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Anorectal malformations associated with a presacral tumor and sacral defect
INTERNATIONAL
573
ABSTRACTS
Total Colonic Aganglionosis: Treatment and Follow-Up. C. Festen,
R. Severijnen.
F.v.d. Stauk, et al. 2 Kinderchir 44: 153- 155, (June),
1989. Eleven cases of total colonic aganglionosis were reviewed. Better knowledge of the clinical picture and greater alertness for the occurrence of this disease has led to earlier diagnosis. Definitive operative treatment is still controversial. Following ileostomy, a crucial point seems to be the allowance of sufficient time for the small bowel to adapt its absorptive ability. The authors are of the opinion that little is to be gained by the application of a difficult procedure, such as Martin’s operation, which involves the risk of serious complications. Their experience confirms that a standard Hischsprung’s operation is, in general, satisfactory. At present (mean follow-up, 6.7 years) all patients show normal growth and development, have no serious perianal problems, and are, with one exception, continent for feces.-Thomas A. Angerpointner Causes of Late Complicntions in Children Operated on for Hirschsprung’s Disease: A Preliminary Immunohistochemical Investigation Using Polyclonal Antibodies Against S-100 Protein. K. Joosten,
M. Pruszczynski,
R.S. V.&f. Severijnen,
et al. Z Kinderchir 44:213-
215, (August), 1989. Late follow-up of 5 1 children operated on for the classical form of Hirschsprung’s disease showed a large number of unsatisfactory results. It is well known from the literature that achalasia of the anal sphincter is one of the most important causes. Twenty-five patients underwent redilatation of the anal sphincter. Complaints in the form of constipation or diarrhea remained in 40% of the cases secondary to redilatation. Reinvestigation of the resected colon specimens using the conventional histological method disclosed the presence of ganglion cells in the proximal resection area in all patients. In the light of reports in the literature stating that despite normal histology innervation disturbances can, nevertheless, occur in the colon, the authors performed a pilot study using polyclonal antibodies against the S-100 protein. In three of four patients who were adequately resected in the sense of conventional histology, clear innervation abnormalities could be found by means of polyclonal antibody staining. Three of those four patients presented with unsatisfactory late results. It remains unclear whether the abnormal S-100 staining is limited to one segment or extends over the whole colon. The question also remains as to whether maturation occurs so that some improvement may be expected on a long-term basis with increasing age.-Thomas
A. Angerpointner
Anorectal Malformations Associated With a Presacral Tumor and Sacral Defect. Y. Tsuchida,
W. Waianasupt,
and T. Nakajo.
Pedi-
atr Surg Int 4:398-402, (October), 1989. The triad of anorectal malformation, presacral mass, and sacral bony defect is quite rare. During the past 18 years, five patients with this triad were treated in the authors’ institutions. Four had covered anal or anorectal stenosis; one infant had anorectal agenesis. There was a central defect of the sacral bone in three and sacral hemivertebrae in two patients. An additional case that had an intact sacrum but was otherwise associated with the same anomalies is also reported. In the literature, the authors could find 51 cases (including five cases of their own) that fall under this symptom complex. All patients had a presacral tumor, the most common being teratomas and meningoceles (20 cases each). Anal or anorectal stenoses were most frequently encountered (46 cases), whereas malformations such as anorectal agenesis (3) or anorectal stenosis plus rectovaginal fistula (2) were noted less frequently. Therefore, the authors contend that the spectrum of this symptom complex is broader than was previously considered. Among these patients, constipation was the
most common presenting symptom. Barium enema and computed tomographic studies were helpful in identifying the presence, extent, and nature of the mass. The embryogenesis is discussed, reviewing the clinical and pathological characteristics of these patients.Prem Puri
ABDOMEN The Efficacy of Hepatoportoenterostomy in Biliary Atresia. J.L.
Grosfeld,
J.F. Fitzgerald,
R. Predaina, et al. Surgery 106:692-701,
(October), 1989. This series outlines the treatment and outcome of 66 infants with biliary atresia. Forty-eight of these patients underwent hepatoportoenterostomy (HPE) and four underwent hepatoportocholecystostomy. Twenty-five percent of these patients had successful HPE, with improvement in 19% and no improvement in 43%. The success was predicted by the time of operation with a 31% overall success for those patients who underwent surgery at less than, 90 days of age. A number of different procedures were utilized in this series, with a preference for the Suruga and Sawaguchi procedures. Stomas allowed for bile clearance studies determined by ostomy:serum ratios. These ratios were found to be good prognostic determinants when greater than 15 at 12 weeks postsurgery. Reoperation for complications was only successful in that group of patients who had an initial clearance of jaundice. The report continues to describe those patients who were referred for liver transplantation and highlights the “hidden mortality” of those patients that die on transplant waiting lists.-Tom Tracy. Jr Long-Term Follow-Up Study of Petients With Cholangitb After Successful Kasai Operation in Biliary Atresia: Selection of Recipients for Liver Transplantation. N. Ohkohchi, T. Chiba, R. Ohi, et al. J Pediatr Gastroenterol Nutr 9:416-420, (November), 1989.
