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Value of a simple biochemical ratio in distinguishing upper and lower sites of gastrointestinal haemorrhage
290
Results and Complications of Surgery for Gastro-oesophageal Reflux. L. Spitz and J. Kirtane. Arch Dis Child 60:743-747,
(August), 1985. One hundred six patients with gastro-oesophageal reflux requiring Nissen fundoplication were studied. Presenting symptoms included persistent vomiting, failure to thrive, hematemesis, recurrent chest infections, dysphagia, and anemia. Only four (4%) had apnoeic or cyanotic attacks. All patients had routine upper gastrointestinal contrast studies and endoscopy. Twenty-nine had a stricture at the time of presentation. Indications for surgery were as follows: (A) oesophageal stricture (29); (B) anatomical abnormalities (16); (C) failure of conservative management (62); (D) apnoeic attacks (4); and (E) recurrent chest infections (5). Children with mental retardation formed the largest group (41) who failed to respond to medical management. A standard loose Nissen fundoplication was the operation performed. One or two deep crural sutures were used to tighten the hiatus. Eight patients died, two from postoperative chest complications in retarded children. Three died of unrelated causes. One was a cot-death, one had severe necrotizing enterocolitis superimposed on a severe combined immune deficiency, and one died following empyema and septicemia. Other complications were as follows--(A) prolapse of the fundoplication into the mediastinum (7); (B) para-oesophageal herniation (1); (C) intestinal obstruction due to adhesions (4) and intussusception (1); (D) temporary gas bloat in two retarded children; (E) wound dehiscence (1); (F) dumping syndrome (1); (G) intractable strictures in five, all requiring surgical treatment; and (H) other minor complications--wound infections (3), pulmonary atelectasis (8), and temporary dysphagia (3). The final outcome was 97 patients with complete amelioration of symptoms. Twenty patients developed complications requiring further surgery. Patients with established strictures had a greater risk of complications; therefore, earlier treatment is advised in these cases if possible.--Alasdair H.B. Fyfe Integrity of Cholinergic Innervation to the Lower Esophageal Sphincter in Achalasia. R.H. Holloway, W.J. Dodds, J.F. Helm, et
al. Gastroenterology 90:924-929, (April), 1986. The lower esophageal sphincter (LES) is thought to be innervated by nonadrenergic noncholinergic inhibitory fibers and cholinergic excitatory fibers. In achalasia, LES relaxation is abnormal because the inhibitory fibers to the sphincter are either absent or functionally impaired. The current study was undertaken to evaluate the role of the cholinergic excitatory fibers to the LES. Comparing 27 patients with untreated acbalasia and 21 healthy volunteers, the authors found that atropine substantially reduces the LES pressure in achalasia patients as well as in normal controls, that edrophonium increases LES pressure in achalasia patients while having negligible effect on normal subjects, and the LES in achalasia patients exhibits cyclical phasic contractile activity in synchrony with fasting gastric motor activity. These findings support the hypothesis that the cholinergic LES innervation in achalasia patients is normal or only minimally impaired in contrast to the marked impairment of the inhibitory nerves governing LES relaxation.--Richard R. Ricketts Gastroesphageal Sphincter Pressure and Histologic Changes in Distal Esophagus in Patients With Achalasia of the Esophagus. A.
Csendes, G. Srnok, I. Braghetto, et al. Dig Dis Sci 30:941-945, (October), 1985. A prospective study was performed in 17 patients with achalasia of the esophagus determining the manometric characteristics of the gastroesophageal sphincter and correlating them with histologic analysis by biopsies taken during surgery at the distal narrowed segment of the esophagus at the location of the sphincter. The
INTERNATIONAL ABSTRACTS
histologic findings were compared with ten control cases. Presence or absence of ganglion cells of Auerbach's plexus and appearance of smooth muscle fibers were evaluated. Only one case had Chagas' disease. The mean sphincter pressure was 41 mmHg, with incomplete relaxation in all patients. Histologic analysis showed a complete disappearance of ganglion cells in 94% of the cases and a decrease in the number of neurons with marked chronic inflammatory cells in one case. In all control cases the ganglion cells were normal. Smooth muscle fibers were normal on light microscopy. No relationship was found between resting gastroesophageal sphincter pressure, length and relaxation, and histologic findings in the distal esophagus. These findings suggest that the denervation in the majority of cases is located in Auerbach's plexus with complete absence of ganglion cells and, therefore, absence of postganglionic nerve fibers.--Prem Puri
Significance of Ultrasound for the Diagnosis of Hypertrophic Pyloric Stenosis. R. Grussner, G. Pistor, B. Abou-Touk, et al.
