The prognosis and treatment of total colonic aganglionosis (TCA) vary according to the presence a... more The prognosis and treatment of total colonic aganglionosis (TCA) vary according to the presence and extent of small bowel involvement. Medical records of TCA patients treated in Helsinki University Children's Hospital during 1984-2013 (n=21) were reviewed. The aganglionosis extended up to cecum or distal ileum (n=12), mid small bowel (n=4), or duodenojejunal flexure (n=5). Patients underwent resection of distal aganglionic bowel with ileoanal (IAA) or jejunoanal anastomosis (JAA) with (n=9) or without J-pouch (n=5), Lester-Martin pull-through (n=1), or were left with an end-jejunostomy (n=6). Further procedures included autologous intestinal reconstruction (n=3) and ITx/listing for ITx (n=2). Compared to distal ileum aganglionosis, patients with more proximal disease required parenteral nutrition (PN) more often (100% vs. 25%) and weaned off PN less frequently (p=0.001). At last follow-up 6.5 (interquartile range 2.5-14.5) years postoperatively, all patients with distal ileum aganglionosis were off PN and alive compared to 78% on PN (p<0.001) and 67% alive (p=0.063) of those with more proximal disease. All had normal plasma bilirubin and patients with preserved intestinal continuity (n=13) were continent. Outcomes following restorative proctocolectomy for aganglionosis extending up to mid small bowel are promising, whereas long-term outlook in proximal small intestinal disease is dismal without ITx.
The objective of the study is to define the population-based bowel functional outcomes and entero... more The objective of the study is to define the population-based bowel functional outcomes and enterocolitis following transanal endorectal pull-through (TEPT) in patients with Hirschsprung disease (HD) treated at our institution between 1986 and 2011. 146 consecutive patients who had undergone primary surgical treatment for HD were included. The median follow-up time was 15 (3-33) years. The clinical details and prevalence of enterocolitis were evaluated in all patients, and bowel function in patients >3years of age with functional intestino-anal continuity. No patients were lost to follow-up. Overall survival was 98%. The level of disease was rectosigmoid in 83%, long segment in 7%, total colonic in 4%, and extending up to the small bowel in 6%. 29% had an associated syndrome. 22% had a preoperative stoma. Operations included TEPT (89%), proctocolectomy with ileoanal anastomosis in 9%, and 3% had a permanent endostomy owing to small intestinal aganglionosis. One patient underwent i...
We assessed the occurrence and outcome of major reoperations following repair of esophageal atres... more We assessed the occurrence and outcome of major reoperations following repair of esophageal atresia with or without tracheoesophageal fistula (TOF). Major outcome measures were survival, preservation of native esophagus, and long-term esophageal function. Hospital charts of 258 consecutive patients treated for esophageal atresia from 1980 to 2013 were reviewed. Forty-two (16%) patients required a total of 57 reoperations after primary repair (n=37) or esophageal reconstruction (n=5). The indications were anastomotic leakage (n=17), anastomotic rupture after endoscopic dilatation (n=5), recurrent tracheoesophageal fistula (TOF) (n=12), undiagnosed proximal TOF (n=3), recalcitrant anastomotic stricture (n=11, primary anastomosis 9, reconstruction 2), undetected proximal fistula (n=3), and inadvertently perforated jejunal graft (n=1). Anastomotic leakage and rupture after dilatation were treated with rethoracotomy and suture and recurrent or undetected TOF by open repair. Strictures no...
Anorectal malformations are common anomalies observed in neonates. Survival of these babies is cu... more Anorectal malformations are common anomalies observed in neonates. Survival of these babies is currently achieved in most cases and improvements in operative technique, patient care, and better follow-up have led to improved functional results. A new, simplified classification system (Krickenbeck classification) and method of functional assessment has led to an improved understanding of these anomalies and has allowed for a better comparison of outcomes. Following successful anatomical repair and appropriate programs of bowel care, socially acceptable continence can be achieved in a majority of patients, especially those with an intact sacrum.
