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.

"Hernia-itis", a case report.

We describe a case of an incarcerated hernia in which there was good evidence that infection was the primary aetiology. We propose the term hernia-itis to describe similar cases to facilitate identifying them in the literature.

Surgical management of inflammatory bowel disease.

AIM: To evaluate the outcome and morbidity after major surgical interventions for inflammatory bowel disease (IBD). METHODS: Retrospective case note analysis of 227 children referred to a tertiary referral centre between 1994 and 2002 for treatment of IBD. RESULTS: 26 of 125 children with Crohn's disease (21%) required surgical management. 13 with disease proximal to the left colon underwent limited segmental resections and primary anastomosis, without significant morbidity. Primary surgery for 13 children with disease distal to the transverse colon included 6 subtotal-colectomies or panprocto-colectomies. All seven children undergoing conservative segmental resections (three with primary anastomosis, four with stoma formation), required further colonic resection or defunctioning stoma formation. All three children undergoing primary anastomosis developed a leak or fistula formation. 22 of 102 children with ulcerative colitis (22%) required surgery. Definitive procedures (n = 17) included J-pouch ileoanal anastomosis (n = 11), ileorectal anastomosis (n = 2), straight ileoanal anastomosis (n = 3), and proctectomy/ileostomy (n = 1). Five children await restorative surgery after subtotal colectomy. Median daily stool frequency after J-pouch surgery was 5 (range 3-15), and 10 of 11 children reported full daytime continence. All three children with straight ileoanal anastomosis had unacceptable stool frequency and remain diverted. CONCLUSION: The complication rate after resectional surgery for IBD was 57% for Crohn's disease, and 31% for ulcerative colitis. In children with Crohn's disease, limited resection with primary anastomosis is safe proximal to the left colon. Where surgery is indicated for disease distal to the transverse colon, subtotal or panproctocolectomy is indicated, and an anastomosis should be avoided. Children with ulcerative colitis had a good functional outcome after J-pouch reconstruction. However, the overall failure rate of attempted reconstructive surgery was 24%, largely owing to the poor results of straight ileoanal anastomosis.

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.

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.

Classification and risk-factor analysis of infections in a surgical neonatal unit.

BACKGROUND/PURPOSE: Nosocomial infection may result in significant morbidity in surgical neonates. Traditionally, nosocomial infections are classified using time cut-off points. Gastrointestinal carriage of microorganisms has not been used as a criterion for classifying infection in surgical neonates. The aims of the study were to (1) determine the overall infection rate, (2) distinguish between nosocomial and community acquired infections using a 48-hour postadmission cutoff and the carrier state criterion, and (3) determine risk factors for clinical infection. METHODS: A 1-year prospective observational cohort study was undertaken in a regional neonatal surgical unit between 1997 and 1998. All infants residing for >/=3 days in the unit were included in the study (n = 167). Patient demographics, including illness severity (PRISM score), were recorded for all infants. Surveillance throat and rectal swabs were obtained on admission and twice weekly thereafter to determine carrier status. Carriage was defined as isolation of the same microorganism from at least 2 consecutive surveillance samples. Infective episodes were diagnosed if a clinical diagnosis of local or general inflammation was microbiologically proven. RESULTS: A total of 167 infants responsible for 174 admissions were studied. Median gestational age was 38 weeks (range, 24 to 42), median birth weight was 3 kg (range, 1 to 3.6), median age on admission was 8 days (range, 0 to 142), median length of hospital stay was 8 days (range, 3 to 95). The diagnoses were gastrointestinal disorders (n = 96), abdominal wall defects (n = 22), neural tube defects and hydrocephalus (n = 17), thoracic disorders (n = 16), urologic disorders (n = 12), and abdominal tumours (n = 4). Twenty-five infants had 33 episodes of infection giving an overall infection rate of 14.9%. The predominant infecting organism was Stapylococcus aureus (n = 11); others were enterococcus, coagulase negative staphylococcus, Candida spp, Gram-negative bacilli, and anaerobes. A total of 27 of 33 infective episodes were caused by microorganisms carried by the infants on admission (primary endogenous). Only 6 children had "true" nosocomial infections. Using a traditional 48 hour cutoff, 87% of the infections were classed as nosocomial. Birth weight, presence of central venous line, PRISM score, and length of stay were identified as significant risk factors for developing clinical infection. CONCLUSIONS: (1) Carriage allowed us to identify the true nosocomial infection rate (microorganisms acquired in the unit), which was only 18%. In contrast, using a traditional 48 hour cutoff, 87% of the infections would have been classed as nosocomial and warranted unnecessary cross-infection investigations. (2) The results of this study confirm that birth weight, illness severity (PRISM score), presence of central venous catheter, and length of hospital stay were independent risk factors associated with clinical infection in surgical neonates.

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.

Mycotic aortic aneurysm in the newborn.

A fatal case of mycotic aneurysm of the aorta is presented. This was probably the consequence of umbilical artery catheterization. Unfortunately, the 29-weeks-old newborn died from septic complications.

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.

Intra-arterial calcium stimulation test in the investigation of hyperinsulinaemic hypoglycaemia.

