normalized by pyloromyotomy.--H. Suzuki Esophageal Manometry in Hypertrophic Pyloric Stenosis. M. Honzumi, S. Amano, H. Saijo, et al. Mie Med J 31:135-138, (September), 1981.
Esophageal manometry was carried out in 15 infants with hypertrophic pyloric stenosis before and after pyloromyotomy. Six normal infants were also studied as age-matched controls. High intragastric pressure of 26_+ 7, 1 8 4 2 cm HzO was characteristic of infants with hypertrophic pyloric stenosis. The intragastric pressure decreased within 1 wk after the surgery. Pressure of the lower esophageal sphincter (LES) in these infants was low (28 -+ 13, 10--63 cm H20) in comparison with controls (37 • 4, 24--47 cm H20), but the difference was not significant. No increase of pressure of the LES was noted within 1 wk after the operation. The pressure difference between the LES and the stomach was negative in 9 of 15 infants with pyloric stenosis, whereas this pressure difference was positive in all the postoperative infants l wk after the surgery. The authors suggest that the incompetence of the LES in hypertrophic pyloric stenosis was secondary to the gastric outlet obstruction.--H. Suzuki Hiatus Hernia. K. lkeda, S. Sumitomo, and Y. Hayashida.
Surg Diag Treat [Tokyo] 24:431-438, (April), 1982. The results of follow-up studies in 7 patients with hiatus hernia who underwent Nissen's fundoplication were reported in 6 males and 1 female. The ages at the time of the operation ranged from 1 mo to 4 yr and 6 too. The ages at the time of follow-up ranged from 7 yr and 6 mo to 14 yr and 3 too. None of these patients complained of dysphagia, heart burn, or respiratory symptoms during follow-up. Physical development was satisfactory in 4 of 7 patients. On gastro-esophagograms, reflux was not observed and anatomical correction of hernia was satisfactory. Esophageal manometry was carried out in 6 cases. Manometric length of lower esophageal sphincter ranged from 1.9 to 3.2 cm (mean: 2.6 • 0.6 cm) and
ABSTRACTS
intraluminal pressure of lower esophageal sphincter ranged from 15.9 to 26.4 cm H20 (mean: 20.2 _+4.1 cm H20). There was no significant difference of length and pressure of lower esophageal sphincter between the postoperative patients and age-matched controls. Peristalsis of the esophagus and concomitant relaxation of lower esophageal sphincter were normally observed in all the studied postoperative patients,--H. Suzuki Gastric Duplication. K. Kato, H. Yamaraoto, K. OzakL et aL Shujutsu (Operation), 35:1496-1499, (December), 1981.
Gastric duplication seen in a 12-day-old female baby was reported. She presented with vomiting and abdominal mass. At the operation, a 3 x 4 x 5 em duplication cyst was found in the antral portion of the stomach. Distal gastrectomy and gastroduodenostomy were carried out. Postoperative course was uneventful. The authors collected 14 cases of gastric duplication seen in pediatric age group from the Japanese literature. Five were males and 9 were females. Presenting symptoms were vomiting in 12, abdominal pain in 3, abdominal mass in 7, rectal bleeding in 1, and anorexia in 1 case. Correct diagnosis was made in only 1 case preoperatively. Treatments were gastrectomy in 4, exstirpation of the cyst in 8, and none in 2 cases. There were no deaths.--H. S u z u k i Sonography of Hypertrophic Pyloric Stenosis. S. Strauss, Y. Itzchak, A. Manor, et aL AJR 136:1057-1058, (June), 1981.
Twenty infants (14-49 days) underwent abdominal sonography because of suspected pyloric stenosis. Sixteen infants had ventral-dorsal diameters of the pylorus of 1.5 cm or greater and 15 had the diagnosis confirmed at surgery. One infant with a 2.4-cm diameter pylorus improved with conservative treatment. Four infants with pyloric diameters less than 1.5 cm improved without surgery. The hypertrophied pylorus presents as an anechoic mass with a central collection of echoes representing the opposing surfaces of the narrowed pyloric canal.--Randall 14I.Powell Henoch-Schonlein Syndrome in Chldren: Gastrointestinal Manifestations. C. M. Glasier, M. J. Siegel, W. H. McAlister, et al. A JR 136:1081-1085, (June), 1981.
Twenty-two children ranging in age from 20 mo to 11 yr (mean 5.5 yr) admitted over a 10-yr period to form the basis for this report. Only two patients demonstrated all four characteristic features of Henoch-Schonlein purpura, i.e., abdominal pain, rash, arthralgia, and hematuria. Six patients demonstrated the first 3 features, while 9 patients had only abdominal pain and rash. Three patients developed or presented with intussusception with 2 requiring surgical resection for resolution. Fifteen patients underwent UGI series with SBFT with the most common finding being mucosal abnormalities of the small bowel. The patients with distal ileal involvement had findings similar to Crohn's disease. The differential diagnosis of the small bowel findings include regional enteritis, lymphoma, and submucosal bleeding. The intussusceptions are frequently ileoileal (approximately 50%) and not reduceable by barium enema.--Randall W. Powell