Surgical risk factors for Hickman catheter sepsis: a prospective study.

Catheter-related sepsis (CRS) is a major cause of morbidity in patients receiving chemotherapy and prolonged parenteral nutrition. To determine whether avoiding emergency insertions by using a planned elective list and adopting a 'no-touch' technique has a role in reducing CRS, all cuffed central venous catheters inserted by the open method between 1999 and 2000 were prospectively followed for a total duration of 12 months. The incidence of early sepsis (within 30 catheter days) that could be attributed to surgical factors was studied. CRS was defined as the presence of any two of the following: (1) signs of clinical sepsis without an obvious focus; (2) positive cultures in blood obtained from the catheter; and (3) clinical improvement following removal. A total of 146 catheters were inserted in 130 patients; 15 had a second and 1 had a third catheter inserted. Early CRS was encountered in 13 cases (9%); 95 catheters were inserted on an elective list and 51 on an emergency basis. The distributions of age, sex, number of lumens, neutrophil count, and underlying diagnosis were similar between the groups. There was no significant difference (P = 1) between elective (9/95) and emergency (4/51) insertions. A total of 47 catheters were inserted by the 'no-touch' technique and 48 by the manual technique. There was no significant difference in early sepsis (P = 0.7) between the two techniques (6/47 vs 3/48). Thus avoiding emergency insertion or adopting a 'no-touch' technique does not reduce early CRS. Larger prospective studies are warranted to identify surgical risk factors.

Implanted vascular access devices (ports) in children: complications and their prevention.

Implanted vascular access devices (ports) play a major role in the management of children with cystic fibrosis (CF) and many haematological conditions. With the expanding use of ports, new and more frequent complications are being encountered. To retrospectively review the complications associated with ports, the case notes of all patients who underwent insertion of a port between 1997 and 2000 were analysed. Details of the underlying disorder, type of vascular device, nature of use, and complications were recorded; 55 ports were inserted in 41 patients (a second port was required in 12, a third port in 2) during this period. Their underlying diagnoses were CF (11), haemophilia (4), haemolytic anaemias (2), immunological disorders (6), solid neoplasms (8), and leukaemia (10). Thirteen ports (24%) were removed and replaced for various complications: infection (2), blockage (4), leak (2), dislodgement (2), and malposition (3). Including four port-related problems managed conservatively (3 access problems managed by change in access technique; 1 blockage managed by urokinase), the over all complication rate was 31%. Ports thus have a high complication rate with long-term use. Selecting the right port system, proper installation of the port chamber, and efficient handling and maintenance by trained staff could prevent the vast majority of port-related complications.

Perianal abscess in childhood.

Perianal abscess (PA) is a common condition encountered in childhood, but its optimal primary treatment is uncertain. Treatment of PA by incision and drainage (I & D) alone is associated with an unacceptably high recurrence rate, either as fistula-in-ano (FIA) or as PA. To identify possible causes of recurrence and assess the value of concomitant laying open of a fistulous tract at the time of primary incision and drainage, the case notes of all children who presented to our institution with a PA between January 1992 and January 1997 were reviewed retrospectively. Thirty-three cases were identified (29 boys and 4 girls). A fistulous tract was identified and laid open at the time of primary drainage in 20 cases, whilst 13 were treated by I & D alone. Following primary drainage, there were 7 recurrences (21.2%) (FIA 5 and PA 2). All recurrences had been primarily treated by drainage alone, whilst there were no recurrences in patients who had also undergone fistulotomy at the time of primary drainage. Thus the primary treatment of PA in childhood should involve a careful search for a coexisting fistula and treatment of this by fistulotomy.

Pneumatic dilatation for childhood achalasia.

