Long-term outcome and colonic motility after the Duhamel procedure for Hirschsprung's disease.

BACKGROUND/PURPOSE: The aim of this study was to investigate long-term functional outcome and colonic motility in children who had undergone the Duhamel-type operation for Hirschsprung's disease (HSCR). METHODS: All patients (n = 91) who underwent the Duhamel or Lester Martin Modified Duhamel operation for HSCR from 1980 to 1991 were included in the study. Twenty-two healthy age-matched children were used as controls. Functional outcome was assessed by questionnaire (response rate 100%). Total and segmental colonic transit time (CTT) was determined using the saturation method (80% participation rate). RESULTS: Outcome scores were significantly worse in the study group for patients with rectosigmoid (RS, P < .001), long segment (LS, P < .001), and total colonic (TC) aganglionosis (P < .05), when compared with controls. The CTT was significantly prolonged in the RS group (P = .01) compared with LS, TC, and control groups; this was caused by prolonged "rectosigmoid" transit in the RS group compared with controls (P = .012). There was a positive linear correlation (P = .0002) between age and outcome score in patients with RS disease unrelated to CTT. Nine patients required a late long-term enterostomy. A satisfactory outcome (defined as outcome score > or = 10th percentile of the control group, and absence of stoma or requirement for major revisional surgery) was seen in only 42% of patients overall and in 79% of patients over 14 years of age. CONCLUSIONS: The Duhamel procedure, in common with other pull-through procedures, is associated with significant long-term morbidity, the aetiology of which is poorly understood.

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.

Audit of 200 consecutive aortic aneurysm repairs carried out by a single surgeon in a district hospital: results of surgery and factors affecting outcome.

It has been suggested that surgery for abdominal aortic aneurysm (AAA) be confined to designated centres. A prospective audit of 200 consecutive AAA repairs at a district general hospital was performed between 1981 and 1990. The 30-day mortality rates for elective, symptomatic and ruptured aneurysm repair were 1.4%, 3.5% and 30%, respectively. The major factor affecting outcome after the mode of presentation was the age of the patient, with 30-day mortality rates for emergency treatment increasing from 21% (age range 60-69 years) to 42% (age range 70-79 years). This mortality rate for ruptured aneurysms is an underestimate, with two-thirds of patients with rupture dying before reaching hospital and some patients dying in hospital undiagnosed. The major contribution to improved overall mortality would therefore be detection before rupture (usually by ultrasound) and improved diagnostic accuracy. Many patients with ruptured aneurysms had symptoms for only a short period before presentation (42% for less than 6 h) and required urgent surgery (26% reached theatre within 1 h). These two factors make long-distance transfer of these patients an unrealistic option. The concentration of this type of surgery in relatively few centres will distance the patient from their relatives and reduce the opportunity for the majority of junior doctors to acquire an understanding of the presentation, natural history and management of aortic aneurysms. This understanding when combined with a screening programme is likely to have a far greater impact on the overall mortality from AAA than restricting the centres for surgical treatment.

Retained central venous lines (CVLs) after attempted removal: an 11-year series and literature review.

BACKGROUND: Central venous lines (CVLs) are frequently used in the management of many neonatal and pediatric conditions. Failure to remove the luminal part of the line (retained CVL) is rare. Consequently, there is lack of experience and consensus in its optimal management. AIM: To document the incidence and management of retained CVLs in a tertiary pediatric surgical centre with access to interventional cardiology services. To review the literature and report efficacy/morbidity of attempted extraction of retained CVLs. METHODS: Children with retained CVLs were identified from departmental morbidity and mortality records over an 11-year period. A literature search was performed in PubMed and Scopus to identify studies reporting retained CVLs (earliest date to 1 January 2012). This was supplemented by scanning bibliographies of retrieved articles. RESULTS: The 11-year incidence of retained CVL was 0.3% (n=10; median duration in-situ 66.5 {range 47-146} months). The underlying pathology in 8 was cystic fibrosis. Antegrade transfemoral snare retrieval was successful in 6 of 7 attempts. In the remaining 3, a conservative approach was adopted following consultation with the family. None of the 4 with retained CVL developed complications (median follow-up 7.5 {range 1-53} months). The literature describes 38 pediatric index cases (including 10 from the current series). Seventeen (49%) were managed conservatively either intentionally or by default after failed endovascular removal attempt (n=4). No complications directly attributed to retained CVLs have been reported (median follow-up 40 {range 1-120} months). Reported morbidity associated with endovascular retrieval includes: procedural failure 30%, line embolization 8%, and intra-operative thrombo-embolism 8%. CONCLUSION: Literature regarding management of retained CVLs is anecdotal. Although uncommon, the complication should feature in consent for removal of CVLs. Conservative management carries long-term risks of infection, thrombosis, and even migration, albeit unquantified over a child's lifetime. Endovascular retrieval is feasible with appropriate expertise.