Pediatr Surg Int 1:130-134, (June), 1986. The sonographic features of hypertrophic pyloric stenosis (HPS) were evaluated in a 5-year prospective study (1981 to 1985) at the University Clinic of Pediatric Surgery in Mainz. In 37 cases real-time ultrasound was performed preoperatively to measure muscle thickness, diameter, and length of the pylorus. The recorded values were compared with those of a control group. Muscular wall thickness proved to be the most reliable parameter, while pyloric canal length was the most difficult measurement to obtain. Diameter alone was not adequate to establish the sonographic diagnosis, which depends on the individually and functionally different luminal width and submucosal thickness. Significant sonographic criteria for the diagnosis of HPS are a muscle thickness of 5 mm or more and a diameter of 15 mm or more. These data are confirmed by intraoperative measurements. Indirect signs of HPS are a lack of passage through the narrowed pyloric canal, gastric distension combined with unproductive contractions, and, less commonly, gastroesophageal reflux. Although history and clinical examination are the main diagnostic means, ultrasound should be the initial imaging procedure if the clinical diagnosis is doubtful.--Prem Puri
CircumumbUical Incision for Pyloromyotomy. K.C. Tan and A.
Bianchi. Br J Surg 73:399, (May), 1986. The authors describe a series of 40 children, aged 3 to 8 weeks, with infantile hypertrophic pyloric stenosis who had a Ramstedt pyloromyotomy through a circumumbilical incision. Delivery of the pylorus was relatively easy. Seven children required antibiotics for a cellulitic flare around the wound with one purulent discharging wound requiring drainage. There was one wound dehiscence. All others healed primarily with a resultant scar that was hardly visible. The appearance was cosmetically superior in the long-term to other incisions commonly used.--Tony Sparnon
Value of a Simple Biochemical Ratio in Distinguishing Upper and Lower Sites of Gastrointestinal Haemorrhage. J.A. Snook, G.E.
Holdstock, and J. Barnforth. Lancet i: 1064-1065, (May 10), 1986. Fifty-nine cases of overt gastrointestinal haemorrhage were reviewed in which the source of bleeding was not initially evident but was identified by later investigation. The admission plasma urea to creatinine concentration ratio proved highly accurate in distinguishing upper and lower gastrointestinal sources of haemorrhage. Regardless of blood volume lost, 33 of 38 patients with upper gastrointestinal haemorrhage (87%) had a ratio of 100 or more, and
INTERNATIONAL ABSTRACTS
291
20 of 21 patients with lower gastrointestinal haemorrhage (95%) had a ratio of less than 100, an overall accuracy of 90%. Elevation of the ratio was not related to clinical evidence of hypovolemia. This simple ratio may be valuable in determining the sequence of investigations in patients with an unidentified source of gastrointestinal haemorrhage.--Prem Purl Unusual Association of Upper Gastro-lntestinal Anomalies in a Newborn. M. Dassonville, J.M. Bouton, L. Verstreken, et al. Z
Kinderchir 41:112-113, (April), 1986. A newborn with persistent nonbilious vomiting is described. The postprandial vomiting was due to hypertrophic pyloric stenosis, intestinal malrotation, and gastric antral diaphragm. The anomalies were successfully treated by three subsequent operations. The follow-up was normal for 2 years with weight and size at the 90th percentile.-- Thomas A. Angerpointner Surgical Management of Multiple Intestinal Atresias. E. L Hatch
and R. T. Schaller, Jr. Am J Surg 151:550-552, (May), 1986. This is basically a review paper stimulated by recent improved techniques utilized in the management of intestinal atresia. Principles of tapering or resection, maximal preservation of bowd length, and insurance of distal patency are emphasized. The importance of parenteral nutrition can also not be underemphasized.--Thomas V. Whalen Torsion of a Caecal Diverticulum in a Young Child, G.A.D. McPher-
son, S. Holland, and H.B. Ross. Br J Surg 72:714, (September), 1985. A 2-year-old boy presented with a three-day history of vomiting and a tender distended abdomen. Plain abdominal x-rays demonstrated a large air-filled viscus on the right side of the abdomen. Laparotomy revealed a 10 cm diameter diverticulum arising from the medial border of the cecum which had undergone torsion of its ped~cle. It was removed successfully. These rare diverticula usually mimic acute appendicitis. Sometimes they present as a hard mass. Melena has also been reported. Removal of an isolated asymptomatic diverticulum found incidentally at laparotomy is recommended.--N.P. Madden Crohn's Disease: Maintenance of Remission by Diet. V.A.
