Keith B. Stokes

Totally implantable venous access devices in children with cystic fibrosis : incidence and type of complications

BACKGROUND Totally implantable vascular access devices (TIVADs) are accepted as a safe and effective method of facilitating long term intravenous therapy. We report our experience of the use of these devices in children with cystic fibrosis with a particular focus on the incidence and type of complications. METHODS The medical records of patients with cystic fibrosis who underwent placement of a TIVAD at the Royal Children’s Hospital, Melbourne, Australia from January 1987 to October 1996 were reviewed. Venous ultrasonography with Doppler was performed in surviving patients with a TIVAD in situ from November 1996 to April 1997 to detect occult thrombotic complications. RESULTS A total of 57 TIVADs were implanted in 44 children with a median functional duration of 700 days (range 27–3347 days). Twenty one children had devices inserted without complications. Forty eight complications (30 mechanical, 18 infectious) occurred in 36 devices in 23 children during a total functional duration of 53 057 catheter days. Mechanical complications occurred in 53% of devices (one per 1712 catheter days). Symptomatic venous thrombosis occurred five times in four patients (9%). Infectious complications occurred in 32% (one per 2948 catheter days) while sepsis occurred in five devices (9%). Doppler ultrasonography detected unsuspected thrombosis in two of 10 patients examined. CONCLUSIONS While TIVADs provided effective long term intravenous access, septic and thrombotic complications caused significant morbidity in this population. Careful patient selection, adherence to aseptic technique for access and blood sampling, and periodic ultrasonography with Doppler to detect early thrombosis may help reduce these risks.

The diagnostically difficult intussusception: its characteristics and consequences

In the absence of classical symptomatology, the diagnosis of intussusception may be difficult to make. A retrospective review of 630 episodes of intussusception admitted to the Royal Childrens Hospital, Melbourne, over a 15-year period revealed difficulties in diagnosis in 318 cases. The adverse effect of an incorrect initial diagnosis on duration of symptoms at the commencement of treatment, need for surgery, resection rate, complications, and hospital stay is examined. Problems in the assessment of children with intussuception are identified and discussed in the hope that an increased awareness of the diagnostic difficulties and potential pitfalls will reduce the morbidity of this common paediatric condition.

Fulminant hepatic failure with intractable ascites due to an echovirus 11 infection successfully managed with a peritoneo-venous (LeVeen) shunt.

A case is described of an infant who at 5 days of age developed an echovirus 11 infection with hepatic failure and intractable ascites, refractory to medical treatment. The ascites was successfully controlled with a peritoneo-venous (Le Veen) shunt inserted at 6 weeks of age. The shunt was subsequently removed when the infant was 4 months of age with no recurrence of ascites. At long-term follow-up he is growing and developing normally. This case shows that an adult Le Veen shunt can be successfully modified for use in the newborn with worthwhile long-term survival.

Esophageal atresia with tracheoesophageal fistula: End-to-end versus end-to-side repair*

Despite early enthusiasm by some authors for the end-to-side repair of esophageal atresia with tracheoesophageal fistula, many have returned to the end-to-end technique. The present study compares the results of these two operative procedures. A retrospective analysis was made of 52 consecutive cases in which primary repair was performed. The patients were divided according to the three preoperative risk groups descibed by Waterston. The mortality for the end-to-end and end-to-side repairs was similar in each of the three risk groups. Similarly, there was no significant differnences in the incidence of anastomotic leak or recanalization of the fistula. However, the rate of anastomotic stricture in the end-to-end group was significantly higher (p less than 0.001) than in the end-to-side group. A description of the end-to-side technique is given, and its advantages are outlined.

Prognosis of Congenital Diaphragmatic Hernia

Summary: Congenital diaphragmatic hernia (CDH) contributes significantly to perinatal morbidity and mortality. This retrospective study examines the experience of a major teaching hospital to establish survival rates and factors influencing outcome. Survival rates were found to relate closely to the stage at which the diagnosis was made and the presence of associated anomalies. Ultrasound diagnosis early in pregnancy is associated with a higher mortality rate than diagnosis made late in pregnancy or after delivery. Logistic regression analysis and chi‐squared analysis did not establish to a significant degree that any factor, alone or in combination, was a reliable prognostic indicator. It is acknowledged, however, that figures in this series are small. Survival figures are presented to facilitate reliable parental counselling. In particular, the presence of associated major anomalies and the gestational age at which diagnosis is made are of critical importance in accurately counselling parents regarding the prognosis for survival. In this study, excluding terminations, the mortality rate for isolated CDH diagnosis before the 21st week was 45.5%, with a corresponding survival rate of 54.5%. Once the infant was liveborn, however, the survival rate rose to 68.0%, and if the infant survived transfer to a paediatric surgical unit, the survival rate in this study was 73.9%.

