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Simplified retrorectal pull-through procedure for Hirschsprung's disease
INTERNATIONAL ABSTRACTS
292
tal anomalies, the large proportion of neonates with long-segment disease, and the reliability of the diagnostic barium enema, this subgroup of patients with Hirschsprung's disease emphasizes the special diagnostic and management considerations required in the newborn infant who presents with sepsis of unknown etiology, intestinal obstruction, or constipation. A high index of suspicion, liberal use of suction rectal biopsy, early leveling colostomy, and definitive treatment by endorectal pull-through are important in achieving 0% operative mortality and excellent functional results.-Prem Puri
only four late deaths have been observed (three with Down's syndrome). Fecaloma occurred in 12 of 14 patients who underwent the classic operation described by Duhamel, and postoperative enterocolitis was noted in four of six patients who had Martin's "long" anastomosis. No urologic problems or anastomotic leaks were identified, and only one patient developed a rectal stricture postoperatively. Growth and development and fecal continence have been excellent in the vast majority of patients. The modified Duhamel pull-through operation is associated with excellent survival and highly acceptable long-term function.--Prem Puri
Aganglionosis of the Entire Bowel: Four New Cases and Review of the Literature. M. Di Lorenzo, S. Yasbeck, and P. Brochu. Br J
Management of Hirschsprung's Disease With the Swenson Procedure With Emphasis on Long-term Follow-up. J.J. Weitzman.
Surg 72:657-658, (August), 1985.
Pediatr Surg lnt 1:100o104, (June), 1986.
Four cases of aganglionosis of the entire bowel were identified over a 10-year period. All were male and presented within 48 hours of birth with bile stained vomiting, abdominal distension, and failure to pass meconium. On barium enema all four failed to show reflux of contrast into the ileum. There was delayed evacuation, but appearances were not otherwise remarkable. Macroscopic findings at laparotomy were unreliable. Two of the infants were identical twins. One of these was found to have a 75 cm "skip" area with normal ganglion cells in the small bowel, but the duodenum and first part of the jejunum were aganglionic. All four babies died during the neonatal period, and renal anomalies were found at autopsy. The only means of identifying the extent of aganglionosis is by serial biopsies.--N.P. Madden
In a 20-year period (1965 to 1986), 80 patients with Hirschsprung's disease were managed with the Swenson procedure. Twothirds of the patients had disease limited to the rectosigmoid and sigmoid colon. In the remaining patients, aganglionosis extended into variable lengths of colon, including five instances of total colonic aganglionosis. All patients had a protective enterostomy at the time of pull-through. There was no operative mortality. There was one late death due to fulminating enterocolitis. All but one of the remaining patients are in good health. One patient, lost to follow-up for 10 years, has chronic enterocolitis. No patients, including seven who had anastomotic leaks, ever had problems with rectal stricture. There are no instances of urinary dysfunction or problems with male sexual function. Sixty-one patients have had long-term follow-up. Fifty two have completely normal rectal function, although in some instances it was not achieved for several years. Eight patients are too young to evaluate. Four patients have minor problems with occasional fecal soiling but are expected to recover. Only two of five mentally retarded patients are becoming toilet trained. Despite the appearance of many other operations designed to cure Hirschsprung's disease, the Swenson procedure is still employed by many surgeons. Long-term follow-up is necessary for final evaluation of rectal function.--Prem Puri
The Definitive Management of Hirschsprung's Disease With the Endorectal Pull-through Procedure. T.Z. Polley, Jr, A.G. Coran,
and J.R. Wesley. Pediatr Surg Int 1:90-94, (June), 1986. From July 1974 to August 1985, 99 patients were treated for Hirschsprung's disease. Seventy-five were initially diagnosed and treated at the authors' institution. Of these 75 patients, 55 had standard rectosigmoid disease and 20 had long-segment disease. The endorectal pull-through (ERPT) procedure was used consecutively in 73 of the 75 patients. The other two children died, one because of total intestinal aganglionosis and the other due to severe congenital anomalies, prior to a definitive procedure. Of the 24 patients referred by other institutions, 12 underwent repeat pull-through procedures, and 12 required lesser operations to deal with the complications of prior procedures. There were no postoperative deaths and no permanent enterostomies among the 73 consecutive ERPT patients. The follow-up in this series ranges from 4 months to 10 years, and all patients have fecal continence with an average of 3 to 4 stools daily. Our treatment plan involves the use of suction rectal biopsy for diagnosis and leveling colostomy in the neonatal period. An endorectal pull-through procedure is then carried out between 8 months and 1 year of age. The excellent functional results, 0% operative mortality, low morbidity, and technical ease of performing the ERPT underscore the authors enthusiasm for continued use of this procedure in Hirschsprung's disease.--Prem Puri
Simplified Retrorectal Pull-Through Procedure for Hirschsprung's Disease. E.S. Golladay and D.L. Mollitt. South Med J 79:840-843,
(July), 1986. The authors have used a modified Duhamel pull-through procedure by using the ILS circular anastomotic stapler to fashion the end-to-side colorectal anastomosis in six children with Hirschsprung's disease. The GIA stapler is used to anastomose and remove a portion of the common wall between the colon and the rectum. Ages of these children ranged from 4 to 15 months at the time of operation. The authors believe this modification allows a more rapid, wide, and precise anastomosis than previous techniques. There have been no complications.--George Holcomb, Jr
ABDOMEN Gas in the Hepatic Portal Veins. D.M. Oriffiths and M.H. Gough.