Jones,
R.J. Dickinson, E. Workman, et al. Lancet 8448:177-180, (July), 1985. Twenty patients with Crohn's disease took part in a controled trial in which remission was maintained by either an unrefined carbohydrate fiber-rich diet or a diet that excluded specific foods to which a patient was intolerant. Seven out of the ten patients on the exclusion diet remained in remission for 6 months compared with none out of the ten on an unrefined carbohydrate fiber-rich diet (P < .05, Fisher's exact test). In an uncontroled study an exclusion diet allowed 51 out of 77 patients to remain well on the diet alone for periods of up to 51 months, and with an average annual relapse rate of less than lO%.--Prem Purl Endotoxaemia in Active Crohn's Disease. T r e a t m e n t W i t h W h o l e Gut Irrigation and B-Aminosalicyclic Acid. W. Wellman, P.C. Fink,
F. Benner, et al. Gut 27:814-820, (July), 1986. Endotoxins in plasma were monitored during treatment in 18 patients hospitalized for acute exacerbation of Crohn's disease. Systemic endotoxemia was found on admission in all but one. The patients were randomly divided into two groups: one receiving treatment with total parenteral nutrition and steroids. To decrease
the absorbable endotoxin pool, the other group was additionally treated with whole gut irrigation, and 5-aminosalicylie acid was added to the lavage fluid. In most of these patients endotoxemia cleared after intestinal lavage and they needed shorter hospitalization. Earlier improvement was also indicated by a faster decrease of the Crohn's disease activity index and vanHees index. In the group receiving conservative treatment alone, endotoxemia was controled within 3 weeks. The authors conclude that endotoxemia occurs in most patients suffering from active Crohn's disease. Control of endotoxemia after intestinal lavage suggests that systemic endotoxemia is caused by absorption of endotoxins from the gut. Earlier improvement after whole gut irrigation indicates its beneficial effect in active Crohn's disease.--Prem Purl
Relationship Between Disease Activity Indices and Colonoscopic Findings in Patients W i t h Colonic Inflammatory Bowel Disease, P.
Gomes, Claire Du Boulay, C.L. Smith, et al. Gut 27:92-95, (January), 1986. The Crohn's disease activity index, a similar index devised for patients with ulcerative colitis, and other commonly used laboratory indicators of disease activity have been studied in 50 patients with colonic inflammatory bowel disease undergoing routine colonoseopic assessment and compared with the histologic extent and activity of disease. There was only poor correlation between the colonoscopic or histologic findings and the indices of disease activity studied, showing that these are not reliable measures of disease activity or extent at the tissue level.--Prem Purl
Pseudomembranous Colitis in Children and Adults. R.P. Bolton and D.F.M. Thomas. Br J Hosp Med 35:37-42, (January), 1986.
The etiology, clinical features, and specific therapy of antibiotic associated Pseudomembranous colitis is presented. Identification of Clostridium diffieile as the major pathogen is discussed in relation to the rational approach to therapy and associated disease.--Amir Azmy
Hirschsprung's Disease in the Newborn. An 11 Year Experience.