This review addresses the range of operations suggested to be of contemporary value in the trea... more This review addresses the range of operations suggested to be of contemporary value in the treatment of constipation with critical evaluation of efficacy data, complications, patient selection, controversies and areas for future research.
Fecal continence and quality of life were evaluated by a questionnaire completed by 33 patients (... more Fecal continence and quality of life were evaluated by a questionnaire completed by 33 patients (mean age, 35 years; 25 males, 8 females) who had undergone anorectal reconstruction for a high or intermediate anorectal anomaly between 1946 and 1962. The malformation was classified as high in 30 cases and as intermediate in three. The primary reconstruction was abdominoperineal in 23 patients and direct perineal in 10. Ten patients had undergone secondary sphincter reconstructions. Fecal-continence was assessed by the scoring system described by Holschneider. Thirty-five healthy people with a similar age and sex distribution were used as controls. Only 6 patients (18%), including all three with an intermediate anomaly, had good fecal continence. None had completely normal bowel habits. Eighteen patients (54%) had a fair continence outcome. Seven patients (21%) had complete fecal incontinence. A permanent colostomy had been performed in two patients. Urinary incontinence was reported by 11 patients (33%). Ten patients (30%) had difficulties with sexual functions. Social problems related to defective continence were found in 28 patients (85%). All controls had good fecal continence; 80% had completely normal bowel habits. The authors conclude that most adults with high anorectal malformations who have undergone abdominoperineal or direct perineal repair suffer from severely defective fecal continence and have poor quality of life.
Constipation is a major complication in patients who have undergone posterior sagittal anorectopl... more Constipation is a major complication in patients who have undergone posterior sagittal anorectoplasty (PSARP) operation for a high anorectal malformation. Overflow incontinence is the main cause of fecal soiling in these patients. The aim of this study was to outline the natural history of constipation in patients with high anorectal malformations and relate this to the functional outcome at the end of the patient's growth period. The study group consisted of 22 pubertal or postpubertal patients (median age 15; range, 13 to 25) with high or intermediate anorectal malformations repaired by PSARP procedure. The patients have been followed-up since birth. Constipation was defined as a need to use medical treatment or diet to ensure bowel emptying. Continence was classified as follows: grade 1, no soiling in any circumstances; grade 2, staining less than once a week, no fecal accidents; grade 3, staining more than once a week, no fecal accidents; grade 4, daily soiling or accidents, need for regular enemas, or the antegrade colonic enema procedure. All patients underwent anorectal manometry and magnetic resonance imaging of the spine and spinal cord. At the time of the study 2 (9%) of the 22 study group patients had constipation, but 15 (68%) had been constipated before puberty. Eleven patients (50%) were fully continent (grade 1) without constipation. Six of those had a history of constipation associated soiling. Three patients (14%) had occasional staining (grade 2) and no constipation. Two of them had been constipated with significant soiling before the onset of puberty. In the 5 (22%) patients with frequent staining (grade 3) the degree of soiling had decreased after the disappearance of constipation. Two of the 3 patients with poor outcome (grade 4) require regular enemas for recalcitrant constipation. Spinal cord anomalies were detected in 4 and abnormal sacrum in 15 patients. Of the anorectal manometric parameters, only the force of voluntary sphincter squeeze correlated with the functional result. In the majority of patients who underwent PSARP procedure for high anorectal malformation, constipation disappears at adolescence, and this is associated with improved fecal continence outcome.