Hirschsprung's Disease. Experience With the Duhamel Operation in 1 9 5 C a s e s . D.W. Vane and J.L. Grosfeld. Pediatr Surg Int
1:95-99, (June), 1986. From 1972 to 1985, 214 patients were treated for Hirschsprung's disease. One patient underwent a Swenson pull-through operation, 13 a Soave procedure, and 195 had modifications of the Duhamel procedure. Six children are newly diagnosed and awaiting definitive therapy. Of the patients in whom a modified Duhamel procedure was performed, 153 were males (78%) and seven (4%) had Down's syndrome. There was no operative mortality for the procedure, and
Br J Surg 73:172-176, (March), 1986. This article reviews hepatic portal vein gas. It is not a specific disease, but a feature common to many serious illnesses. It may be diagnosed by a plain abdominal radiograph, ultrasound, or CT scan. The appearance of tubular lucencies is seen branching horizontally from the porta hepatis to the liver edge. Intestinal mucosal ulceration and radiographical bowel distension occur in 85% of cases. There are many iatrogenic and pathologic causes, the majority being associated with necrosis of bowel mucosa. The clinical course is usually one of rapidly progressive deterioration, and there is a high
292
tal anomalies, the large proportion of neonates with long-segment disease, and the reliability of the diagnostic barium enema, this subgroup of patients with Hirschsprung's disease emphasizes the special diagnostic and management considerations required in the newborn infant who presents with sepsis of unknown etiology, intestinal obstruction, or constipation. A high index of suspicion, liberal use of suction rectal biopsy, early leveling colostomy, and definitive treatment by endorectal pull-through are important in achieving 0% operative mortality and excellent functional results.-Prem Puri
only four late deaths have been observed (three with Down's syndrome). Fecaloma occurred in 12 of 14 patients who underwent the classic operation described by Duhamel, and postoperative enterocolitis was noted in four of six patients who had Martin's "long" anastomosis. No urologic problems or anastomotic leaks were identified, and only one patient developed a rectal stricture postoperatively. Growth and development and fecal continence have been excellent in the vast majority of patients. The modified Duhamel pull-through operation is associated with excellent survival and highly acceptable long-term function.--Prem Puri
Aganglionosis of the Entire Bowel: Four New Cases and Review of the Literature. M. Di Lorenzo, S. Yasbeck, and P. Brochu. Br J
Management of Hirschsprung's Disease With the Swenson Procedure With Emphasis on Long-term Follow-up. J.J. Weitzman.
Surg 72:657-658, (August), 1985.
Pediatr Surg lnt 1:100o104, (June), 1986.
