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Anorectal manometry in the diagnosis of Hirschsprung's disease—Comparison with clinical and radiological criteria
ABSTRACTS
has been shown to induce a remission in a large number of patients with Crohn's disease. Younger patients with Crohn's disease have an increased rate of recurrence, 70% compared with a rate of 30% for those over 40. Ulcerative colitis in childhood presents fewer problems than Crohn's disease because the diagnosis is easier, proctocolectomy is curative, medical treatment is more clearly defined, and growth retardation is much less common. However, children are more likely to have active and more extensive disease than adults, with 90% of children having moderate to severe disease. Of those patients who present with fulminant colitis, only one-third will respond quickly to medical management, but the remission is short-lived and the majority come to surgery sooner rather than later. Almost every child developing toxic megacolon comes to early surgery within 48 to 72 hours. Ultimately, proctocolectomy is curative, although sphincter-saving procedures and anorectal pull-throughs are now showing encouraging results. The prognosis in Crohn's disease is in part determined by the site of the disease, in that children with ileocolic lesions fair worse than children with isolated small bowel disease not involving the duodenum. Also, patients under 20 not only have a high recurrence rate, but also have a significantly higher mortality rate than their older counterparts. The prognosis for ulcerative colitis is probably better than that reported in a large retrospective study from the Mayo Clinic (1918 1965) which found a mortality rate of 20% per decade for those in whom total colitis began in childhood. That study also found that in patients with ulcerative colitis, after the first decade, the risk of developing cancer was 20% per decade.--Richard R. Ricketts
Perianal Disease in Children and Adolescents with Crohn's
Disease. J. Markowitz, F. Daum, H. Aifes, et al. A m J Gastroentol 86:829-833, (May), 1984. One hundred forty-nine children and adolescents with Crohn's disease seen between 1972 and 1982 were reviewed with respect to the presence of perianal disease. Perianal disease was defined as, (a) anal canal lesions consisting of large, edematous skin tags and/or deep anal fissures, (b) fistulas, and (c) abscesses. Seventy-three patients (49%) had perianal disease. Patients with perianal disease had essentially the same incidence of active rectal disease as those without perianal disease (61% v 49%). However, those patients with the most severe perianal disease, fistulas, and abscesses, all had active rectal involvement. The frequency of granulomata in the rectal biopsy was significantly greater in the perianal disease group compared with the nonperianal group (30% v 9%). For patients with only fissures or tags, local care and scrupulous perianal hygiene were adequate treatment. Patients with fistulas or abscesses often required surgical therapy. Proper recognition of the perianal lesions seen in children with Crohn's disease is of clinical importance since perianal disease has preceded the development of intestinal symptoms in 10% to 25% of patients with Crohn's disease. Children with "hemorrhoids" should be suspected of having Crohn's disease and, therefore, should be evaluated appropriately.--Richard R. Ricketts
885 Clinical Impact of Colectomy and Ileorectal Anastomosis in the Management of Crohn's Disease, N.S. Ambrose,
M.R.B. Keighley, J. Alexander-Williams, et al. Gut 25:223227, (March), 1984. Sixty-three patients with Crohn's colitis were treated by colectomy and ileorectal anastomosis between 1951 and 1981. Patients were not considered for this procedure if they had several perianal disease or if the rectum was grossly abnormal. The mean follow-up since colectomy in this group of patients was 9.5 years. The cumulative recurrence rates increased with time and reached 64% at ten years. The cumulative reoperation rates also increased with time and was 48% in ten years. Recurrence developing in the ileum proximal to the anastomosis often was resected and intestinal continuity preserved, whereas anorectal recurrence usually required proctectomy. In spite of the high recurrence rate, this procedure has a useful place in restoring the chronically ill patient to good health and it avoids the morbidity of a permanent stoma, pelvic dissection, and rectal e x c i s i o n . Richard R. Ricketts Diagnosis and Treatment of Hirschsprung's Disease in