The choice of ileo-anal reconstruction method in children undergoing proctocolectomy remains cont... more The choice of ileo-anal reconstruction method in children undergoing proctocolectomy remains controversial. Although in adults ileo-anal pouch reconstruction has gained overall acceptance, many paediatric surgeons still advocate straight ileo-anal pull-through. The aim of this study was to assess the outcome and long-term functional results in children who have undergone proctocolectomy and ileo-anal anastomosis (IAA) with a J-pouch. Medical records of 40 consecutive children who had proctocolectomy and J-pouch IAA between 1991 and 1999 were reviewed for early and late complications, fecal frequency, day- and night-time continence, and pouchitis. The indication for surgery was ulcerative colitis (UC) in 29 (median age at operation, 13 years; range, 9 to 16), Hirschsprung's disease (HD) in 10 (median age at operation, 1.5 years; range, 1 month to 5 years), and familial adenomatous polyposis (FAP) in 1 (age at operation, 6 years). Six of the HD patients had primary pull-through for total colonic aganglionosis and 4 a redo operation for failed primary reconstruction of long segment aganglionosis. There were no fatalities. Early complications (wound infection, early bowel obstruction, prolonged fever) occurred in 12 of 29 (41%) and late complications (bowel obstruction 9, pouch fistula 2) in 11 of 29 (38%) of the UC patients. Overall, 16 of 29 (53%) of the UC patients had complications. All patients with early complications were on systemic steroids at the time of the operation. Pouchitis occurred in 30% of the patients. None of the pouches had to be removed. At last follow-up all patients were continent during the day, 2 patients used protective pads during the night because of occasional staining. The median bowel frequency per 24 hours was 4 (range, 2 to 7); only 2 patients (7%) had to empty their bowel during the night. One (10%) of the HD patients had wound infection, and 3 had episodes of postoperative enterocolitis. Pouchitis-type symptoms have not occurred in HD patients. The median bowel frequency for 24 hours was 3 (range, 2 to 5). None of the HD patients needs to evacuate during the night. The 4 HD patients who are older than 3 years of age are continent. J-pouch IAA is a feasible method of reconstruction in children requiring proctocolectomy. Major complication are common but occur mainly in immunosuppressed patients suffering from UC. Despite high incidence of complications, long-term functional results in terms of continence and bowel frequency are excellent and ensure good quality of life in the great majority of patients.
Fecal continence and quality of life were evaluated by a questionnaire in 83 adult patients (mean... more Fecal continence and quality of life were evaluated by a questionnaire in 83 adult patients (mean age, 35 years; 53 women, 30 men) who underwent surgery for a low anorectal anomaly between 1947 and 1963. Fecal continence was assessed by a score described by Holschneider. Seventy-eight healthy people with similar age and sex distributions were used as controls. All controls had good fecal continence, 76% with completely normal bowel function. The aberrations in anal function found in 24% of the controls were minor, such as constipation or occasional slight smearing. Only 60% of the patients who had a low anorectal anomaly had good continence and completely normal bowel function was observed in 15%. Male patients had a slightly better outcome than females. Social problems related to deficient fecal control were reported by 39% of the patients. In addition, 13% of the patients had difficulties in sexual functions. Other health problems were reported by 52% of the patients. Social or sexual problems associated with anal function were not reported by the control population; 6% of them had other health problems. The present controlled study shows that at the adult age, a significant proportion of patients with low anorectal anomalies suffer from deficient fecal control and a diminished quality of life.
The bowel function of 46 patients who had undergone internal sphincter-saving posterior sagittal ... more The bowel function of 46 patients who had undergone internal sphincter-saving posterior sagittal anorectoplasty (PSARP) was evaluated by a questionnaire 3 to 10 years after the closure of the colostomy. The bowel function was assessed by a multivariate scoring method. All patients had also been followed up clinically and manometrically. Seventy healthy children with a similar age and sex distribution were used as controls. Sixteen (35%) patients had bowel function scores that were within the range of the scores of healthy children (mean score of healthy children +/- SD). Another 16 (35%) patients with intermediate scores had a clinically good continence. Fourteen (30%) patients with low scores had a clinically fair or poor functional result; 3 of them required enemas because of severe constipation and 11 used protective aids or had to change underwear frequently because of daily soiling. There was a correlation between a good continence outcome and the presence of a functional internal sphincter and high anorectal resting pressure. Absence of a functional internal sphincter, severe sacral anomalies, and constipation were associated with poor functional results. It is concluded that normal bowel function after internal sphincter-saving PSARP may be expected in a significant proportion of children with high or intermediate anorectal anomalies.