Treatment of achalasia by pneumatic balloon dilatation (PBD) is well established in adults. Due to limited experience and the rarity of the condition in children, there are relatively few reports in the paediatric literature. Although PBD has been reported as a primary method of treatment, there are no reports of secondary PBD for childhood achalasia. Between 1995 and 1999, five patients underwent treatment for achalasia (age: 9-14 years, M:F = 4:1). The presenting symptoms were dysphagia (5). vomiting episodes (2), aspiration (1), food-bolus obstruction (1), and failure to thrive (1). In all patients a barium swallow and manometry were used to confirm the diagnosis. Three underwent primary PBD. Two who had previously undergone surgical myotomy underwent secondary PBD for recurrence of symptoms. Dilatation was performed using a 35-mm balloon with the child under general anaesthesia. Technical success was defined as demonstration of a waist under screening at lower pressures followed by abolition of the waist at higher pressures. In addition to reviewing our results, a systematic review of the literature was performed (Medline, Cochrane Library, Pubmed, Embase). Three patients (primary dilatation) showed excellent improvement after a single dilatation. In two cases (secondary dilatation) three and five attempts were required. No complications were encountered. The mean follow-up period was 2 years (1-3.5 years) and four patients remained asymptomatic, an overall success rate of 80%. The literature review revealed similar good results in most of the recent reports. Thus, PBD as a primary treatment for childhood achalasia has a success rate of 70%-90% with minimal side effects, short hospital stay, and good patient acceptability over an operation. We have also established the usefulness of this method as a secondary treatment when symptoms recur after surgery.

Prenatal sonographic features of congenital lobar emphysema.

The prenatal sonographic features of congenital lobar emphysema (CLE) have not been well characterised. Five cases have been reported in the literature and on all these occasions either an echogenic (3) or a cystic (2) lung lesion was detected prenatally and the diagnosis was confirmed after the operation. This is the sixth case of CLE in the literature with prenatal sonographic features documented. The prenatal scans of a 23-year-old lady performed at 22 weeks of gestation revealed cystic lesions and increased echogenicity of the right fetal lung. There were no other anomalies and the karyotype was normal. The lesion decreased in size at 28 weeks and the baby was born by a normal vaginal delivery at 41 weeks. CT scan performed on day 6 confirmed cystic changes on the right lung with compression of the right lower lobe. A repeat CT scan performed at 4 months revealed extensive cystic changes in a hyper-inflated right lung and mediastinal shift to the left. At operation, abnormally inflated right upper and middle lobes were found suggesting a CLE. There were no subsequent complications after removal and histology confirmed CLE. The reported cases are reviewed and the prenatal sonographic features of CLE are discussed.

Mixed foregut cyst associated with esophageal atresia.

The authors report an unusual case of a thoracoabdominal foregut malformation with components of bronchogenic, esophageal duplication, and pancreatic enterogenous cysts, that presented in a child with esophageal atresia. J Pediatr Surg 36:939-940.

"Cuff-stitch" to prevent inadvertent dislodgement of central venous catheters.

The insertion of central venous catheters (CVCs) is an established practice in the management of children who need long-term total parenteral nutrition or chemotherapy. Inadvertent falling out of CVCs before the cuff becomes incorporated in the tissues is a commonly encountered problem. The technique described involves inserting a circular stitch in the subcutaneous plane before the catheter is placed. Once the CVC is pulled into position, the "cuff-stitch" lays around the catheter distal to the cuff, narrows the tunnel, and prevents accidental dislodgement.

Intestinal complications associated with twin-twin transfusion syndrome after antenatal laser treatment: Report of two cases.

Two infants found to have ileal atresia after birth and who had intrauterine laser treatment to interupt twin to twin transfusion are presented. The donor twin in each pregnancy died in utero.

Ecthyma gangraenosum--a trap for the unwary.

Neutropaenic patients are at particular risk of developing a pseudomonal fasciitis known as ecthyma gangraenosum. Despite the similarities with necrotising fasciitis, Fournier's gangrene has a very different aetiology and management.

Mucin gene expression in Barrett's oesophagus: an in situ hybridisation and immunohistochemical study.

