Urinary incontinence - an unusual indication for early vaginoplasty in late presenting congenital adrenal hyperplasia.

BACKGROUND: Congenital adrenal hyperplasia (CAH) is an autosomal recessive condition leading to deficient cortisol with an incidence of 1/16,000. Patients with CAH typically present early with ambiguous genitalia or as an emergency with adrenal crisis. CASE: We report an atypical late presentation of a 4-year-old girl with pubertal-like symptoms and urinary incontinence, due to a persistent urogenital sinus (UGS). An early vaginoplasty was successfully performed allowing the patient to achieve continence. CONCLUSION: Literature describing the symptoms of CAH with UGS is scarce. The case is unusual in demonstrating pubertal-like symptoms and urinary incontinence due to the late presentation of a persistent UGS, highlighting the need for an open mind in assessment of children with urinary incontinence. Timing of surgery is controversial, and cases need to be considered on an individual basis.

Infections in gastroschisis: organisms and factors.

This study aimed to define the incidence, causative organisms and predisposing factors leading to infection related morbidity in newborns with gastroschisis. All gastroschisis patients admitted over the 5-year period (1999-2004) were retrospectively reviewed. Surveillance samples, wound, blood, urine and fecal cultures were analyzed. Duration of total parenteral nutrition, antibiotic therapy, feeding regimes and demographic data were also analyzed. Multiple logistic regression was employed using the SPSS system and p < 0.05 was considered as significant. Seventy-two neonates were identified with 53% having abnormal gut carriage mostly due to Enterobacter and Klebsiella. Wound infection occurred in 20% of cases. Abnormal gut carriage predisposed to the development of wound infection. Line sepsis occurred in 21% of neonates. Endogenous coagulase negative Staphylococcus caused 74% of septic episodes. There was no correlation between abnormal gut carriage and the development of line sepsis. Overall survival was 96%. The cause of infections in gastroschisis patients appears to be multifactorial. A multidisciplinary team can play an important role in reducing the incidence of infections. Strict aseptic protocols and auditing practice can be the invaluable tools in decreasing morbidity rates.

Surgical strategies in the management of ecthyma gangrenosum in paediatric oncology patients.

Clinical presentation and microbiology profiles of neutropenic paediatric oncology patients presenting with ecthyma gangrenosum (EG) were studied. Surgical strategies deployed for these critically ill children are reported. Between 1994 and 2005, all children with EG were identified. Case notes were reviewed. Hospital course and long-term outcome were documented. Ten patients were identified. Eight had acute lymphoblastic leukaemia, one child had acute myeloid leukaemia and another had rhabdomyosarcoma. Lesions occurred in the perineal region (n = 5), buttocks (n = 2), thigh (n = 2) and the face (n = 1). Seven children had positive blood cultures for Pseudomonas aeruginosa. Surgery included (1) radical debridement, and (2) debridement with covering colostomy for four of those with perianal lesions. Ecthyma gangrenosum is a rapidly spreading and potentially lethal condition. Paediatric oncology patients with neutropenia are at a high risk. Surgical excision is crucial for progressive lesions to prevent mortality.

Comparison of functional outcomes of Duhamel and transanal endorectal coloanal anastomosis for Hirschsprung's disease.

PURPOSE: The aim of this study was to determine the morbidity and medium-term functional outcome of the Duhamel operation and laparotomy and transanal endorectal coloanal anastomosis (TECA) for Hirschsprung's disease (HSCR). METHODS: The study populations were 34 consecutive children who underwent the Duhamel operation (or Lester Martin modification) and 37 who had the TECA. Demographic details were obtained by case note review, and functional outcome was determined by a combination of outpatient interview, questionnaire, and telephone enquiry. RESULTS: There was no difference between the groups with respect to age, gender, and length of aganglionic segment. Seventy percent presented as neonates (Duhamel, 24 of 34; TECA, 26 of 37). A single-stage primary pull-through was performed in 17 of 37 children in the TECA group, and in 1 of 34 from the Duhamel group. There was a single perioperative death in the Duhamel group and an unrelated, late death in the TECA group. Postoperative enterocolitis was seen in 13 of 37 TECA children and in a single child from the Duhamel group. A stricture of the pull-through segment was seen in 7 of 37 children after TECA and required temporary diversion in 2 of 9. Late division of a rectal spur was required in 6 of 33 Duhamel children. Requirement for late myectomy was the same in both groups (Duhamel 3 of 33, TECA 4 of 37). Complications requiring stoma formation occurred in 5 of 37 after TECA and 2 of 33 after the Duhamel operation. Two children from the TECA group and 1 from the Duhamel group remain diverted. One child from each group required a re-pull-through procedure. Two patients were lost to follow-up in the TECA group, leaving 34 children in this group and 33 in the Duhamel group in whom functional outcome could be assessed. Functional outcome was similar in the 2 groups. CONCLUSIONS: TECA and Duhamel procedures have similar medium-term functional outcomes. TECA has a high incidence of postoperative enterocolitis and transient stricture formation but is suitable for single-stage neonatal treatment of HSCR.

