Subcutaneous infusion ports in the pediatric patient with hemophilia.

Central venous access devices have become important tools in the management of pediatric patients with malnutrition, malignancy, and infections requiring long-term antibiotic treatment. Hemophilia presents a lifetime challenge for venous access and at times can be an urgent or life-threatening situation. Since 1986, the authors have implanted 11 subcutaneous infusion ports in nine patients with hemophilia. The systems have remained in place for up to 7 years, without major complications or problems. Two catheters were replaced, after 4 and 6 years, because of skin erosion and blockage, respectively. One catheter was removed after 7 years because of blockage following local trauma and was not replaced. A recent survey through the Canadian Hemophilia Centre Directors Group obtained a further 45 subcutaneous infusion ports in other centers across Canada. The benefits of this system are overwhelming enthusiasm by the parents and children and no major complications. Some of the patients are now HIV-positive and are able to use their system for ongoing drug therapy.

Biliary tract reconstruction in liver transplantation.

Refinements in biliary tract reconstruction and the frequent use of cholangiography have produced a marked decline in the number of deaths from biliary complications after liver transplantation. The authors' method of reconstruction differs from those of others in that it employs no stents or T tubes and retains the donor gallbladder, allowing access to the biliary tract for radiologic purposes in the post-transplant period. In a series of 161 consecutive liver transplants, the frequency of biliary complications was 13.6% (15 anastomotic and 7 gallbladder-related). Of three deaths that occurred in patients with biliary complications, one was due to the complication itself. A Roux-en-Y reconstruction with anastomosis to the donor duct was associated with the lowest anastomotic complication rate (2.2%). Upper abdominal surgery before transplantation, especially shunting, was a major risk factor for biliary complications.

Staging laparotomy for Hodgkin's disease in children.

Staging laparotomy in patients with Hodgkin's disease continues to be a controversial procedure in their management. Between 1970 and 1986, 67 patients up to 18 years of age were seen with Hodgkin's disease. The results of staging laparotomy performed on 39 of these children are reviewed. The clinical stage was changed as a result of laparotomy in 43.6% of cases, with 12.8% of cases upstaged and 30.8% of cases downstaged. All changes in stage modified the proposed treatment for the patient. In 20.5% of patients the laparotomy was positive, and in all cases the spleen was involved. Preoperative lymphangiography did not accurately identify nodal disease. Of the patients with negative laparotomies, 10% developed relapse in the abdomen. Major complications included three episodes of bacterial sepsis, with one death due to Streptococcal pneumonia and one to Neisseria gonorrhea. All septic events occurred prior to the use of pneumococcal vaccine and prophylactic antibiotics. One patient required reoperation for intestinal obstruction with bowel resection. None of the currently used noninvasive tests accurately identifies intraabdominal disease. Therefore, staging laparotomy continues to play an important role in the early management of Hodgkin's disease.

Fibrous hamartoma of infancy.

Fibrous hamartoma of infancy is an uncommon, benign, subcutaneous fibrous proliferation, usually found during the first 2 years of life. It predominantly affects healthy boys and can be found in almost any subcutaneous tissue. This tumor can cause much concern about malignancy because it is firm and may be fixed to underlying tissues. Despite the occasional local recurrence, the clinical course is benign and the prognosis is excellent. Treatment is by local excision. The diagnosis is made easily by the characteristic histologic features. In order to avoid the misdiagnosis of malignancy and unnecessary radical therapy, both surgeon and pathologist must be familiar with this entity. We have reviewed six cases of fibrous hamartoma of infancy seen over a 5-year period.

Liver transplantation: the University Hospital-Children's Hospital of Western Ontario experience.

Patients who currently benefit the most from liver transplantation are those with end-stage, non-malignant liver disease. Primary biliary cirrhosis and cirrhosis from chronic active hepatitis (hepatitis B negative) have been the most common indications in our experience. Overall survival rates in excess of 70% at 1 year are now common and those patients who live the first year have a very good prospect of long-term survival. Complete rehabilitation occurs in about 80% of survivors. Patients on life support systems before transplantation and those awaiting urgent retransplantation have the highest mortality rates. Modern anesthetic and surgical techniques have made the operation much safer and more straightforward. Biliary tract complications remain common, especially in patients with a history of previous upper abdominal surgery. Cyclosporine has had a major impact, but in the context of its use in combination with other immunosuppressive agents (antilymphocyte globulin, steroids, azathioprine and OKT3).