BACKGROUND AND AIMS: Mucin genes are expressed in a site specific manner throughout the gastrointestinal tract. Little is known about the expression pattern in the oesophagus. In this study we have investigated MUC gene expression in both the normal oesophagus and specialised intestinal metaplasia (Barrett's oesophagus). PATIENTS: Archived paraffin embedded material from eight specimens of normal oesophagus, 18 Barrett's oesophagus, eight gastric metaplasia, six high grade dysplasia, and six cases of adenocarcinoma were examined for expression of the mucin genes MUC1-6. METHODS: Mucin mRNA was detected by in situ hybridisation using [(35)S] dATP labelled oligonucleotide probes. Mucin core protein was detected by immunohistochemistry. RESULTS: Normal oesophagus expressed MUC5B in the submucosal glands and MUC1 and MUC4 in the stratified squamous epithelium. Barrett's oesophagus strongly expressed MUC5AC and MUC3 in the superficial columnar epithelium, MUC2 in the goblet cells, and MUC6 in the glands. In high grade dysplasia and adenocarcinoma there was downregulation of MUC2, MUC3, MUC5AC, and MUC6, but upregulation of MUC1 and MUC4 in half of the specimens examined. CONCLUSIONS: Normal oesophagus and Barrett's oesophagus have a novel pattern of mucin gene expression. Barrett's oesophagus expressed the mucins associated with normal gastric epithelium and normal intestinal epithelium. While most mucin genes were downregulated in severely dysplastic and neoplastic tissues, there was upregulation of the membrane bound mucins MUC1 and MUC4. This may prove useful in detecting early signs of progression to adenocarcinoma of the oesophagus.

Conservative treatment for girls with nonmetastatic rhabdomyosarcoma of the genital tract: A report from the Study Committee of the International Society of Pediatric Oncology.

PURPOSE: To report the results of a conservative multimodal approach in girls with nonmetastatic rhabdomyosarcoma (RMS) of the genital tract, treated in International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumors 84 and 89 protocols. PATIENTS AND METHODS: From 1984 to 1994, 38 girls with RMS of the genital tract (vulva, vagina, uterus) were treated in SIOP protocols. With the exception of patients with rare small tumors, which were resected at the start of the studies, all patients received initial chemotherapy (CHT) (ifosfamide, vincristine, and actinomycin D). Local treatment including surgery, brachytherapy (BT), and external-beam radiotherapy (ERT) was given only to girls who did not achieve complete remission (CR) with CHT or who subsequently relapsed. RESULTS: The primary tumor originated in the vulva or vagina in 27 girls and in the uterus in 11. The overall survival rate (+/- SE) was 91% +/- 6% at 5 years, and the event-free survival rate was 78% +/- 7%. At a median follow-up of 5 years, 30 girls were alive and in first CR and five were alive and in second CR. Four patients treated with complete resection of the tumor at diagnosis received less CHT. Thirteen patients were treated with CHT alone. In 17 patients, local treatment was necessary to achieve complete local control, for a residual mass after initial CHT (10 patients), for viable tumor on biopsy (three patients), or for local relapse (four patients). The local treatment used was radiotherapy (RT) (ERT in three patients, BT in seven), radical surgery with uterine ablation (three patients), RT and radical surgery (three patients), and conservative surgery with RT (one patient). CONCLUSION: Girls with nonmetastatic RMS of the genital tract have an excellent prognosis. We found no difference in outcome between uterine and vulvovaginal RMS. Local treatment does not seem necessary in patients who have a complete response to CHT. When a local treatment is needed, BT may be an alternative to radical surgery or ERT.

Role of surgical biopsies in the management of bone marrow transplant patients.