Japan. K. Ikeda, and S. Goto. A n n Surg 199:400-405, (April), 1984. A nationwide survey in Japan of 1,628 patients with Hirschsprung's Disease from 135 institutions is reported. Diagnosis was generally made by barium enema, anorectal manometry, and histochemical studies. Anorectal manometry has been used increasingly in combination with barium enema for diagnosis. Forty-eight per cent were diagnosed in the first month of life and 83.4% by the end of the first year. The overall incidence of associated anomalies was 11.1% with Down's syndrome and cardiac anomalies being the most frequent. Almost 80% of patients had aganglionosis limited to the rectum and the sigmoid colon. The incidence of total colonic with or without small bowel aganglionosis was 8.5%. The overall incidence of preoperative enterocolitis was 29.2%. Enterostomy was created in 61.3% with disease to the sigmoid, 95.5% in those with right colon extention, and 97.1% in total colonic with our without small bowel aganglionosis. Retrorectal transanal pull-through as described by Duhamel and modified by Ikeda was the most common definitive operation. The Z-shaped anastomosis described by Ikeda was used in 30.7% of all procedures. Endorectal pull-through was used in 27.6% as the definitive operation. The mortality rate was highest in patients with total calonic aganglionosis. Sepsis was the most frequent cause of death.--Richard J. Andrassy Anorectal Manometry in the Diagnosis of Hirschsprung's Disease--Comparison With Clinical and Radiological Criteria. G.A. Lanfranchi, G. Bazzocchi, S. Federici, et al. A m J
Gastroento179:270-275, (April), 1984. Thirty-four patients, 21 males and 13 females (average age: 4.8 years; range 17 days through 16 years) in whom the diagnosis of Hirschsprung's disease was suspected, were analyzed with respect to the reliability of clinical symptoms, radiological parameters, and anorectal manometry in arriv-
886
ABSTRACTS
ing at the correct diagnosis. Nineteen patients had histologically proven Hirschsprung's disease while the remaining 15 cases had idiopathic constipation. In this study, anorectal manometry correctly diagnosed all patients. The manometric parameters that were found diagnostic of Hirschsprung's disease included (a) an absent rectoanal inhibitory reflex, (b) a significant lower anal resting pressure, (c) a lower pain threshhold, and (d) a decreased frequency of spontaneous rhythmic oscillations. It should be noted, however, that the average age of the patients with aganglionosis was 4.3 years and that only four children less than one year were included in their report. The reliability of the manometric studies in this report is excellent, particularly in cases with short and ultrashort aganglionosis for which clinical and radiological examinations were highly inaccurate.--Richard R. Ricketts
ABDOMEN Longitudinal Pancreaticojejunostomy in Chronic Pancreati-
H. W. Scott, W. HI. Neblett, J.A. O'Neill, etal. Ann Surg 199:610-622, (May), 1984.
tis With O n s e t in Childhood.
Recurrent pancreatitis in childhood is uncommon and may be due to cystic fibrosis, hereditary hyperparathyroidism, hyperlipidemia, ductal obstruction secondary to choledochal cyst, gastric duplication, ampullary stenosis, idiopathic fibrosing pancreatitis, and hereditary or familial pancreatitis. Eight patients whose symptoms began in childhood were treated by longitudinal pancreaticojejunostomy; seven of the eight had hereditary pancreatitis. Duration of symptoms ranged from 2 to 36 years (mean 10.5 years). Recurrent epigastric pain and elevated serum amylase were characteristic. The five most recent patients had ERCP and demonstrated dilatation of the pancreatic duct; four patients had stones. Surgical drainage of the obstructed pancreatic duct by longitudinal pancreaticojejunostomy achieved uniformly excellent results with no significant complications. The fo]lowing principles of operative management are suggested: (1) preoperative ERCP should be performed in order to define precisely the ductal anatomy, including existence of proximal obstruction and intraductal stones; (2) the presence of pseudocysts may necessitate distal resection combined with ductal drainage; (3) splenectomy should be avoided if possible; (4) the duct of Wirsung should be identified along the ventral aspect of the pancreas and filleted longitudinally; (5) all calculi should be extracted from the duct whenever possible; and (6) a long side-to-side pancreaticojejunstomy should be constructed, preferably with a retrocolic Roux-en-Y limb of jejunum. Early diagnosis and operation is suggested and supported by the excellent results in terms of relief of pain, elimination of pancreatitis, and preservation of pancreatic function.--Richard Jr. Andrassy Surgical Judgement in the Management of Abdominal Stab
W.C. Lee, J.F. Uddo, and F.C. Nance. Ann Surg 199:549-554, (May), 1984.
Wounds.
Two hundred and nineteen patients with abdominal stab wounds were treated during a 10-year retrospective period under a protocol of selective management. The patients' ages ranged from 9 to 64 years with a mean of 26.6 years. Of the 219 patients, 111 (50.7%) were treated nonoperatively. No complications secondary to conservative treatment were iden-
tiffed (0% morbidity). Eighteen patients observed initially underwent subsequent exploratory laparotomy. The average delay from admission to the time of exploration in this group was 11.2 hours. The negative or unnecessary laparotomy rate was 7.8%. The false-negative examination rate was 5.5%. Overall mortality was 2.3%. Peritoneal lavage was not utilized in the evaluation of the patients. Overall, 194 of 219 patients (88.6%) were correctly diagnosed upon initial evaluation. A total of 17 (7.8%) patients had a negative laparotomy or trivial injuries. This study suggests that selective management of stab wounds of the abdomen may be safely practiced in a smaller community hospital.--Riehard J. Andrassy
GENITOURINARY TRACT Distal Hypospadias Repair. P.F. Nasrallah and H.B. Minott.