OBJECTIVE: To investigate the use of a calcium infusion test in the diagnosis and localisation of insulin secreting tumours in children. PATIENTS: Three patients with persistent hypoglycaemia of infancy (PHHI). PROCEDURE: During planned selective coeliac and mesenteric arteriography, serial samples were taken from a catheter in the right hepatic vein for insulin measurement following the injection of calcium gluconate. RESULTS: In all three children, selective intra-arterial calcium stimulation produced a significant rise in plasma insulin and was of value in localising the pancreatic abnormality in one child. In vitro studies on islets of Langerhans isolated from this patient following partial pancreatectomy showed unresponsive intracellular calcium signalling of the cells when stimulated with high extracellular concentrations of glucose and potassium or with sulphonylurea drugs (tolbutamide), but normal responsiveness to increasing extracellular calcium concentrations. CONCLUSIONS: The findings suggest a functional abnormality of the calcium channel in PHHI and provide a rationale for the reported efficacy of channel blocking drugs in this condition. The role of selective intra-arterial calcium stimulation in the diagnosis of hyperinsulinaemic hypoglycaemia in childhood warrants further investigation.

Cutaneous angiosarcoma of the distal extremity.

A case of childhood cutaneous angiosarcoma is presented to emphasize the importance of accurate histological diagnosis in lymphovascular malformations that behave atypically and to review the natural history, aetiology, and differential diagnosis of this condition.

Pancreatic inflammatory tumour: a rare entity in childhood.

Pancreatic tumours are rare childhood neoplasms. Inflammatory myofibrohistiocytic tumours (IMTs) represent an uncommon but distinct pathological subgroup that creates diagnostic and therapeutic dilemmas. We report a case of IMT arising from the body and tail of the pancreas in an 8-year-old girl presenting with a mass and abdominal pain. A locally aggressive tumour with no evidence of distant metastasis was encountered at laparotomy and resected. Pathologically, the tumour revealed a mixed inflammatory cell infiltrate with myofibrohistiocytic proliferation. These features can resemble a sarcoma. A review of the literature is provided which emphasises the clinical features, pathological findings, and management strategies for these unusual tumours. Complete surgical excision, aided by radiological surveillance, appears to offer the best guidelines for definitive management.

Air gun injuries in children: a continuing cause for concern.

Air guns are commonly used for sporting entertainment. The inappropriate use of these weapons often leads to injury. In general, trauma inflicted by air weapons is trivial. However, the potential for more serious and fatal injuries is significant. We report two cases of serious air gun injury in children and a review of the relevant legislation covering the use of air weapons in the UK. Enhanced public education and awareness as well as coordinated policies by organizations involved in the care of children is desirable to prevent future calamities involving air weapons.

Evidence-based surgery: interventions in a regional paediatric surgical unit.

OBJECTIVES: To determine the proportion of paediatric surgical interventions that are evidence-based and to identify areas where randomised controlled trials (RCTs) or further research are required. DESIGN: Prospective review of paediatric general surgical inpatients. SETTING: A regional paediatric surgical unit. SUBJECTS: All consecutive paediatric general surgical patients admitted in November, 1995. MAIN OUTCOME MEASURES: Each patient on whom a diagnosis had been made was allocated a primary diagnosis and primary intervention (n = 281). On the basis of expert knowledge, Plusnet Medline, and ISI Science Citation database searches, each intervention was categorised according to the level of supporting evidence: category 1, intervention based on RCT evidence; category 2, intervention with convincing non-experimental evidence such that an RCT would be unethical and unjustified; category 3, intervention without substantial supportive evidence. RESULTS: Of 281 patient interventions, 31 (11%) were based on controlled trials and 185 (66%) on convincing non-experimental evidence. Only 23% of interventions were category 3. CONCLUSIONS: In common with other medical specialties, the majority of paediatric surgical interventions are based on sound evidence. However, only 11% of interventions are based on RCT data, perhaps reflecting the nature of surgical practice. Further RCTs or research is indicated in a proportion of category 3 interventions.

Transtrigonal approach for excision of müllerian duct structures.

Persistent müllerian structures attached to the prostatic urethra in the male may cause urinary incontinence and infection. The transtrigonal technique allows complete excision with preservation of normal bladder function and control. We describe the operative technique in detail.

Evaluation of an enteric-coated naproxen pellet formulation.

An enteric-coated, pellet formulation of naproxen has been evaluated in eight healthy subjects. Each volunteer was dosed with 153Sm-labelled, enteric-coated pellets on two occasions, once whilst fasted and once after breakfast. Gastrointestinal transit was followed using gamma scintigraphy and drug absorption compared with that from uncoated naproxen pellets dosed on a separate occasion. The pH in the stomach and intestines was monitored using radiotelemetry capsules. Gastric emptying was delayed by dosing after breakfast, but small intestinal transit of the enteric-coated formulation was the same on both occasions. The highest pH recorded from the stomach was 4.0 and in all subjects the pH rose to at least 7.3 in the small intestine. The onset of drug absorption was fastest from the uncoated formulation and slowest from the coated pellets taken after breakfast. The total amount of drug absorbed was the same on all three occasions.