BACKGROUND: Bone marrow transplantation (BMT) patients frequently develop life-threatening problems that have similar clinical presentations but differing aetiologies. Despite intensive investigation by haematological, biochemical, and microbiological means, accurate diagnosis is not always possible. Histological and microbiological examination of biopsies from the affected organ may be indicated to enable an accurate diagnosis to be made in these patients. Here we assess the indications, findings, and outcomes in patients who have required surgical biopsy after BMT. PROCEDURE: We retrospectively reviewed all BMT patients who had surgical biopsies between February 1994 and January 1997. Twenty-six patients (1-46 years, median age 10 years) underwent 40 biopsies from the upper and lower GI tract, lung, or liver. Indications for BMT were: relapsed leukaemia = 18; other types of leukaemia = 3; aplastic anaemia=3; other diseases = 2. Type of BMT: matched related donor = 3, unrelated T-cell depleted donor = 23. RESULTS: Eleven (42%) cases had a change in management; 4 (16%) patients avoided further aggressive therapy because of poor prognosis. Unexpected diagnoses were found in 7 biopsies: 1 acute colitis, 1 duodenal ulcer, 1 liver aspergilloma, 2 transfusion siderosis, 1 radiation fibrosis of the lung, and 1 cytomegalovirus infection of the lung. Three patients were noted to have complications after their procedure. CONCLUSIONS: Surgical biopsies for undiagnosed problems can be of benefit in the management of very sick patients who have received bone marrow transplantations. Despite the fact that these patients are so unwell, there is a low rate of complications related to surgery and anaesthesia.

Histochemical and genetic analysis of colonic mucin glycoproteins in Hirschsprung's disease.

BACKGROUND/PURPOSE: Mucin glycoproteins (mucins) recently have been shown to be deficient in the colonic mucosa of patients with Hirschsprung's disease (HD). The authors performed a detailed histo- and immunohistochemical analysis of mucins in the colonic mucosa and studied the expression of mucin genes to characterize histologically mucin quality and gene expression in HD compared with controls. METHODS: Paraffin-embedded 4-microm thick sections from patients with HD (n = 11 ganglionic, 10 aganglionic) and controls (n = 19) were taken. Slides were stained with mild periodic acid Schiff with and without saponification with KOH (reacts with O-actylated mucins), high iron diamine/alcian blue (differentiates sulphated v nonsulphated mucins), the monoclonal antimucin antibodies, PR3A5 (against di- and tri-O-acetylated sialic acids) and 91.9H (against sulphated mucins). O-acetylation and sulphation both confer an increased resistance of mucins to bacterial degradation and are thought to be important in the defensive function of the colonic mucus gel layer. In situ hybridization was used to study expression of the mucin genes MUC 1, 2, 3, 4, 5AC, 5B, 6, 7, and 8. [35S]-sulphate-labelled antisense oligonucleotide 48mer probes designed to the known tandem repeat domains of MUC genes were used. After hybridization and washing the slides were opposed to Hyperfilm MP for 7 days. The autoradiographs were scored by three independent observers for differences in expression and by image analysis. Those with positive findings were dipped in photographic emulsion, developed, and counterstained for photomicrographs. RESULTS: There were different patterns of staining dependent on the region of the colon and especially the age of the patient with three reagents. No significant differences in the histological staining pattern was detected between HD patients and controls. The colonic mucins in HD were found to be primarily O-acetylated and sulphated. The MUC gene expression was similar in patients and controls. MUC2 and 4 were strongly expressed, MUC1, 3, and 5B had moderate to weak expression, and MUC 5AB, 6, 7, and 8 had baseline expression. CONCLUSIONS: The mucin glycoproteins in children with HD, although quantitatively deficient, show no qualitative differences on histo- and immunohistochemical staining from normal controls. The expression of all the known mucin genes, the genetic control of mucin secretion, and the quality of mucins, is similar to normal controls.

Where should paediatric surgery be performed?