Four cases of aganglionosis of the entire bowel were identified over a 10-year period. All were male and presented within 48 hours of birth with bile stained vomiting, abdominal distension, and failure to pass meconium. On barium enema all four failed to show reflux of contrast into the ileum. There was delayed evacuation, but appearances were not otherwise remarkable. Macroscopic findings at laparotomy were unreliable. Two of the infants were identical twins. One of these was found to have a 75 cm "skip" area with normal ganglion cells in the small bowel, but the duodenum and first part of the jejunum were aganglionic. All four babies died during the neonatal period, and renal anomalies were found at autopsy. The only means of identifying the extent of aganglionosis is by serial biopsies.--N.P. Madden
In a 20-year period (1965 to 1986), 80 patients with Hirschsprung's disease were managed with the Swenson procedure. Twothirds of the patients had disease limited to the rectosigmoid and sigmoid colon. In the remaining patients, aganglionosis extended into variable lengths of colon, including five instances of total colonic aganglionosis. All patients had a protective enterostomy at the time of pull-through. There was no operative mortality. There was one late death due to fulminating enterocolitis. All but one of the remaining patients are in good health. One patient, lost to follow-up for 10 years, has chronic enterocolitis. No patients, including seven who had anastomotic leaks, ever had problems with rectal stricture. There are no instances of urinary dysfunction or problems with male sexual function. Sixty-one patients have had long-term follow-up. Fifty two have completely normal rectal function, although in some instances it was not achieved for several years. Eight patients are too young to evaluate. Four patients have minor problems with occasional fecal soiling but are expected to recover. Only two of five mentally retarded patients are becoming toilet trained. Despite the appearance of many other operations designed to cure Hirschsprung's disease, the Swenson procedure is still employed by many surgeons. Long-term follow-up is necessary for final evaluation of rectal function.--Prem Puri
The Definitive Management of Hirschsprung's Disease With the Endorectal Pull-through Procedure. T.Z. Polley, Jr, A.G. Coran,
and J.R. Wesley. Pediatr Surg Int 1:90-94, (June), 1986. From July 1974 to August 1985, 99 patients were treated for Hirschsprung's disease. Seventy-five were initially diagnosed and treated at the authors' institution. Of these 75 patients, 55 had standard rectosigmoid disease and 20 had long-segment disease. The endorectal pull-through (ERPT) procedure was used consecutively in 73 of the 75 patients. The other two children died, one because of total intestinal aganglionosis and the other due to severe congenital anomalies, prior to a definitive procedure. Of the 24 patients referred by other institutions, 12 underwent repeat pull-through procedures, and 12 required lesser operations to deal with the complications of prior procedures. There were no postoperative deaths and no permanent enterostomies among the 73 consecutive ERPT patients. The follow-up in this series ranges from 4 months to 10 years, and all patients have fecal continence with an average of 3 to 4 stools daily. Our treatment plan involves the use of suction rectal biopsy for diagnosis and leveling colostomy in the neonatal period. An endorectal pull-through procedure is then carried out between 8 months and 1 year of age. The excellent functional results, 0% operative mortality, low morbidity, and technical ease of performing the ERPT underscore the authors enthusiasm for continued use of this procedure in Hirschsprung's disease.--Prem Puri
Simplified Retrorectal Pull-Through Procedure for Hirschsprung's Disease. E.S. Golladay and D.L. Mollitt. South Med J 79:840-843,
(July), 1986. The authors have used a modified Duhamel pull-through procedure by using the ILS circular anastomotic stapler to fashion the end-to-side colorectal anastomosis in six children with Hirschsprung's disease. The GIA stapler is used to anastomose and remove a portion of the common wall between the colon and the rectum. Ages of these children ranged from 4 to 15 months at the time of operation. The authors believe this modification allows a more rapid, wide, and precise anastomosis than previous techniques. There have been no complications.--George Holcomb, Jr
ABDOMEN Gas in the Hepatic Portal Veins. D.M. Oriffiths and M.H. Gough.
Hirschsprung's Disease. Experience With the Duhamel Operation in 1 9 5 C a s e s . D.W. Vane and J.L. Grosfeld. Pediatr Surg Int
1:95-99, (June), 1986. From 1972 to 1985, 214 patients were treated for Hirschsprung's disease. One patient underwent a Swenson pull-through operation, 13 a Soave procedure, and 195 had modifications of the Duhamel procedure. Six children are newly diagnosed and awaiting definitive therapy. Of the patients in whom a modified Duhamel procedure was performed, 153 were males (78%) and seven (4%) had Down's syndrome. There was no operative mortality for the procedure, and
Br J Surg 73:172-176, (March), 1986. This article reviews hepatic portal vein gas. It is not a specific disease, but a feature common to many serious illnesses. It may be diagnosed by a plain abdominal radiograph, ultrasound, or CT scan. The appearance of tubular lucencies is seen branching horizontally from the porta hepatis to the liver edge. Intestinal mucosal ulceration and radiographical bowel distension occur in 85% of cases. There are many iatrogenic and pathologic causes, the majority being associated with necrosis of bowel mucosa. The clinical course is usually one of rapidly progressive deterioration, and there is a high