Colonic transit time--what is normal?

BACKGROUND: Constipation is a common problem in childhood, and various radiologic methods have been advocated for investigation. Colonic transit time (CTT) has been used in adults to investigate colonic motility, but few studies evaluate this method in children. Data on CTT in the normal paediatric population are scarce. METHODS: The colonic transit time was measured in 22 healthy children (median age, 10 years; range, 4 to 15 years) by Abrahamsson's method. Children took bolus ingestions of radiopaque markers on 6 consecutive days, and on day 7 a single abdominal x-ray was performed. This was evaluated for total and segmental colonic transit time. RESULTS: The mean total CTT was 40 hours, and the upper limit of normal (95th percentile) was 84 hours. The upper limit of normal for segmental transit time was as follows: 14 hours for the ascending, 33 hours for the transverse, 21 hours for the descending, and 41 hours for the rectosigmoid colon. CONCLUSIONS: CTT provides an objective measure to assess childhood constipation. To date, 6 studies using 5 different methods have been published reporting values for healthy children. Comparing these, Abrahamson's method has low radiation exposure and is well tolerated. This study contributes additional normal values in children.

After the honeymoon--medium-term outcome of antegrade continence enema procedure.

PURPOSE: The aim of this study was to determine medium-term outcomes of the antegrade continence enema (ACE) procedure. METHODS: A retrospective casenote review plus telephone questionnaire was conducted. The study was performed at a regional paediatric surgical centre. The subjects were consecutive children undergoing the ACE procedure over a 5 year period. Main outcome measures were use of the ACE; reversal rates; complications, ease of use, effectiveness, and satisfaction scores. Data are expressed as median (range). RESULTS: Thirty-two (52%) of 62 children undergoing the ACE procedure were girls. The age at the time of operation was 11.5 (3.8 to 17.6) years. Underlying diagnoses included spina bifida (n = 31), anorectal malformations (n = 15), slow-transit constipation (n = 9), Hirschsprung's disease (n = 2), sacral agenesis (n = 2), and trauma/tumour (n = 2). Median follow-up was 5.4 (3.25 to 8.25) years. Eleven of 62 (18%) children were no longer using the ACE (n = 5) or had it surgically reversed (n = 6; 14.1 +/- 9.3 months postprocedure). Reasons for disuse/reversal were lack of effectiveness (n = 4), complications (n = 2), noncompliance (n = 3), independent continence (n = 1), and pain (n = 1). Five (8%) children currently have a colostomy. Gender (P =.31; Fisher's Exact), age (Pearson), and underlying diagnoses (P =.07, Chi2) were not predictors of failure. Overall, stomal stenosis was the most common complication, affecting 26 of 62 (41%) children. Of 32 questionnaire respondents to linear scores, ease of use was rated as 2 (0 to 8, 0, very easy; 10, very difficult), discomfort on use as 3 (0 to 9; 0, no pain; 10, very painful), overall satisfaction as 9 (0 to 10; 0, completely dissatisfied; 10, completely satisfied). Eighty-four percent were completely continent or had soiling less than once a month. There was a significant correlation between the level of continence and satisfaction with the procedure (P =.04; Pearson). CONCLUSIONS: The ACE procedure offers significant benefits to some children with incontinence or intractable constipation. However, it is not universally successful, and other continence promoting strategies may need to be considered.

Double blind randomised controlled trial of topical glyceryl trinitrate in anal fissure.