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.

Less is best? The impact of urokinase as the first line management of empyema thoracis.

To assess the impact of intrapleural urokinase and small tube thoracostomy on the management of childhood empyema thoracis. The study population included 38 children presenting consecutively to a regional surgical unit with empyema thoracis from January 2001 to December 2003. Children with malignancy, immunodeficiency and complex intercurrent illness were excluded. Primary outcome variables were the need for second intervention and duration of stay, with other variables including duration of antibiotics, serial CRP and amelioration of pyrexia. Interventions were: tube thoracostomy (16-20 Fr) alone (n=2), tube thoracostomy (6-10 Fr)+urokinase (n=17), thoracoscopy (tube: 20-24 Fr) (n=9), thoracotomy (tube: 16-24 Fr) (n=10). There were no differences in age, weight or length of prodromal symptoms, between treatment groups. There were no differences in primary outcome variables, although no child undergoing thoracotomy required further intervention. The duration of intravenous antibiotics was similar in all groups. Amelioration of pyrexia was more rapid in children undergoing thoracotomy. There were no differences seen with regard to decline in CRP level. Small tube thoracostomy and intrapleural urokinase had a similar outcome to more invasive therapies such as thoracotomy or thoracoscopy thereby supporting the evidence base for urokinase and tube drainage as first line intervention.

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.

Paediatric anatrophic nephrolithotomy; stone clearance - at what price?

OBJECTIVE: To evaluate the functional outcome of anatrophic nephrolithotomy in children. PATIENTS AND METHODS: All children undergoing anatrophic nephrolithotomy for complex branching and multiple renal calculi over an 11-year period were studied prospectively. Demographic data, treatment details and outcome, as assessed by X-ray, ultrasonography and isotope studies, were recorded. Anatrophic nephrolithotomy was carried out with surface cooling of the kidney followed by nephrostomy drainage for 5-7 days. RESULTS: Nine children (median age 4 years, range 7 months to 9 years) underwent anatrophic nephrolithotomy. Predisposing factors included urinary tract infection (by Proteus mirabilis) in all and hyper-calciuria in two children. The median (range) total ischaemic time at operation was 25 (15-40) min and the operative duration 150 (120-200) min. Three children required a blood transfusion. Stone clearance was incomplete in one child. There was no recurrent stone formation after a long-term follow-up (median 32 months, range 14-107) in the other patients. Isotope studies showed impaired split renal function (<40%) in six children before surgery; there was a significant decline (>5%) in divided function in five children (range 6-16%) after surgery. CONCLUSION: Anatrophic nephrolithotomy is an effective means of rendering children with branching calculi stone-free, but this study suggests that it leads to some further parenchymal damage.

Small bowel tumours: a diagnostic challenge.

In a 20-year retrospective study of 43 patients having surgery for small bowel neoplasia in a district general hospital, the commonest pathologies were lymphoma (12), carcinoid (12) and adenocarcinoma (11). Malignant neoplasms occurred in 38 patients presenting on average at 68 years of age. In 4 patients a synchronous gastrointestinal neoplasm was found. The average duration of symptoms was 11 weeks for operated patients. In 31 patients the presentation was as a surgical emergency. Only 10 had imaging investigations prior to emergency presentation from which diagnostic information was obtained in 1 patient and helpful information in a further 4. The 5-year survival figures of patients with small bowel neoplasia were carcinoid (50%), lymphoma (27%) and adenocarcinoma (15%), reflecting the poor prognosis of these patients even after 'curative resection'. Patients with carcinoid tumours were the exception, as long-term survival was not unusual even without complete resection of all macroscopic disease.

Tumour vasculature--a potential therapeutic target.

The tumour vasculature is vital for the establishment, growth and metastasis of solid tumours. Its physiological properties limit the effectiveness of conventional anti-cancer strategies. Therapeutic approaches directed at the tumour vasculature are reviewed, suggesting the potential of anti-angiogenesis and the targeting of vascular proliferation antigens as cancer treatments.

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.

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.

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.

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.

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.