J Urol 131:923-930, (May), 1984. The authors report some dissatisfaction with the MAGPI operation for subcoronal hypospadias repair. A procedure for distal hypospadias that incorporates a more vigorous vertical glans incision plus more aggressive distal urethral mobilization is described. Reconstruction has been successful in all 28 patients who have undergone this operation. In the event mobilization and advancement of the urethra with this technique results in tethering of the glans with ventriflexion, the Mustard6 repair is preferred.--George Holcomb, Jr Laparoscopy for Localization of Nonpalpable Testes. D.H.
Lowe, W.A. Brock, and G.W. Kaplan. J Urol 131:728-729, (April), 1984. Laparoscopy was performed on 33 consecutive boys with 36 nonpalpable testes whenever one or both testes were not palpable. It was successful in 29 patients (88%). Patient age ranged from 1 to 17 years with the mean of 3.9 years. Six (18%) were between one and two years of age. Anatomic localization of nonpalpable testes facilitated accurate planning of operative repair, thereby potentially improving the ultimate result. Additionally, the technique rendered exploration unnecessary in patients with the intraabdominal vanishing testis syndrome. No complications were noted as a result of laparoscopy in these patients.--George Holcomb, Jr Urethral Reconstruction in Boys with Classical Bladder Exstrophy, H. Lepor, E. Shapiro, and Robert D. Jeffs. J Urol
131:512-514, (March), 1984. Penile reconstruction in male patients with classical bladder exstrophy includes penile lengthening, release of the dorsal chordee, and reconstruction of the urethra. The authors prefer performance of the first two procedures during the primary bladder closure. Urethroplasty usually follows bladder neck reconstruction. The procedures used in 24 boys with bladder exstrophy for urethral construction between 1975 and 1982 are described. Fistulas requiring surgical revision developed in 21% of these patients. A prior osteotomy was associated with a decreased fistula rate. The definitive assessment of penile reconstruction will be determined when these boys reach sexual maturity.--George Holcomb, Jr
has been shown to induce a remission in a large number of patients with Crohn's disease. Younger patients with Crohn's disease have an increased rate of recurrence, 70% compared with a rate of 30% for those over 40. Ulcerative colitis in childhood presents fewer problems than Crohn's disease because the diagnosis is easier, proctocolectomy is curative, medical treatment is more clearly defined, and growth retardation is much less common. However, children are more likely to have active and more extensive disease than adults, with 90% of children having moderate to severe disease. Of those patients who present with fulminant colitis, only one-third will respond quickly to medical management, but the remission is short-lived and the majority come to surgery sooner rather than later. Almost every child developing toxic megacolon comes to early surgery within 48 to 72 hours. Ultimately, proctocolectomy is curative, although sphincter-saving procedures and anorectal pull-throughs are now showing encouraging results. The prognosis in Crohn's disease is in part determined by the site of the disease, in that children with ileocolic lesions fair worse than children with isolated small bowel disease not involving the duodenum. Also, patients under 20 not only have a high recurrence rate, but also have a significantly higher mortality rate than their older counterparts. The prognosis for ulcerative colitis is probably better than that reported in a large retrospective study from the Mayo Clinic (1918 1965) which found a mortality rate of 20% per decade for those in whom total colitis began in childhood. That study also found that in patients with ulcerative colitis, after the first decade, the risk of developing cancer was 20% per decade.--Richard R. Ricketts
Perianal Disease in Children and Adolescents with Crohn's
Disease. J. Markowitz, F. Daum, H. Aifes, et al. A m J Gastroentol 86:829-833, (May), 1984. One hundred forty-nine children and adolescents with Crohn's disease seen between 1972 and 1982 were reviewed with respect to the presence of perianal disease. Perianal disease was defined as, (a) anal canal lesions consisting of large, edematous skin tags and/or deep anal fissures, (b) fistulas, and (c) abscesses. Seventy-three patients (49%) had perianal disease. Patients with perianal disease had essentially the same incidence of active rectal disease as those without perianal disease (61% v 49%). However, those patients with the most severe perianal disease, fistulas, and abscesses, all had active rectal involvement. The frequency of granulomata in the rectal biopsy was significantly greater in the perianal disease group compared with the nonperianal group (30% v 9%). For patients with only fissures or tags, local care and scrupulous perianal hygiene were adequate treatment. Patients with fistulas or abscesses often required surgical therapy. Proper recognition of the perianal lesions seen in children with Crohn's disease is of clinical importance since perianal disease has preceded the development of intestinal symptoms in 10% to 25% of patients with Crohn's disease. Children with "hemorrhoids" should be suspected of having Crohn's disease and, therefore, should be evaluated appropriately.--Richard R. Ricketts