AIMS: To determine the effectiveness and safety of topical glyceryl trinitrate (GTN) in the management of acute anal fissure in children. METHODS: Individual children were randomised to receive GTN paste or placebo for six weeks in addition to oral senna and lactulose. Patients took laxatives alone for a further 10 weeks. Each week a research nurse telephoned families to assess pain scores and give advice. Main outcome measures were validated standardised pain scores and time to painless defaecation. RESULTS: Forty subjects were recruited from 46 eligible children; 31 children completed the trial (13 in the GTN group and 18 in the placebo group). No differences in the proportion of those achieving pain free defaecation with relation to time were seen between the two groups. Similarly, there were no significant differences in pain scores between the two groups over the 16 week study period. However, in both groups pain scores had decreased significantly. There were no differences in the incidence of rectal bleeding, faecal soiling, presence of visible fissure, skin tag, or faecal loading at outpatient review at the time of recruitment, or at 6 weeks and 16 weeks. No serious adverse effects were observed. CONCLUSIONS: This study suggests that 0.2% GTN paste is ineffective in the treatment of acute anal fissures in childhood. However the overall fissure healing rate is high (84%) with associated reduction in pain scores, suggesting that a nurse based treatment programme can achieve a high rate of fissure healing.

Phenotypic presentation and outcome of esophageal atresia in the era of the Spitz classification.

PURPOSE: The aim of this study was to investigate contemporary patterns of presentation and outcome in newborns with esophageal atresia with or without tracheoesophageal fistula (OA) using modern prognostic criteria to appraise survival. METHODS: Over a 12-year period (1986 through 1997), 134 patients with OA were admitted to a single institution. Patient demographics, the presence of cardiac defects, other associated abnormalities (VACTERL), surgical intervention, and patient outcome were recorded. To identify and evaluate changes in the pattern of clinical presentation, frequency of associated anomalies and outcome, patients were analyzed during 4 consecutive time periods, 1986 to 1988, 1989 to 1991, 1992 to 1994, and 1995 to 1997. RESULTS: A primary repair or delayed primary repair was performed in 113 (84%) patients, with a staged procedure in 19 (14%). Two babies with trisomy 18 did not undergo surgery. Thirty-eight newborns (28%) had a major cardiac malformation (excluding patent ductus arteriosus, unless needing ligation), and 25 (19%) had recognized VACTERL associations. There was a significant increase in the proportion of infants with major cardiac defects diagnosed over the study period, 5 of 34 patients between 1986 and 1988 to 19 of 41 patients between 1995 and 1997 (chi(2) test, P <.001), but the incidence of VACTERL associations remained unchanged. Overall survival rate was 86% in those who underwent surgery. The relative risk of mortality in patients with major cardiac disease and VACTERL associations was 3.47 (95% CI; 1.51 to 7.96) and 2.54 (95% CI; 1.14 to 4.86), respectively. Birth weight was significantly higher in infants who survived (2.68 kg) compared with those who died (2.16 kg, P =.003). Thirty percent of infants with more than one system abnormality died compared with 8% of infants with 1, system abnormality (P =.004). CONCLUSIONS: This study has found a significant increase in the frequency of cardiac abnormalities encountered in a cohort of OA patients during the period under review. Cardiac disease and multiple abnormalities carried a substantial increased risk of mortality. In the era of the Spitz classification, the phenotypic presentation is important to accurately assess caseload severity and prognosis.

Cystic retroperitoneal lymphangioma: treatment by image-guided percutaneous catheter drainage and sclerotherapy.

Retroperitoneal cystic lymphangiomas are extremely rare and the majority are symptomatic during childhood. Although benign, they can compress and infiltrate vital structures. Surgery is curative but is associated with a high complication rate. An alternative treatment strategy is image-guided percutaneous catheter drainage of the lymphangioma followed by sclerotherapy. Resolution of a large retroperitoneal cystic lymphangioma in a 4-year-old child treated by this technique is reported. To our knowledge, this technique has not been previously described in this condition and we believe that it offers significant advantages over surgery.

Umbilical pyloromyotomy--an alternative to laparoscopy?

AIMS: To evaluate the utility of umbilical pyloromyotomy for infantile hypertrophic pyloric stenosis (IHPS) compared to published series promoting laparoscopy. METHODS: Eighty-six babies with IHPS had pyloromyotomy using an umbilical skin fold incision. Operating times, post-operative hospital stay and cosmetic appearance of the umbilical wound were studied. Data extracted from recent series promoting laparoscopy were identified using a MEDLINE search strategy and used for comparative analysis. RESULTS: Mean operating time for umbilical pyloromyotomy was 30 min (range 15-50 min). All patients went home at an average period of 58 h (range 48-72 h) following surgery. The umbilical scar was barely visible in the post-operative period. Laparoscopic pyloromyotomy operating times ranging from 18-41 min (mean overall 30 min) are recorded in the literature. Post-operative stay following laparoscopy has been variable (23-91 h), where reported. In contrast with umbilical pyloromyotomy, "pox" marks observed following port insertions for laparoscopy can give an unsightly scar. CONCLUSIONS: This study has found that umbilical pyloromyotomy can be performed with minimal morbidity and equivalent operating times to laparoscopy. The shorter hospital stay reported in some series promoting laparoscopy must be balanced against local practice influencing hospital stay, the financial implications of offering a laparoscopic service, the skills needed for laparoscopy, and the short learning curve required by paediatric surgical trainees to become proficient at umbilical pyloromyotomy. The cosmesis of the umbilical incision is excellent. These findings suggest that umbilical pyloromyotomy is a reliable alternative to laparoscopy.