C ASE R EPORT: Colonic duplication: A rare cause of obstruction

Complete duplication of the entire large bowel with partial ileal involvement is very rare and diagnosis can often be difficult as illustrated by this case report. We also review the other clinical associations of this rare condition and briefly discuss the embryology of duplications of the gastrointestinal tract.

Diagnostic delay in Crohn's disease

The interval between the onset of symptoms and confirmation of the diagnosis of Crohns disease in children is often many months or years. A number of factors contribute to this diagnostic delay, but the most significant appear to be delay in the patient seeking initial medical help and failure of the medical practitioner to consider the diagnosis at the time of the first medical contact. In this review of 55 children with Crohns disease the intervals between onset of symptoms, first presentation and diagnosis were examined and the causes of diagnostic delay determined. The main reason a diagnosis was not made at first presentation was because of ignorance of the fact that Crohns disease could occur in children. In older children, the combination of tiredness, lethargy and iron deficiency anaemia with vague or intermittent abdominal pain was a frequent cause of diagnostic difficulty. However, even in the absence of major gastrointestinal symptoms, the significance of growth failure and delayed puberty was usually recognised if the patient was referred to a paediatrician. Diagnostic delay could well be reduced if medical practitioners involved in the primary care of children were educated to recognise the various ways in which Crohns disease presents in this age group.

Retained common-duct stones after open cholecystectomy and duct exploration in children

Abstract. A retrospective study was performed of 250 patients with cholelithiasis treated at the Royal Childrens Hospital, (RCH) Melbourne, over 25 years by open operation; 32 (12.8%) had proven choledocholithiasis on either preoperative imaging, operative cholangiography (OpCG), or postoperative investigation. A further 3 had underlying congenital biliary abnormalities and were excluded from further study. Thirty-one of the 32 were explored at open operation, 27 after OpCG and 4 on clinical grounds. One retained common-bile-duct (CBD) stone was undetected until the postoperative period (1/250, 0.25%). Seven ducts were not cleared, giving a duct exploration failure rate of 22.6% (7/31). All 8 retained CBD stones were identified in the early postoperative period and managed with a variety of techniques, including endoscopic retrograde cholangio-pancreatography (ERCP). The incidence of retained stones after open CBD exploration was high (22.6%), and can be attributed to difficulties in operative technique dealing with the smaller paediatric CBD. In addition, haemolytic disease seems to induce a propensity for choledocholithiasis. Given the small numbers presenting with cholelithiasis to RCH (10 per year), it is suggested that a selective approach to CBD exploration is appropriate in children. With the increasing use of laparoscopic cholecystectomy in children and the inherent technical difficulties of laparoscopic operative cholangiography, ERCP may offer an alternative solution in dealing with CBD stones rather than open or laparoscopic CBD exploration.

Pancreatic pseudocysts in children

Pancreatic pseudocyst (PPC) is an uncommon condition in childhood and is almost always associated with blunt abdominal trauma. Additional disease within the pancreas is rare, unlike adult experience. Prior to the advent of ultrasonography (US) assessment of the cyst was difficult. Subsequently, it has become apparent that PPC may develop but still undergo spontaneous regression. We have reviewed eight children with PPC seen at the Royal Childrens Hospital between 1967 and 1987: six males and 2 females ranging in age from 2 to 12 years. The aetiology was blunt abdominal trauma in seven cases and idiopathic pancreatitis in one. Four children presented acutely and the diagnosis of PPC was made by US within 10 days of admission. One of these children had a diagnostic laparotomy for traumatic pancreatitis and his PPC was identified subsequently on serial US. The PPC in this child and one other resolved spontaneously after a period of total parenteral nutrition and intestinal rest. In two cases, the PPC failed to resolve and drainage was necessary. Late presentation (5–16 weeks) after recognised injury occured in four children; all required surgery within 1 week of admission. No surgical complications occured. Conservative management of PPC has been condemned because of the risk of complications occurring during the waiting period. This applies to adults who have developed a PPC in a pancreas diseased due to alcohol and/or cholelithiasis; the natural history of PPC is quite different in children. We thus recommend a trial of conservative treatment for all children who develop a PPC early, but consider that surgery is the best choice for those who present later.

Paediatric acalculous cholecystitis

Three cases of acalculous cholecystitis were found in a review of gall bladder disease over a 34-year period. Each case is presented and the literature is reviewed.