AriéL. Bensoussan

Aberrant cervical thymus in children: Three case reports and review of the literature

Aberrant migration of thymic tissue occurs with ectopic thymus in the mediastinum, base of the skull, tracheal bifurcation, and cervical region. A recent review of the literature showed a total of 76 reported cases of aberrant thymus or thymic cysts in patients who presented with primary neck masses. We report three additional cases of ectopic cervical thymus. All three patients presented with asymptomatic cervical masses, and preoperative diagnosis included branchial cleft cyst, cervical lymphangioma, and cervical teratoma. All patients underwent complete surgical resection of the masses. Aberrant cervical thymus rarely produces symptoms because it does not invade contiguous strictures. Despite its rarity, it should be considered in the differential diagnosis of asymptomatic neck masses in children.

Vascular complications after pediatric liver transplantation

From February 1986 to July 1994, 81 hepatic transplantations were performed in 73 children, with an overall patient survival rate of 83%. Forty-two patients received whole-liver grafts (WLG) and 39 had reduced-size grafts (RSG). The mean patient weight was 19.7 kg, with 29 patients weighing less than 10 kg. Seventeen vascular complications (21%) occurred in 13 children: 8 (10%) had hepatic artery thrombosis (HAT), 5 (6%) had portal vein thrombosis (PVT), 1 had both HAT and PVT (1%), and 3 (4%) had aortic conduit perforation (ACP). There was no significant difference in the incidence of HAT between RSG (5%) and WLG (14%) or between children weighing less than 10 kg (10%) and those weighing more than 10 kg (10%). The site of arterial reconstruction, end-to-end to the recipient common hepatic artery or end-to-side to the infrarenal aorta, had no significant effect on the occurrence of HAT (7% v 8%), but HAT occurred in 2 of 6 cases (33%) in which an aortic conduit was used. PVT documented in 5 cases (6%) was associated with technical complications (2), preduodenal portal vein (2), and a circulating cardiolipid antibody (1), and required thrombectomy, with no graft loss. Combined HAT and PVT was found in one patient 2 years postretransplantation for HAT. Although graft function is normal, portal hypertension persists. The aortic conduit, used in six patients, led to arterial perforation (3), HAT (2), and death (2). Of the 8 cases of HAT, 1 was diagnosed during autopsy and 7 occurred within 30 days and required retransplantation (6) or thrombectomy with rearterialization (1).(ABSTRACT TRUNCATED AT 250 WORDS)

Benign liver tumors in children: A 25-year experience

The most common benign liver tumors are hemangiomas and hamartomas, both of mesenchymal origin. Mortality for patients with these tumors has traditionally been substantial despite benign histology. Between 1965 and 1989, 22 patients were treated for a benign liver tumor. This represents 42% of all primary neoplasms of the liver observed during this period. Incidental findings of liver tumors at autopsy were excluded from this series. There were 9 boys and 13 girls with a mean age at presentation of 2.3 years (range, birth to 14 years). Sixteen had hemangiomas and presented earlier in life (mean age, 4.4 months). In this subgroup, high-output cardiac failure was present in 58% of the newborns. Seven hemangiomas were resected, four were observed, three were treated with digitalis, diuretics, and steroids, and one received epsilon-aminocaproic acid. Nonhemangiomatous tumors included four hamartomas, one focal nodular hyperplasia, and one nodular transformation. All six were resected. There was one death early in the series. At a mean follow-up of 38 months, 21 of the 22 patients are cured or asymptomatic. In the past, mortality rates of close to 90% have prompted many investigators to advocate resection of every symptomatic hemangioma. With the availability of more sophisticated imaging techniques and refinements in the treatment of cardiac failure, surgery can be used more selectively. Hepatic resections, once considered heroic, can now be performed with minimal morbidity and virtually no mortality. The 96% survival in this series of benign liver tumors contrasts with high mortality rates reported in the literature and illustrates the spectacular improvements that have been made in the diagnosis and management of these once ill-reputed tumors.

Neonatal Gastrointestinal Perforations

Neonatal gastrointestinal perforation has been associated with mortality rates of 40% to 70%. Over the past 20 years, 81 infants (46 boys and 35 girls) were treated for a gastrointestinal perforation at this institution. Perforation occurred from birth to 50 days (average, 8.2 days). Etiologies included necrotizing enterocolitis (NEC) (68%), meconium ileus (10%), and idiopathic gastric perforation (7%). Seventy-six infants underwent surgical exploration and five infants, considered too small or too sick to withstand a laparotomy, were treated with peritoneal lavage only. There were 29 deaths, an overall mortality of 36%. Ninety percent of the death occurred in patients with NEC, while all patients with gastric perforations survived. There has not been a significant improvement in survival in recent years, partly because of an increase in the proportion of NEC-related perforations. However, there is a narrowing of the mortality gap between low birth weight and normal weight infants. As the risk inherent to laparotomy in neonates is decreasing, other factors, such as the underlying etiology or the site of perforation, play a more important prognostic role.