We have tried to review the evidence for the organisation of paediatric surgical care. Difficulties arise because of the lack of published data from district general hospitals concerning paediatric surgical conditions. Hence much of the debate about the surgical management of children is based on anecdotal evidence. However, at a time when the provision of health care is being radically reorganised to an internal market based on a system of purchasers and providers it is more important than ever to understand the issues at stake. Two separate issues have been discussed: the role of the specialist paediatric centre and the provision of non-specialist paediatric surgery in district general hospitals. There are arguments for and against large regional specialist paediatric centres. The benefits of centralisation include concentration of expertise, more appropriate consultant on call commitment, development of support services, and junior doctor training. The disadvantages include children and their families having to travel long distances for care, and the loss of expertise at a local level. If specialist paediatric emergency transport is available the benefits of centralisation far outweigh the adverse effects of having to take children to a regional paediatric intensive care centre. Specialist paediatric centres are aware of the importance of treating children and their parents as a family unit as highlighted by the Platt committee; this is an important challenge and enormous improvements have occurred to provide proper accommodation for families while their children are treated in hospital. To keep these arguments of large distances and separation from the home in context, one paediatric intensive care unit in Victoria, Australia, providing a centralised service to a region larger in are than England and with a similar admission rate, has a lower mortality rate than the decentralised paediatric intensive care provided in the Trent region of the UK. There is clear evidence that all neonatal surgery and anaesthesia should be conducted only by specialists. The debate now centres around the number of complex surgical cases a unit should treat to maintain its specialist status. The NHS executive, in its guidelines on contracting for specialist services, emphasises that "Sensible contracting needs to take into account the optimum population size not only for the stability of contracted referrals but also to give sufficient 'critical mass' for clinical effectiveness." Achieving this balance has consequences, not just for the maintenance of surgical expertise, but for the essential ancilliary services. There is clear evidence in anaesthesia that anaesthetists doing small numbers of neonatal procedures had significantly worse results. The same seems to be true in the fields of oncology, radiology, pathology, and intensive care. The reasons why the results of management of certain paediatric conditions are better at specialist centres are open to speculation. Presumably greater exposure to rare complex cases, concentration of expertise, more peer review, and a trickle down effect of the multidisciplinary approach all help to keep health care workers up to date with current world practice. In addition, it allows for appropriate specialist on call rotas and dedicated junior staff. If insufficient numbers of specialist surgical cases are being treated at a centre then the whole multidisciplinary team suffers. The 1989 NCEPOD report states "that paediatricians and general surgeons must recognise that small babies differ from other patients not only in size, and that they pose quite separate problems of pathology and management." The need for large centres of paediatric surgical expertise is now accepted by the Royal College of Surgeons of England, the British Association of Paediatric Surgeons, the Senate of Surgery of Great Britain and Ireland, the Royal College of Paediatrics and Child Health, the Royal College of Anaesthetists, the Audit

Turnover of radioactive mucin precursors in the colon of patients with Hirschsprung's disease correlates with the development of enterocolitis.

BACKGROUND/PURPOSE: Mucin glycoproteins (mucins) in the colonic mucus gel layer interact with pathogens performing protective functions by a variety of mechanisms. It is recognised that patients with Hirschsprung's disease (HD) are prone to episodes of enterocolitis even after corrective surgery, the aetiology of which is poorly understood. The authors correlated the turnover of radioactive mucin precursors in organ culture of the proximal ganglionated colon at the time of pull-through with the development of postoperative enterocolitis. METHODS: The colonic mucins in the retained proximal ganglionated colon of nine HD patients at the time of pull-through were studied. Organ culture of intact mucosa was performed with radioactive mucin precursors 35S-sulphate and 3H-glucosamine. Mucins in the secretions and epithelial cells were then purified by gel filtration. Turnover of the isotopes was determined by relating radioactivity to tissue DNA content. These patients were followed up prospectively for a mean duration of 30.8 months. The patients were assigned to one of two groups according to the criteria of requiring hospital admission for enterocolitis during this period. There were five patients in the group that remained well after corrective surgery and four in the group that developed entercolitis. The turnover values of both radioisotopes were analysed for differences in the two groups of patients. RESULTS: Patients in the enterocolitis group had a median value for turnover of 331 dpm/microg DNA, and the group that was well had a median value of 2044 dpm/microg DNA. These differences were statistically significant (Mann-Whitney, P = .037). CONCLUSIONS: A reduced turnover of mucins as shown by incorporation of radioactive precursors will give rise to a defective colonic mucus-defensive barrier. It can be inferred that the lower the turnover, the more prone a patient is to postoperative enterocolitis. It is therefore possible that organ culture with radioactive mucin precursors of the proximal ganglionated mucosa performed at the time of pull-through has a predictive value in the development of postoperative enterocolitis.