T.Z. Polley, Jr, and A.G. Coran. Pediatr Surg Int 1:80-83, (June), 1986. The records of all patients with Hirschsprung's disease diagnosed and treated at the authors' institution between July 1974 and August 1985 were reviewed. Of these 99 patients, 35 (35%) presented and were diagnosed within the first 30 days of life and constitute the basis of the present study group. Twenty-two infants (63%) had standard reetosigmoid disease with a male-to-female ratio of 2.2:1. Only one infant was premature. The spectrum of presenting signs included abdominal distension in I9 (54%), failure to pass meconium within the first 48 hours of life in 16 (46%), "constipation" in 12 (34%), and vomiting in 9 (26%). Intestinal perforation was a presenting sign in two patients (6%), and enterocolitis occurred preoperatively in four (12%). Evaluation was facilitated by diagnostic barium enema in 60% of the patients. In those infants able to undergo elective evaluation, the definitive diagnosis was made by suction rectal biopsy, which was accurate in all cases. In addition to the high proportion of patients with long-segment disease (13 patients, 37%), there was a significant incidence of associated congenital anomalies (26%), including Down's syndrome in five (14%). Thirty-three of the 35 patients underwent definitive treatment using the endorectal pull-through procedure performed at an average age of 12 months. There was no mortality related to the operation. In addition to highlighting the high incidence of congeni-
Results and Complications of Surgery for Gastro-oesophageal Reflux. L. Spitz and J. Kirtane. Arch Dis Child 60:743-747,
(August), 1985. One hundred six patients with gastro-oesophageal reflux requiring Nissen fundoplication were studied. Presenting symptoms included persistent vomiting, failure to thrive, hematemesis, recurrent chest infections, dysphagia, and anemia. Only four (4%) had apnoeic or cyanotic attacks. All patients had routine upper gastrointestinal contrast studies and endoscopy. Twenty-nine had a stricture at the time of presentation. Indications for surgery were as follows: (A) oesophageal stricture (29); (B) anatomical abnormalities (16); (C) failure of conservative management (62); (D) apnoeic attacks (4); and (E) recurrent chest infections (5). Children with mental retardation formed the largest group (41) who failed to respond to medical management. A standard loose Nissen fundoplication was the operation performed. One or two deep crural sutures were used to tighten the hiatus. Eight patients died, two from postoperative chest complications in retarded children. Three died of unrelated causes. One was a cot-death, one had severe necrotizing enterocolitis superimposed on a severe combined immune deficiency, and one died following empyema and septicemia. Other complications were as follows--(A) prolapse of the fundoplication into the mediastinum (7); (B) para-oesophageal herniation (1); (C) intestinal obstruction due to adhesions (4) and intussusception (1); (D) temporary gas bloat in two retarded children; (E) wound dehiscence (1); (F) dumping syndrome (1); (G) intractable strictures in five, all requiring surgical treatment; and (H) other minor complications--wound infections (3), pulmonary atelectasis (8), and temporary dysphagia (3). The final outcome was 97 patients with complete amelioration of symptoms. Twenty patients developed complications requiring further surgery. Patients with established strictures had a greater risk of complications; therefore, earlier treatment is advised in these cases if possible.--Alasdair H.B. Fyfe Integrity of Cholinergic Innervation to the Lower Esophageal Sphincter in Achalasia. R.H. Holloway, W.J. Dodds, J.F. Helm, et
al. Gastroenterology 90:924-929, (April), 1986. The lower esophageal sphincter (LES) is thought to be innervated by nonadrenergic noncholinergic inhibitory fibers and cholinergic excitatory fibers. In achalasia, LES relaxation is abnormal because the inhibitory fibers to the sphincter are either absent or functionally impaired. The current study was undertaken to evaluate the role of the cholinergic excitatory fibers to the LES. Comparing 27 patients with untreated acbalasia and 21 healthy volunteers, the authors found that atropine substantially reduces the LES pressure in achalasia patients as well as in normal controls, that edrophonium increases LES pressure in achalasia patients while having negligible effect on normal subjects, and the LES in achalasia patients exhibits cyclical phasic contractile activity in synchrony with fasting gastric motor activity. These findings support the hypothesis that the cholinergic LES innervation in achalasia patients is normal or only minimally impaired in contrast to the marked impairment of the inhibitory nerves governing LES relaxation.--Richard R. Ricketts Gastroesphageal Sphincter Pressure and Histologic Changes in Distal Esophagus in Patients With Achalasia of the Esophagus. A.
Csendes, G. Srnok, I. Braghetto, et al. Dig Dis Sci 30:941-945, (October), 1985. A prospective study was performed in 17 patients with achalasia of the esophagus determining the manometric characteristics of the gastroesophageal sphincter and correlating them with histologic analysis by biopsies taken during surgery at the distal narrowed segment of the esophagus at the location of the sphincter. The
INTERNATIONAL ABSTRACTS
histologic findings were compared with ten control cases. Presence or absence of ganglion cells of Auerbach's plexus and appearance of smooth muscle fibers were evaluated. Only one case had Chagas' disease. The mean sphincter pressure was 41 mmHg, with incomplete relaxation in all patients. Histologic analysis showed a complete disappearance of ganglion cells in 94% of the cases and a decrease in the number of neurons with marked chronic inflammatory cells in one case. In all control cases the ganglion cells were normal. Smooth muscle fibers were normal on light microscopy. No relationship was found between resting gastroesophageal sphincter pressure, length and relaxation, and histologic findings in the distal esophagus. These findings suggest that the denervation in the majority of cases is located in Auerbach's plexus with complete absence of ganglion cells and, therefore, absence of postganglionic nerve fibers.--Prem Puri
Significance of Ultrasound for the Diagnosis of Hypertrophic Pyloric Stenosis. R. Grussner, G. Pistor, B. Abou-Touk, et al.