This report of 131 patients from Sendai, Japan covers the time period from 1970 to 1987. In 39 of these patients, jaundice persisted after portoenterostomy, and there were no survivors in this group IO years after the operation. Of those patients without jaundice, 90% were alive after 10 years. Twenty patients died after initially successful portoenterostomy, but the records of nine were reviewed in order to evaluate clinical outcome following episodes of recurrent cholangitis. In those nine patients, when the total bilirubin concentration rose above 10 mg/dL, there was no benefit derived from conservative therapy that included antibiotics, steroids, and choleretic agents. Apart from serum bilirubin levels, there was no prognostic value for liver enzyme and coagulation parameters. From their review of this group of patients, the authors recommend early transplantation for those patients with persistent jaundice after attempted portoenterostomy. It also appears that bilirubin levels greater than 10 mg/dL during bouts of cholangitis are unresponsive to conservative medical treatment and are highly predictive of impending hepatic failure or gastrointestinal bleeding and death within the year. Therefore, these patients should be prepared for liver transplantationTom Tracy, Jr Peritoneal
Dialysis for
Hemolytic-Uremic
Svndrome
in Post-
operative Peritonitis Following Ileocecal Resectien for Intussusception. C. Deindl. R. Eife. and H.-G. Dietz.
Z Kinderchir 44: 169- 171,
(June), 1989. The case of a 3-year-old girl who underwent laparotomy for ileocecal intussusception is reported. Immediately following laparotomy, she developed a hemolyticuremic syndrome with hemolytic anemia, thrombocytopenia, increased serum urea and creatine levels, and anuria. She also developed a subsequent peritonitis, enterocolitis, and sepsis. Following relaparotomy with establishment of an
573
ABSTRACTS
Total Colonic Aganglionosis: Treatment and Follow-Up. C. Festen,
R. Severijnen.
F.v.d. Stauk, et al. 2 Kinderchir 44: 153- 155, (June),
1989. Eleven cases of total colonic aganglionosis were reviewed. Better knowledge of the clinical picture and greater alertness for the occurrence of this disease has led to earlier diagnosis. Definitive operative treatment is still controversial. Following ileostomy, a crucial point seems to be the allowance of sufficient time for the small bowel to adapt its absorptive ability. The authors are of the opinion that little is to be gained by the application of a difficult procedure, such as Martin’s operation, which involves the risk of serious complications. Their experience confirms that a standard Hischsprung’s operation is, in general, satisfactory. At present (mean follow-up, 6.7 years) all patients show normal growth and development, have no serious perianal problems, and are, with one exception, continent for feces.-Thomas A. Angerpointner Causes of Late Complicntions in Children Operated on for Hirschsprung’s Disease: A Preliminary Immunohistochemical Investigation Using Polyclonal Antibodies Against S-100 Protein. K. Joosten,
M. Pruszczynski,
R.S. V.&f. Severijnen,
et al. Z Kinderchir 44:213-
215, (August), 1989. Late follow-up of 5 1 children operated on for the classical form of Hirschsprung’s disease showed a large number of unsatisfactory results. It is well known from the literature that achalasia of the anal sphincter is one of the most important causes. Twenty-five patients underwent redilatation of the anal sphincter. Complaints in the form of constipation or diarrhea remained in 40% of the cases secondary to redilatation. Reinvestigation of the resected colon specimens using the conventional histological method disclosed the presence of ganglion cells in the proximal resection area in all patients. In the light of reports in the literature stating that despite normal histology innervation disturbances can, nevertheless, occur in the colon, the authors performed a pilot study using polyclonal antibodies against the S-100 protein. In three of four patients who were adequately resected in the sense of conventional histology, clear innervation abnormalities could be found by means of polyclonal antibody staining. Three of those four patients presented with unsatisfactory late results. It remains unclear whether the abnormal S-100 staining is limited to one segment or extends over the whole colon. The question also remains as to whether maturation occurs so that some improvement may be expected on a long-term basis with increasing age.-Thomas
A. Angerpointner
Anorectal Malformations Associated With a Presacral Tumor and Sacral Defect. Y. Tsuchida,
W. Waianasupt,
and T. Nakajo.