Infectious complications of pediatric liver transplantation

Twenty-five pediatric orthotopic liver transplantations (OLTs) performed in 22 patients at Sainte-Justine Hospital were reviewed for infections complications. One patient died within 12 hours posttransplantation and is excluded. The patients had an average age of 6.1 years (range, 1.25 to 19 years) and an average weight of 20.4 kg (range, 11 to 55 kg). Two patients (9%) were cytomegalovirus (CMV) seropositive and 9 of 19 patients (48%) were Epstein-Barr virus (EBV) seropositive preoperatively. Five of the donors (20%) were CMV seropositive. The most common indications for OLT were biliary atresia (8) and tyrosinemia (7). There were 4 deaths, for an overall mortality rate of 19%. In 3 patients, deaths were related to infection (CMV hepatitis and duodenitis with aortoduodenal fistula, adult respiratory distress syndrome [ARDS] with Streptococcus viridans pneumonia, Escherichia coli cholangitis with progressive hepatic failure). Fifteen patients (72%) had 41 major infections, most of them bacterial, during the first month posttransplantation. These include pneumonia (25%), line sepsis (17%), cholangitis (14%), and tracheitis (14%). There was only one major viral infection, a CMV hepatitis that occurred in the first month posttransplantation. Three patients had fungal infections (8%) associated with hepatic artery thrombosis and recurrent cholangitis. All three patients required retransplantation. There was only one protozoal infection (Pneumocystis carinii pneumonia) causing life-threatening respiratory failure, from which patient recovered without sequelae. Infection still remains a serious complication of OLT. Bacterial infection is common and is usually associated with technical complications. The low rate of CMV infection is related to low incidence of CMV in the donor pool and the minimal use of strong immunosuppressants.

Results and complications of toupet partial posterior wrap: 10 years' experience☆

A 270 degrees posterior wrap, described by Toupet, was performed in 112 patients with medically refractory gastroesophageal reflux (GER) over a 10-year period. The mean age was 39 months (range, 2 months to 19 years). Thirty percent of the patients were neurologically impaired (NI). The approximation of the crura, the posterior fixation of the wrap, and the posterior partial fundoplication were performed with nonabsorbable sutures, over a bougie. Thirty percent of the NI and 15% of the neurologically normal (NN) children underwent a gastrostomy. Early postoperative complications were found in 24% of NI and 18% of NN children; these were treated medically, except for two bowel obstructions. Eight patients died for reasons unrelated to surgery, and nine were lost to follow-up. The mean follow-up period for the 95 remaining patients was 3.5 years for NI and 4.9 years for NN children. The evaluation showed that 6 patients had temporary dysphagia and two had food impaction. Nine wrap herniations (10%) were found; three of them without symptoms were noted by routine upper gastrointestinal series and received no treatment. Six wrap herniations with recurrent reflux were reoperated on successfully. With 90% of patients free of symptoms, the results of the Toupet procedure compare favorably with those of the Nissen fundoplication because of the retained ability to belch or vomit.

Biliary tract complications in pediatric orthotopic liver transplantation

Biliary tract complications are reported in 15% to 30% of orthotopic liver transplantations (OLTs). Since 1986, 53 OLTs were done in 48 children with a mean age and weight of 5.3 years and 18.9 kg, respectively. Twenty-seven transplantations (51%) were reduced liver grafts (RLG) and 26 (49%) were whole liver grafts (WLG). Since 1988, 70% of transplantations have been RLG. Choledochocholedochostomy (mean weight, 25 kg) with a T-tube (CC) or choledochojejunostomy (CJ) (mean weight, 14.5 kg) were done in 24 (45%) and 29 (55%) cases, respectively. The overall mortality was 19% but none of the deaths were related to biliary problems. There were 13 biliary tract complications (24.5%) in 11 patients including 7 leaks, 5 obstructions, and 1 intrahepatic biloma. Leaks leading to bile peritonitis were managed with simple suture and drainage and were related to the T-tube (4), to the Roux-en-Y loop (2), and to the transection margin of a RLG (1). Obstruction was documented in 5 cases with none associated with hepatic artery thrombosis (HAT). Stenosis after CC reconstruction (2) required conversion to CJ. Two patients had revision of CJ because of kinking of the common bile duct after a left lateral segment graft and an anastomotic stricture 46 months after OLT. The last patient developed a vanishing bile duct syndrome 4 months posttransplant and is awaiting retransplantation. One patient had multiple episodes of cholangitis after HAT and was retransplanted. Neither the type of grafts (RLG 25.9% v WLG 23.1%) nor the type of biliary reconstruction (CC 25% v CJ 24%) influenced the rate of biliary complications.(ABSTRACT TRUNCATED AT 250 WORDS)

Pneumatosis and pneumoperitoneum in chronic idiopathic intestinal pseudoobstruction.