The prognosis and treatment of total colonic aganglionosis (TCA) vary according to the presence a... more The prognosis and treatment of total colonic aganglionosis (TCA) vary according to the presence and extent of small bowel involvement. Medical records of TCA patients treated in Helsinki University Children's Hospital during 1984-2013 (n=21) were reviewed. The aganglionosis extended up to cecum or distal ileum (n=12), mid small bowel (n=4), or duodenojejunal flexure (n=5). Patients underwent resection of distal aganglionic bowel with ileoanal (IAA) or jejunoanal anastomosis (JAA) with (n=9) or without J-pouch (n=5), Lester-Martin pull-through (n=1), or were left with an end-jejunostomy (n=6). Further procedures included autologous intestinal reconstruction (n=3) and ITx/listing for ITx (n=2). Compared to distal ileum aganglionosis, patients with more proximal disease required parenteral nutrition (PN) more often (100% vs. 25%) and weaned off PN less frequently (p=0.001). At last follow-up 6.5 (interquartile range 2.5-14.5) years postoperatively, all patients with distal ileum aganglionosis were off PN and alive compared to 78% on PN (p<0.001) and 67% alive (p=0.063) of those with more proximal disease. All had normal plasma bilirubin and patients with preserved intestinal continuity (n=13) were continent. Outcomes following restorative proctocolectomy for aganglionosis extending up to mid small bowel are promising, whereas long-term outlook in proximal small intestinal disease is dismal without ITx.
The objective of the study is to define the population-based bowel functional outcomes and entero... more The objective of the study is to define the population-based bowel functional outcomes and enterocolitis following transanal endorectal pull-through (TEPT) in patients with Hirschsprung disease (HD) treated at our institution between 1986 and 2011. 146 consecutive patients who had undergone primary surgical treatment for HD were included. The median follow-up time was 15 (3-33) years. The clinical details and prevalence of enterocolitis were evaluated in all patients, and bowel function in patients >3years of age with functional intestino-anal continuity. No patients were lost to follow-up. Overall survival was 98%. The level of disease was rectosigmoid in 83%, long segment in 7%, total colonic in 4%, and extending up to the small bowel in 6%. 29% had an associated syndrome. 22% had a preoperative stoma. Operations included TEPT (89%), proctocolectomy with ileoanal anastomosis in 9%, and 3% had a permanent endostomy owing to small intestinal aganglionosis. One patient underwent i...
We assessed the occurrence and outcome of major reoperations following repair of esophageal atres... more We assessed the occurrence and outcome of major reoperations following repair of esophageal atresia with or without tracheoesophageal fistula (TOF). Major outcome measures were survival, preservation of native esophagus, and long-term esophageal function. Hospital charts of 258 consecutive patients treated for esophageal atresia from 1980 to 2013 were reviewed. Forty-two (16%) patients required a total of 57 reoperations after primary repair (n=37) or esophageal reconstruction (n=5). The indications were anastomotic leakage (n=17), anastomotic rupture after endoscopic dilatation (n=5), recurrent tracheoesophageal fistula (TOF) (n=12), undiagnosed proximal TOF (n=3), recalcitrant anastomotic stricture (n=11, primary anastomosis 9, reconstruction 2), undetected proximal fistula (n=3), and inadvertently perforated jejunal graft (n=1). Anastomotic leakage and rupture after dilatation were treated with rethoracotomy and suture and recurrent or undetected TOF by open repair. Strictures no...
Anorectal malformations are common anomalies observed in neonates. Survival of these babies is cu... more Anorectal malformations are common anomalies observed in neonates. Survival of these babies is currently achieved in most cases and improvements in operative technique, patient care, and better follow-up have led to improved functional results. A new, simplified classification system (Krickenbeck classification) and method of functional assessment has led to an improved understanding of these anomalies and has allowed for a better comparison of outcomes. Following successful anatomical repair and appropriate programs of bowel care, socially acceptable continence can be achieved in a majority of patients, especially those with an intact sacrum.