885 Clinical Impact of Colectomy and Ileorectal Anastomosis in the Management of Crohn's Disease, N.S. Ambrose,
M.R.B. Keighley, J. Alexander-Williams, et al. Gut 25:223227, (March), 1984. Sixty-three patients with Crohn's colitis were treated by colectomy and ileorectal anastomosis between 1951 and 1981. Patients were not considered for this procedure if they had several perianal disease or if the rectum was grossly abnormal. The mean follow-up since colectomy in this group of patients was 9.5 years. The cumulative recurrence rates increased with time and reached 64% at ten years. The cumulative reoperation rates also increased with time and was 48% in ten years. Recurrence developing in the ileum proximal to the anastomosis often was resected and intestinal continuity preserved, whereas anorectal recurrence usually required proctectomy. In spite of the high recurrence rate, this procedure has a useful place in restoring the chronically ill patient to good health and it avoids the morbidity of a permanent stoma, pelvic dissection, and rectal e x c i s i o n . Richard R. Ricketts Diagnosis and Treatment of Hirschsprung's Disease in
Japan. K. Ikeda, and S. Goto. A n n Surg 199:400-405, (April), 1984. A nationwide survey in Japan of 1,628 patients with Hirschsprung's Disease from 135 institutions is reported. Diagnosis was generally made by barium enema, anorectal manometry, and histochemical studies. Anorectal manometry has been used increasingly in combination with barium enema for diagnosis. Forty-eight per cent were diagnosed in the first month of life and 83.4% by the end of the first year. The overall incidence of associated anomalies was 11.1% with Down's syndrome and cardiac anomalies being the most frequent. Almost 80% of patients had aganglionosis limited to the rectum and the sigmoid colon. The incidence of total colonic with or without small bowel aganglionosis was 8.5%. The overall incidence of preoperative enterocolitis was 29.2%. Enterostomy was created in 61.3% with disease to the sigmoid, 95.5% in those with right colon extention, and 97.1% in total colonic with our without small bowel aganglionosis. Retrorectal transanal pull-through as described by Duhamel and modified by Ikeda was the most common definitive operation. The Z-shaped anastomosis described by Ikeda was used in 30.7% of all procedures. Endorectal pull-through was used in 27.6% as the definitive operation. The mortality rate was highest in patients with total calonic aganglionosis. Sepsis was the most frequent cause of death.--Richard J. Andrassy Anorectal Manometry in the Diagnosis of Hirschsprung's Disease--Comparison With Clinical and Radiological Criteria. G.A. Lanfranchi, G. Bazzocchi, S. Federici, et al. A m J
Gastroento179:270-275, (April), 1984. Thirty-four patients, 21 males and 13 females (average age: 4.8 years; range 17 days through 16 years) in whom the diagnosis of Hirschsprung's disease was suspected, were analyzed with respect to the reliability of clinical symptoms, radiological parameters, and anorectal manometry in arriv-
886
ABSTRACTS
ing at the correct diagnosis. Nineteen patients had histologically proven Hirschsprung's disease while the remaining 15 cases had idiopathic constipation. In this study, anorectal manometry correctly diagnosed all patients. The manometric parameters that were found diagnostic of Hirschsprung's disease included (a) an absent rectoanal inhibitory reflex, (b) a significant lower anal resting pressure, (c) a lower pain threshhold, and (d) a decreased frequency of spontaneous rhythmic oscillations. It should be noted, however, that the average age of the patients with aganglionosis was 4.3 years and that only four children less than one year were included in their report. The reliability of the manometric studies in this report is excellent, particularly in cases with short and ultrashort aganglionosis for which clinical and radiological examinations were highly inaccurate.--Richard R. Ricketts
ABDOMEN Longitudinal Pancreaticojejunostomy in Chronic Pancreati-
H. W. Scott, W. HI. Neblett, J.A. O'Neill, etal. Ann Surg 199:610-622, (May), 1984.
tis With O n s e t in Childhood.