Pediatric implications of multiple endocrine neoplasia.

The association of endocrine tumors from several sites has been known for over 50 years but the familial aspects of these relationships have only been appreciated since 1954. The original term multiple familial endocrine adenomatosis (MEA) was changed to multiple endocrine neoplasia (MEN). This report describes two children aged 8 and 11 years, who are cousins with MEN IIa. A strong family history prompted investigation of these children. Pentagastrin stimulation resulted in elevated serum calcitonin levels and subsequent surgery. Unsuspected medullary thyroid carcinoma was found in each child. Proper screening of high-risk individuals should prevent this potentially lethal condition from becoming a major problem.

Midgut volvulus in the adult. A complication of intestinal malrotation.

We report two cases of abnormalities in the second and third stages of intestinal rotation and fixation which presented in adulthood as recurrent midgut volvulus. We review the embryology, symptoms, diagnosis, and treatment of this problem.

Radionuclide scintigraphy: a valuable diagnostic aid in children with splenic trauma.

Preserving the spleen in children with splenic trauma remains a primary concern of surgeons. Nonoperative management has been encouraged in recent years, but this must be balanced against the risk of delayed rupture of the spleen and failure of diagnose other causes of intra-abdominal bleeding. The author reviews his experience over a 10-year period with 36 children who had a diagnosis of ruptured spleen. The role of radionuclide scintigraphy of the spleen has been reviewed recently with particular reference to its influence on the need for laparotomy. The indications for laparotomy and the management of splenic injuries are discussed. In the author's opinion, radionuclide scintigraphy is a valuable diagnostic aid in children with suspected splenic trauma.

Anaesthesia induced rhabdomyolysis--a case report.

Anaesthesia-induced rhabdomyolysis (AIR) is a rare but serious complication of general anaesthesia. We report the occurrence of this event in a previously healthy three-year-old male, with a strongly positive family history of Duchenne muscular dystrophy. Following an uneventful anaesthetic, which included succinylcholine, myoglobinuria developed and led to renal failure which fortunately reversed with conservative treatment. Based on review of reports of similar cases, it is clear that succinylcholine should be avoided in paediatric patients with known myopathy or at high risk for latent myopathy.

Radiotracer assessment of gastric mucosal permeability after hypovolemic shock.

Acute lesions of the gastroduodenal mucosa may result from gastric mucosal ischemia with subsequent cellular dysfunction, capillary leakage and increased susceptibility to mucosal damage. A new technique for measuring the effect of hypovolemic shock and gastric mucosal permeability is presented. Piglets were injected intravenously with human serum albumin labelled with 100 micro Ci of iodine-131 (131I-HSA). Serial blood and gastric samples were obtained for gamma counting during the control, hypovolemic shock and resuscitative periods. Increased gastric acid secretion and clearance of labelled serum albumin occurred after resuscitation from hypovolemic shock. The authors believe that increased capillary permeability and defective gastric mucosal cell function due to ischemia contribute to these changes.

Staging laparotomy for Hodgkin's disease in children.

Resort to laparotomy for the staging of Hodgkin's disease has been controversial because of its questionable advantage over nonsurgical staging methods. The recent concern over splenectomy and subsequent overwhelming infection has added to this debate. The author reviews experience with Hodgkin's disease in 34 patients whose ages ranged from 6 to 18 years. Seventeen patients underwent staging laparotomy after their disease had been staged by standard nonoperative methods; the duration of follow-up was from 2 to 7 years. In 7 of these 17 patients the stage of their disease was changed as a result of the laparotomy findings. Complications have been late septicemia resulting in death in one patient and subacute bowel obstruction not requiring reoperation in two patients. In the author's opinion staging laparotomy in children with Hodgkin's disease is a valuable means of deciding on their subsequent therapy.

Twenty-four hour gastric acidity after vagotomy.

Twenty-four hour intragastric pH during normal daily activity has been studied by nasogastric intubation and aspiration of gastric samples in seven patients four years after vagotomy (four truncal, three selective). Two of these patients also had pre- and early postoperative studies. Mean pH was inversely correlated with basal, insulin- and pentagastrin-stimulated acid outputs. However, one of four patients after truncal vagotomy with drainage, and two of three after selective vagotomy and drainage had most of their hourly intragastric pH values more acidic than pH 3.5 despite more than 70% reductions in preoperative stimulated-acid outputs.