This review addresses the range of operations suggested to be of contemporary value in the trea... more This review addresses the range of operations suggested to be of contemporary value in the treatment of constipation with critical evaluation of efficacy data, complications, patient selection, controversies and areas for future research.
Fecal continence and quality of life were evaluated by a questionnaire completed by 33 patients (... more Fecal continence and quality of life were evaluated by a questionnaire completed by 33 patients (mean age, 35 years; 25 males, 8 females) who had undergone anorectal reconstruction for a high or intermediate anorectal anomaly between 1946 and 1962. The malformation was classified as high in 30 cases and as intermediate in three. The primary reconstruction was abdominoperineal in 23 patients and direct perineal in 10. Ten patients had undergone secondary sphincter reconstructions. Fecal-continence was assessed by the scoring system described by Holschneider. Thirty-five healthy people with a similar age and sex distribution were used as controls. Only 6 patients (18%), including all three with an intermediate anomaly, had good fecal continence. None had completely normal bowel habits. Eighteen patients (54%) had a fair continence outcome. Seven patients (21%) had complete fecal incontinence. A permanent colostomy had been performed in two patients. Urinary incontinence was reported by 11 patients (33%). Ten patients (30%) had difficulties with sexual functions. Social problems related to defective continence were found in 28 patients (85%). All controls had good fecal continence; 80% had completely normal bowel habits. The authors conclude that most adults with high anorectal malformations who have undergone abdominoperineal or direct perineal repair suffer from severely defective fecal continence and have poor quality of life.
Constipation is a major complication in patients who have undergone posterior sagittal anorectopl... more Constipation is a major complication in patients who have undergone posterior sagittal anorectoplasty (PSARP) operation for a high anorectal malformation. Overflow incontinence is the main cause of fecal soiling in these patients. The aim of this study was to outline the natural history of constipation in patients with high anorectal malformations and relate this to the functional outcome at the end of the patient's growth period. The study group consisted of 22 pubertal or postpubertal patients (median age 15; range, 13 to 25) with high or intermediate anorectal malformations repaired by PSARP procedure. The patients have been followed-up since birth. Constipation was defined as a need to use medical treatment or diet to ensure bowel emptying. Continence was classified as follows: grade 1, no soiling in any circumstances; grade 2, staining less than once a week, no fecal accidents; grade 3, staining more than once a week, no fecal accidents; grade 4, daily soiling or accidents, need for regular enemas, or the antegrade colonic enema procedure. All patients underwent anorectal manometry and magnetic resonance imaging of the spine and spinal cord. At the time of the study 2 (9%) of the 22 study group patients had constipation, but 15 (68%) had been constipated before puberty. Eleven patients (50%) were fully continent (grade 1) without constipation. Six of those had a history of constipation associated soiling. Three patients (14%) had occasional staining (grade 2) and no constipation. Two of them had been constipated with significant soiling before the onset of puberty. In the 5 (22%) patients with frequent staining (grade 3) the degree of soiling had decreased after the disappearance of constipation. Two of the 3 patients with poor outcome (grade 4) require regular enemas for recalcitrant constipation. Spinal cord anomalies were detected in 4 and abnormal sacrum in 15 patients. Of the anorectal manometric parameters, only the force of voluntary sphincter squeeze correlated with the functional result. In the majority of patients who underwent PSARP procedure for high anorectal malformation, constipation disappears at adolescence, and this is associated with improved fecal continence outcome.