Fissurectomy as a treatment for anal fissures in children.

INTRODUCTION: Anal fissures, characterised by painful defecation and rectal bleeding, are common in both children and infants. A significant proportion are resistant to simple laxative therapy, and no simple surgical treatment has been described which does not risk compromising sphincteric function. This study reports the initial experience of fissurectomy as a treatment of this condition. PATIENTS AND METHODS: Over a 36 month period, 37 children with an anal fissure were treated by fissurectomy. There were 14 boys and 23 girls, with an age range of 17 weeks to 12 years. Fissurectomy was performed under general anaesthetic, with additional caudal anaesthesia. Stay sutures were used to avoid the need for an anal retractor, thereby preventing stretching of the internal anal sphincter. Of the 37 operations, 36 (97%) were performed as day cases and all children were discharged on laxative therapy. RESULTS: At review, 6 weeks postoperatively, 30 (81%) were asymptomatic. Six (16%) patients were symptomatic; however, 4 of these had failed to comply with the postoperative laxative regimen. One patient failed follow-up. CONCLUSIONS: Fissurectomy is a successful treatment for anal fissures, when combined with postoperative laxative therapy. As dilatation of the internal anal sphincter is not involved, the risk of iatrogenic faecal incontinence is obviated.

Transanal endorectal coloanal surgery for Hirschsprung's disease: experience in two centers.

BACKGROUND/PURPOSE: Transanal mucosal proctectomy with low coloanal anastomosis has been used widely in the treatment of rectal malignancies, ulcerative colitis, and familial polyposis. The use of this technique for Hirschsprung's disease is a relatively new concept. The aim of this study was to evaluate and compare the results of transanal endorectal coloanal anastomosis (TECA) for Hirschsprung's disease from 2 centers. METHODS: All children who underwent TECA for Hirschsprung's disease at Alder Hey Children's Hospital, Liverpool, England from January 1995 to December 1998 (n = 41) and the Children's Hospital, Helsinki, Finland from June 1988 to December 1998 (n = 95) were evaluated. Patient demographics, age at diagnosis, initial management, length of aganglionic segment, and age at operation were documented. Postoperative complications and functional outcome were analyzed. RESULTS: Patient demographics were similar in the 2 centers. Age at diagnosis was less than 1 month in 71% of children at Liverpool, compared with 53% at Helsinki. Sixteen (39%) patients in Liverpool and 75 (79%) patients in Helsinki underwent primary TECA without colostomy. Postoperative enterocolitis occurred in 14 of 136 patients (10%). An ischemic stricture of the colon was documented in 4 children in the Liverpool series, 2 of whom had TECA as a salvage procedure after a previously failed Duhamel pull-through operation. Frequency of bowel movements, seen in the immediate postoperative period in most patients gradually improved with time from a median of 5 (range, 2 to 12) bowel movements a day at 3 months after TECA to 2 (range, 1 to 6) bowel movements a day at 2 years' follow-up. Assessment of continence was possible in 51 of 136 patients (37%) over the age of 4 years. Thirty-nine children had normal bowel function giving an overall success rate of 76%. CONCLUSIONS: Transanal endorectal coloanal anastomosis is a good technique for treatment of Hirschsprung's disease with few operation-related complications. Based on the data emerging from these 2 centers the functional outcome is highly satisfactory and comparable with other established procedures.

Functional results following the antegrade continence enema procedure.