Pediatr Surg Int 1:130-134, (June), 1986. The sonographic features of hypertrophic pyloric stenosis (HPS) were evaluated in a 5-year prospective study (1981 to 1985) at the University Clinic of Pediatric Surgery in Mainz. In 37 cases real-time ultrasound was performed preoperatively to measure muscle thickness, diameter, and length of the pylorus. The recorded values were compared with those of a control group. Muscular wall thickness proved to be the most reliable parameter, while pyloric canal length was the most difficult measurement to obtain. Diameter alone was not adequate to establish the sonographic diagnosis, which depends on the individually and functionally different luminal width and submucosal thickness. Significant sonographic criteria for the diagnosis of HPS are a muscle thickness of 5 mm or more and a diameter of 15 mm or more. These data are confirmed by intraoperative measurements. Indirect signs of HPS are a lack of passage through the narrowed pyloric canal, gastric distension combined with unproductive contractions, and, less commonly, gastroesophageal reflux. Although history and clinical examination are the main diagnostic means, ultrasound should be the initial imaging procedure if the clinical diagnosis is doubtful.--Prem Puri
CircumumbUical Incision for Pyloromyotomy. K.C. Tan and A.
Bianchi. Br J Surg 73:399, (May), 1986. The authors describe a series of 40 children, aged 3 to 8 weeks, with infantile hypertrophic pyloric stenosis who had a Ramstedt pyloromyotomy through a circumumbilical incision. Delivery of the pylorus was relatively easy. Seven children required antibiotics for a cellulitic flare around the wound with one purulent discharging wound requiring drainage. There was one wound dehiscence. All others healed primarily with a resultant scar that was hardly visible. The appearance was cosmetically superior in the long-term to other incisions commonly used.--Tony Sparnon
Value of a Simple Biochemical Ratio in Distinguishing Upper and Lower Sites of Gastrointestinal Haemorrhage. J.A. Snook, G.E.
Holdstock, and J. Barnforth. Lancet i: 1064-1065, (May 10), 1986. Fifty-nine cases of overt gastrointestinal haemorrhage were reviewed in which the source of bleeding was not initially evident but was identified by later investigation. The admission plasma urea to creatinine concentration ratio proved highly accurate in distinguishing upper and lower gastrointestinal sources of haemorrhage. Regardless of blood volume lost, 33 of 38 patients with upper gastrointestinal haemorrhage (87%) had a ratio of 100 or more, and
INTERNATIONAL ABSTRACTS
291
20 of 21 patients with lower gastrointestinal haemorrhage (95%) had a ratio of less than 100, an overall accuracy of 90%. Elevation of the ratio was not related to clinical evidence of hypovolemia. This simple ratio may be valuable in determining the sequence of investigations in patients with an unidentified source of gastrointestinal haemorrhage.--Prem Purl Unusual Association of Upper Gastro-lntestinal Anomalies in a Newborn. M. Dassonville, J.M. Bouton, L. Verstreken, et al. Z
Kinderchir 41:112-113, (April), 1986. A newborn with persistent nonbilious vomiting is described. The postprandial vomiting was due to hypertrophic pyloric stenosis, intestinal malrotation, and gastric antral diaphragm. The anomalies were successfully treated by three subsequent operations. The follow-up was normal for 2 years with weight and size at the 90th percentile.-- Thomas A. Angerpointner Surgical Management of Multiple Intestinal Atresias. E. L Hatch
and R. T. Schaller, Jr. Am J Surg 151:550-552, (May), 1986. This is basically a review paper stimulated by recent improved techniques utilized in the management of intestinal atresia. Principles of tapering or resection, maximal preservation of bowd length, and insurance of distal patency are emphasized. The importance of parenteral nutrition can also not be underemphasized.--Thomas V. Whalen Torsion of a Caecal Diverticulum in a Young Child, G.A.D. McPher-
son, S. Holland, and H.B. Ross. Br J Surg 72:714, (September), 1985. A 2-year-old boy presented with a three-day history of vomiting and a tender distended abdomen. Plain abdominal x-rays demonstrated a large air-filled viscus on the right side of the abdomen. Laparotomy revealed a 10 cm diameter diverticulum arising from the medial border of the cecum which had undergone torsion of its ped~cle. It was removed successfully. These rare diverticula usually mimic acute appendicitis. Sometimes they present as a hard mass. Melena has also been reported. Removal of an isolated asymptomatic diverticulum found incidentally at laparotomy is recommended.--N.P. Madden Crohn's Disease: Maintenance of Remission by Diet. V.A.