The choice of ileo-anal reconstruction method in children undergoing proctocolectomy remains cont... more The choice of ileo-anal reconstruction method in children undergoing proctocolectomy remains controversial. Although in adults ileo-anal pouch reconstruction has gained overall acceptance, many paediatric surgeons still advocate straight ileo-anal pull-through. The aim of this study was to assess the outcome and long-term functional results in children who have undergone proctocolectomy and ileo-anal anastomosis (IAA) with a J-pouch. Medical records of 40 consecutive children who had proctocolectomy and J-pouch IAA between 1991 and 1999 were reviewed for early and late complications, fecal frequency, day- and night-time continence, and pouchitis. The indication for surgery was ulcerative colitis (UC) in 29 (median age at operation, 13 years; range, 9 to 16), Hirschsprung's disease (HD) in 10 (median age at operation, 1.5 years; range, 1 month to 5 years), and familial adenomatous polyposis (FAP) in 1 (age at operation, 6 years). Six of the HD patients had primary pull-through for total colonic aganglionosis and 4 a redo operation for failed primary reconstruction of long segment aganglionosis. There were no fatalities. Early complications (wound infection, early bowel obstruction, prolonged fever) occurred in 12 of 29 (41%) and late complications (bowel obstruction 9, pouch fistula 2) in 11 of 29 (38%) of the UC patients. Overall, 16 of 29 (53%) of the UC patients had complications. All patients with early complications were on systemic steroids at the time of the operation. Pouchitis occurred in 30% of the patients. None of the pouches had to be removed. At last follow-up all patients were continent during the day, 2 patients used protective pads during the night because of occasional staining. The median bowel frequency per 24 hours was 4 (range, 2 to 7); only 2 patients (7%) had to empty their bowel during the night. One (10%) of the HD patients had wound infection, and 3 had episodes of postoperative enterocolitis. Pouchitis-type symptoms have not occurred in HD patients. The median bowel frequency for 24 hours was 3 (range, 2 to 5). None of the HD patients needs to evacuate during the night. The 4 HD patients who are older than 3 years of age are continent. J-pouch IAA is a feasible method of reconstruction in children requiring proctocolectomy. Major complication are common but occur mainly in immunosuppressed patients suffering from UC. Despite high incidence of complications, long-term functional results in terms of continence and bowel frequency are excellent and ensure good quality of life in the great majority of patients.
Fecal continence and quality of life were evaluated by a questionnaire in 83 adult patients (mean... more Fecal continence and quality of life were evaluated by a questionnaire in 83 adult patients (mean age, 35 years; 53 women, 30 men) who underwent surgery for a low anorectal anomaly between 1947 and 1963. Fecal continence was assessed by a score described by Holschneider. Seventy-eight healthy people with similar age and sex distributions were used as controls. All controls had good fecal continence, 76% with completely normal bowel function. The aberrations in anal function found in 24% of the controls were minor, such as constipation or occasional slight smearing. Only 60% of the patients who had a low anorectal anomaly had good continence and completely normal bowel function was observed in 15%. Male patients had a slightly better outcome than females. Social problems related to deficient fecal control were reported by 39% of the patients. In addition, 13% of the patients had difficulties in sexual functions. Other health problems were reported by 52% of the patients. Social or sexual problems associated with anal function were not reported by the control population; 6% of them had other health problems. The present controlled study shows that at the adult age, a significant proportion of patients with low anorectal anomalies suffer from deficient fecal control and a diminished quality of life.
The bowel function of 46 patients who had undergone internal sphincter-saving posterior sagittal ... more The bowel function of 46 patients who had undergone internal sphincter-saving posterior sagittal anorectoplasty (PSARP) was evaluated by a questionnaire 3 to 10 years after the closure of the colostomy. The bowel function was assessed by a multivariate scoring method. All patients had also been followed up clinically and manometrically. Seventy healthy children with a similar age and sex distribution were used as controls. Sixteen (35%) patients had bowel function scores that were within the range of the scores of healthy children (mean score of healthy children +/- SD). Another 16 (35%) patients with intermediate scores had a clinically good continence. Fourteen (30%) patients with low scores had a clinically fair or poor functional result; 3 of them required enemas because of severe constipation and 11 used protective aids or had to change underwear frequently because of daily soiling. There was a correlation between a good continence outcome and the presence of a functional internal sphincter and high anorectal resting pressure. Absence of a functional internal sphincter, severe sacral anomalies, and constipation were associated with poor functional results. It is concluded that normal bowel function after internal sphincter-saving PSARP may be expected in a significant proportion of children with high or intermediate anorectal anomalies.
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