Recurrent pancreatitis in childhood is uncommon and may be due to cystic fibrosis, hereditary hyperparathyroidism, hyperlipidemia, ductal obstruction secondary to choledochal cyst, gastric duplication, ampullary stenosis, idiopathic fibrosing pancreatitis, and hereditary or familial pancreatitis. Eight patients whose symptoms began in childhood were treated by longitudinal pancreaticojejunostomy; seven of the eight had hereditary pancreatitis. Duration of symptoms ranged from 2 to 36 years (mean 10.5 years). Recurrent epigastric pain and elevated serum amylase were characteristic. The five most recent patients had ERCP and demonstrated dilatation of the pancreatic duct; four patients had stones. Surgical drainage of the obstructed pancreatic duct by longitudinal pancreaticojejunostomy achieved uniformly excellent results with no significant complications. The fo]lowing principles of operative management are suggested: (1) preoperative ERCP should be performed in order to define precisely the ductal anatomy, including existence of proximal obstruction and intraductal stones; (2) the presence of pseudocysts may necessitate distal resection combined with ductal drainage; (3) splenectomy should be avoided if possible; (4) the duct of Wirsung should be identified along the ventral aspect of the pancreas and filleted longitudinally; (5) all calculi should be extracted from the duct whenever possible; and (6) a long side-to-side pancreaticojejunstomy should be constructed, preferably with a retrocolic Roux-en-Y limb of jejunum. Early diagnosis and operation is suggested and supported by the excellent results in terms of relief of pain, elimination of pancreatitis, and preservation of pancreatic function.--Richard Jr. Andrassy Surgical Judgement in the Management of Abdominal Stab
W.C. Lee, J.F. Uddo, and F.C. Nance. Ann Surg 199:549-554, (May), 1984.
Wounds.
Two hundred and nineteen patients with abdominal stab wounds were treated during a 10-year retrospective period under a protocol of selective management. The patients' ages ranged from 9 to 64 years with a mean of 26.6 years. Of the 219 patients, 111 (50.7%) were treated nonoperatively. No complications secondary to conservative treatment were iden-
tiffed (0% morbidity). Eighteen patients observed initially underwent subsequent exploratory laparotomy. The average delay from admission to the time of exploration in this group was 11.2 hours. The negative or unnecessary laparotomy rate was 7.8%. The false-negative examination rate was 5.5%. Overall mortality was 2.3%. Peritoneal lavage was not utilized in the evaluation of the patients. Overall, 194 of 219 patients (88.6%) were correctly diagnosed upon initial evaluation. A total of 17 (7.8%) patients had a negative laparotomy or trivial injuries. This study suggests that selective management of stab wounds of the abdomen may be safely practiced in a smaller community hospital.--Riehard J. Andrassy
GENITOURINARY TRACT Distal Hypospadias Repair. P.F. Nasrallah and H.B. Minott.
J Urol 131:923-930, (May), 1984. The authors report some dissatisfaction with the MAGPI operation for subcoronal hypospadias repair. A procedure for distal hypospadias that incorporates a more vigorous vertical glans incision plus more aggressive distal urethral mobilization is described. Reconstruction has been successful in all 28 patients who have undergone this operation. In the event mobilization and advancement of the urethra with this technique results in tethering of the glans with ventriflexion, the Mustard6 repair is preferred.--George Holcomb, Jr Laparoscopy for Localization of Nonpalpable Testes. D.H.
Lowe, W.A. Brock, and G.W. Kaplan. J Urol 131:728-729, (April), 1984. Laparoscopy was performed on 33 consecutive boys with 36 nonpalpable testes whenever one or both testes were not palpable. It was successful in 29 patients (88%). Patient age ranged from 1 to 17 years with the mean of 3.9 years. Six (18%) were between one and two years of age. Anatomic localization of nonpalpable testes facilitated accurate planning of operative repair, thereby potentially improving the ultimate result. Additionally, the technique rendered exploration unnecessary in patients with the intraabdominal vanishing testis syndrome. No complications were noted as a result of laparoscopy in these patients.--George Holcomb, Jr Urethral Reconstruction in Boys with Classical Bladder Exstrophy, H. Lepor, E. Shapiro, and Robert D. Jeffs. J Urol
131:512-514, (March), 1984. Penile reconstruction in male patients with classical bladder exstrophy includes penile lengthening, release of the dorsal chordee, and reconstruction of the urethra. The authors prefer performance of the first two procedures during the primary bladder closure. Urethroplasty usually follows bladder neck reconstruction. The procedures used in 24 boys with bladder exstrophy for urethral construction between 1975 and 1982 are described. Fistulas requiring surgical revision developed in 21% of these patients. A prior osteotomy was associated with a decreased fistula rate. The definitive assessment of penile reconstruction will be determined when these boys reach sexual maturity.--George Holcomb, Jr