Jones,
R.J. Dickinson, E. Workman, et al. Lancet 8448:177-180, (July), 1985. Twenty patients with Crohn's disease took part in a controled trial in which remission was maintained by either an unrefined carbohydrate fiber-rich diet or a diet that excluded specific foods to which a patient was intolerant. Seven out of the ten patients on the exclusion diet remained in remission for 6 months compared with none out of the ten on an unrefined carbohydrate fiber-rich diet (P < .05, Fisher's exact test). In an uncontroled study an exclusion diet allowed 51 out of 77 patients to remain well on the diet alone for periods of up to 51 months, and with an average annual relapse rate of less than lO%.--Prem Purl Endotoxaemia in Active Crohn's Disease. T r e a t m e n t W i t h W h o l e Gut Irrigation and B-Aminosalicyclic Acid. W. Wellman, P.C. Fink,
F. Benner, et al. Gut 27:814-820, (July), 1986. Endotoxins in plasma were monitored during treatment in 18 patients hospitalized for acute exacerbation of Crohn's disease. Systemic endotoxemia was found on admission in all but one. The patients were randomly divided into two groups: one receiving treatment with total parenteral nutrition and steroids. To decrease
the absorbable endotoxin pool, the other group was additionally treated with whole gut irrigation, and 5-aminosalicylie acid was added to the lavage fluid. In most of these patients endotoxemia cleared after intestinal lavage and they needed shorter hospitalization. Earlier improvement was also indicated by a faster decrease of the Crohn's disease activity index and vanHees index. In the group receiving conservative treatment alone, endotoxemia was controled within 3 weeks. The authors conclude that endotoxemia occurs in most patients suffering from active Crohn's disease. Control of endotoxemia after intestinal lavage suggests that systemic endotoxemia is caused by absorption of endotoxins from the gut. Earlier improvement after whole gut irrigation indicates its beneficial effect in active Crohn's disease.--Prem Purl
Relationship Between Disease Activity Indices and Colonoscopic Findings in Patients W i t h Colonic Inflammatory Bowel Disease, P.
Gomes, Claire Du Boulay, C.L. Smith, et al. Gut 27:92-95, (January), 1986. The Crohn's disease activity index, a similar index devised for patients with ulcerative colitis, and other commonly used laboratory indicators of disease activity have been studied in 50 patients with colonic inflammatory bowel disease undergoing routine colonoseopic assessment and compared with the histologic extent and activity of disease. There was only poor correlation between the colonoscopic or histologic findings and the indices of disease activity studied, showing that these are not reliable measures of disease activity or extent at the tissue level.--Prem Purl
Pseudomembranous Colitis in Children and Adults. R.P. Bolton and D.F.M. Thomas. Br J Hosp Med 35:37-42, (January), 1986.
The etiology, clinical features, and specific therapy of antibiotic associated Pseudomembranous colitis is presented. Identification of Clostridium diffieile as the major pathogen is discussed in relation to the rational approach to therapy and associated disease.--Amir Azmy
Hirschsprung's Disease in the Newborn. An 11 Year Experience.
T.Z. Polley, Jr, and A.G. Coran. Pediatr Surg Int 1:80-83, (June), 1986. The records of all patients with Hirschsprung's disease diagnosed and treated at the authors' institution between July 1974 and August 1985 were reviewed. Of these 99 patients, 35 (35%) presented and were diagnosed within the first 30 days of life and constitute the basis of the present study group. Twenty-two infants (63%) had standard reetosigmoid disease with a male-to-female ratio of 2.2:1. Only one infant was premature. The spectrum of presenting signs included abdominal distension in I9 (54%), failure to pass meconium within the first 48 hours of life in 16 (46%), "constipation" in 12 (34%), and vomiting in 9 (26%). Intestinal perforation was a presenting sign in two patients (6%), and enterocolitis occurred preoperatively in four (12%). Evaluation was facilitated by diagnostic barium enema in 60% of the patients. In those infants able to undergo elective evaluation, the definitive diagnosis was made by suction rectal biopsy, which was accurate in all cases. In addition to the high proportion of patients with long-segment disease (13 patients, 37%), there was a significant incidence of associated congenital anomalies (26%), including Down's syndrome in five (14%). Thirty-three of the 35 patients underwent definitive treatment using the endorectal pull-through procedure performed at an average age of 12 months. There was no mortality related to the operation. In addition to highlighting the high incidence of congeni-