Pedi-
atr Surg Int 4:398-402, (October), 1989. The triad of anorectal malformation, presacral mass, and sacral bony defect is quite rare. During the past 18 years, five patients with this triad were treated in the authors’ institutions. Four had covered anal or anorectal stenosis; one infant had anorectal agenesis. There was a central defect of the sacral bone in three and sacral hemivertebrae in two patients. An additional case that had an intact sacrum but was otherwise associated with the same anomalies is also reported. In the literature, the authors could find 51 cases (including five cases of their own) that fall under this symptom complex. All patients had a presacral tumor, the most common being teratomas and meningoceles (20 cases each). Anal or anorectal stenoses were most frequently encountered (46 cases), whereas malformations such as anorectal agenesis (3) or anorectal stenosis plus rectovaginal fistula (2) were noted less frequently. Therefore, the authors contend that the spectrum of this symptom complex is broader than was previously considered. Among these patients, constipation was the
most common presenting symptom. Barium enema and computed tomographic studies were helpful in identifying the presence, extent, and nature of the mass. The embryogenesis is discussed, reviewing the clinical and pathological characteristics of these patients.Prem Puri
ABDOMEN The Efficacy of Hepatoportoenterostomy in Biliary Atresia. J.L.
Grosfeld,
J.F. Fitzgerald,
R. Predaina, et al. Surgery 106:692-701,
(October), 1989. This series outlines the treatment and outcome of 66 infants with biliary atresia. Forty-eight of these patients underwent hepatoportoenterostomy (HPE) and four underwent hepatoportocholecystostomy. Twenty-five percent of these patients had successful HPE, with improvement in 19% and no improvement in 43%. The success was predicted by the time of operation with a 31% overall success for those patients who underwent surgery at less than, 90 days of age. A number of different procedures were utilized in this series, with a preference for the Suruga and Sawaguchi procedures. Stomas allowed for bile clearance studies determined by ostomy:serum ratios. These ratios were found to be good prognostic determinants when greater than 15 at 12 weeks postsurgery. Reoperation for complications was only successful in that group of patients who had an initial clearance of jaundice. The report continues to describe those patients who were referred for liver transplantation and highlights the “hidden mortality” of those patients that die on transplant waiting lists.-Tom Tracy. Jr Long-Term Follow-Up Study of Petients With Cholangitb After Successful Kasai Operation in Biliary Atresia: Selection of Recipients for Liver Transplantation. N. Ohkohchi, T. Chiba, R. Ohi, et al. J Pediatr Gastroenterol Nutr 9:416-420, (November), 1989.
This report of 131 patients from Sendai, Japan covers the time period from 1970 to 1987. In 39 of these patients, jaundice persisted after portoenterostomy, and there were no survivors in this group IO years after the operation. Of those patients without jaundice, 90% were alive after 10 years. Twenty patients died after initially successful portoenterostomy, but the records of nine were reviewed in order to evaluate clinical outcome following episodes of recurrent cholangitis. In those nine patients, when the total bilirubin concentration rose above 10 mg/dL, there was no benefit derived from conservative therapy that included antibiotics, steroids, and choleretic agents. Apart from serum bilirubin levels, there was no prognostic value for liver enzyme and coagulation parameters. From their review of this group of patients, the authors recommend early transplantation for those patients with persistent jaundice after attempted portoenterostomy. It also appears that bilirubin levels greater than 10 mg/dL during bouts of cholangitis are unresponsive to conservative medical treatment and are highly predictive of impending hepatic failure or gastrointestinal bleeding and death within the year. Therefore, these patients should be prepared for liver transplantationTom Tracy, Jr Peritoneal
Dialysis for
Hemolytic-Uremic
Svndrome
in Post-
operative Peritonitis Following Ileocecal Resectien for Intussusception. C. Deindl. R. Eife. and H.-G. Dietz.
Z Kinderchir 44: 169- 171,
(June), 1989. The case of a 3-year-old girl who underwent laparotomy for ileocecal intussusception is reported. Immediately following laparotomy, she developed a hemolyticuremic syndrome with hemolytic anemia, thrombocytopenia, increased serum urea and creatine levels, and anuria. She also developed a subsequent peritonitis, enterocolitis, and sepsis. Following relaparotomy with establishment of an