Chronic idiopathic intestinal pseudoobstruction is a diagnosis of exclusion for the rare patient with severe small bowel atony without any demonstrable organic cause. The very poor prognosis associated with this disease has been somewhat improved with the advent of parenteral nutrition; nevertheless, these patients follow a stormy course characterized by malnutrition, recurrent obstruction, infection, hemorrhage, and perforation. We describe a 16-year-old boy with this disease who presented to us with vague abdominal pain and pneumoperitoneum. Laparotomy showed pneumatosis cystoides intestinalis without intestinal perforation. The presence of free air in the abdominal cavity is an almost pathognomonic sign of intestinal perforation. Pneumatosis cystoides intestinalis is one of the very few nonsurgical causes of pneumoperitoneum. Beyond the neonatal period, in which it is the hallmark of necrotizing enterocolitis, it has been described in patients with severe obstructive lung disease, in collagenous disorders, and in the short-bowel syndrome. In the present case, it may have resulted from chronic intestinal distension, allowing air under pressure to dissect through the bowel wall. The challenge of such a unique association of conditions lies in the avoidance of unnecessary surgery in a chronic, essentially nonsurgical disease while avoiding unnecessary delay in a possibly acute surgical event.

Anticoagulation without catheter removal in children with catheter-related central vein thrombosis

Catheter-related central venous thrombosis is a serious and common problem among children. The traditional management has been anticoagulation and early catheter removal. Unfortunately, many patients require a new catheter, which is associated with complications that include possible further thrombosis. Although others have used thrombolytic agents in attempts to avoid catheter removal, the authors of the present study believe that the associated complications occur too frequently and are too serious. They have had success with standard anticoagulation in a limited number of patients. Between February 1991 and April 1994, 17 patients (6 weeks to 19 years of age) were treated for catheter-related deep venous thrombosis. Eight patients underwent early catheter removal accompanied by anticoagulation; two of them had intrinsic catheter problems that necessitated removal, and one had hemophilia. Nine others received anticoagulation without catheter removal. Of these, one required catheter removal after 10 days heparin administration failed to diminish the thrombosis. Another patient responded well to anticoagulation but required catheter removal several weeks later because of catheter-site infection. The other seven patients responded well to anticoagulation, and their catheters were retained. For patients with a functional catheter essential to their care, anticoagulation may safely prevent catheter removal.

Pediatric liver transplantation: The montreal experience

The introduction of cyclosporine A in 1980 greatly improved the survival of children with end-stage liver disease undergoing orthotopic liver transplantation. The average 1-year survival rate following hepatic transplantation increased from 30% in 1963 to 70% in 1980. This report summarizes the initial experience of two pediatric hospitals in Montreal--Hôpital Ste-Justine and Montreal Childrens Hospital. Since December 1985, 13 orthotopic liver transplantations have been performed in 11 patients: six females and five males. The median age was 18 months (range, 13 months to 17 years) and the median weight was 10 kg (range, 8.5 to 38 kg). The indications for transplantation were biliary atresia (5 patients), tyrosinemia (2 patients), biliary hypoplasia (1 patient), Amerindian cirrhosis (1 patient), Crigler-Najjar syndrome, type I (1 patient), and fulminant non-A, non-B hepatitis with grade IV encephalopathy (1 patient). Immunosuppression was ensured by cyclosporine A, azathioprine, and steroids. The function of 11 grafts was immediate following revascularization of the grafts. One graft had delayed function due to preservation injury, and one had primary nonfunction. Surgical complications included bile peritonitis (1), ruptured aneurysm of an aortic conduit (1), and thrombosis of the hepatic artery necessitating retransplantation (2). Three of our patients died, one from postoperative cerebral edema, one of primary nonfunction of the graft, and one of ruptured aneurysm of an aortic conduit. Our overall survival rate was 72% with a follow-up of 3 to 32 months. Pediatric liver transplantation can now provide successful treatment and cure of liver diseases considered, until recently, dismal and hopeless.