BACKGROUND: Forty children who underwent the antegrade continence enema (ACE) procedure for faecal soiling were studied to determine factors predictive of outcome. METHODS: There were four patient groups: (1) ambulant with spinal dysraphism (n = 13), (2) wheelchair bound with spinal dysraphism (n = 14), (3) ambulant with miscellaneous disorders (n = 11) and (4) wheelchair bound with miscellaneous disorders (n = 2). Effectiveness of the procedure was assessed using technical evaluation and quality-of-life improvement (QOLI) scores (0-5). Objective assessment included colonic transit time (CTT) and anorectal manometry. Median follow-up was 21 (range 5-37) months. RESULTS: Some 28 of 40 children achieved continence. The procedure was reversed in four of 40 children. Of the other 36 children with a functioning ACE stoma, all reported improvement in quality of life (mean QOLI score 3.5). There were no significant differences in technical evaluation score, QOLI score, CTT, manometry findings or continence between ambulant groups and the wheelchair-bound group with miscellaneous disorders. QOLI score, anorectal squeeze pressure and continence were significantly poorer in those who were wheelchair bound with spinal dysraphism. Absent squeeze pressure was associated with poor outcome. CONCLUSION: Wheelchair-bound children with spinal neuropathy have a poorer outcome following the ACE procedure. Although ACE is an effective method of promoting faecal continence, it is essential to determine the aetiology of incontinence and sphincter function before operation.

Management of the impalpable testis: the role of laparoscopy.

PURPOSE: To evaluate the use of laparoscopy in the management of the impalpable testis. DESIGN: A retrospective analysis of the clinical findings, interventions, and outcome in 87 consecutive boys undergoing laparoscopy for 97 impalpable testes. RESULTS: Fifty seven testes were either absent (n = 35) or present as a small remnant (n = 22), which was removed at contemporaneous groin exploration. There were 27 intra-abdominal testes, including four hypoplastic testes, which were removed laparoscopically. The 13 remaining viable testes were located in the groin. Conventional orchidopexy followed laparoscopy for 21 testes, and was successful in 17 cases. Two stage laparoscopically assisted Fowler Stevens orchidopexies were performed for 13 intra-abdominal testes, with eight satisfactory results. Ultrasound evaluation significantly reduced the number of conventional orchidopexies following laparoscopy. IMPLICATIONS: Laparoscopy is a rational and safe approach for precise localisation of the impalpable testis. Laparoscopically assisted two stage orchidopexy is a successful treatment procedure for intra-abdominal testes.

Management of blunt liver trauma in children.

INTRODUCTION: Management of liver trauma in childhood represents a rare but formidable challenge. METHODS: Clinical presentation, grade of liver injury and Injury Severity Score (ISS) were studied in 11 cases of blunt liver trauma to examine factors influencing outcome. RESULTS: Seven of the 11 children were injured severely and had an ISS greater than 16. Seven who were haemodynamically stable were treated without operation, but four required surgery for grade III, IV and V liver injuries. Two children had primary repair of hepatic lacerations. Perihepatic packing was employed in two other cases (grade IV and V injury) for uncontrollable haemorrhage. Delayed debridement and thrombectomy plus vena cava repair with suturing of liver lacerations in these patients obviated heroic efforts at primary repair. Nine children survived. There were two deaths from head and neck trauma. DISCUSSION: Selected children with liver trauma can be managed non-operatively using established trauma guidelines. Perihepatic packing is recommended in unstable patients with complex injuries, followed by delayed definitive repair.

Wandering calcified ovary in children.

A case of a mobile, calcified, intrapelvic mass representing an autoamputated ovary in an asymptomatic girl is presented. There was no previous history of acute abdominal pain indicating possible torsion of the adnexa. The diagnosis should be suspected when radiological investigations show a freely mobile, calcified intraperitoneal mass incidentally in young females with absent adnexa. The diagnostic and therapeutic role of laparoscopy in this case is emphasised.

Posterior urethral valves. Does prenatal diagnosis influence prognosis?

Forty-eight boys with posterior urethral valves were reviewed to assess the impact on prognosis of prenatal ultrasonography, which had been the method of diagnosis in 17 of them; the remaining 31 presented postnatally. Renal function before treatment was mildly to moderately impaired in 59% of those presenting prenatally, 42% presenting before 3 months of age and 25% of those first presenting when older than 3 months. All but one of the infants diagnosed prenatally had clinical signs and/or elevated serum creatinine in the early neonatal period. Eight had associated maternal oligohydramnios, of whom 6 presented prenatally and 2 postnatally: this factor was associated with a poor prognosis, with renal impairment persisting in 6 of these patients after treatment. Renal function did not relate consistently to the presence or otherwise of vesicoureteric reflux (unilateral or bilateral). Twelve of the patients who presented postnatally had normal fetal ultrasonography late in pregnancy, of whom 11 had upper tract dilatation when investigated. Six of these patients who currently have impaired renal function did not have maternal oligohydramnios, and it is argued that antenatal detection of the condition would have been beneficial in less than 20% of patients presenting postnatally with posterior urethral valves.