Dickens St-Vil

Complications of lymphangiomas in children.

One hundred ninety-three cases of lymphangioma presenting in children were reviewed over a 10-year period. The diagnosis was established before 5 years of age in 87.0%. The distribution of lymphangiomas was: cervical (31.4%), craniofacial (18.9%), extremity (18.9%), trunk (9.2%), intraabdominal (9.2%), cervicoaxillothoracic (4.9%), multiple (3.8%), cervicomediastinal (2.2%), and intrathoracic (1.6%). Of 164 patients undergoing primary therapy, total excision was performed in 77.4% and partial excision in 20.7% with recurrence rates of 11.8% and 52.9%, respectively. Thirty-eight percent of all operations for lymphangiomas were performed for resection of recurrent or residual disease. The incidence of postoperative complications was 31.3%. Forty-four additional procedures were required to manage these complications. Sclerotherapy with 50% dextrose was used as an adjunct in 9.5%. Recurrences were seen after total resection in 17.6% of operations in which 50% dextrose was used and 11.8% in which it was not used. Seroma formation was noted in 3.5% of total resections and 40.0% of resections for recurrent disease in which adjunctive sclerotherapy with 50% dextrose was used. Seromas occurred in 9.8% of wounds in which local drains were used and 3.6% of wounds in which drains were not used. Infectious complications occurred after 6.6% of operations using perioperative antibiotics and 3.5% not using antibiotics. The management of lymphangiomas in children remains a difficult problem. Despite being benign lesions, lymphangiomas are attended by a high rate of morbidity from complications of the disease and its management. Complete excision still offers the best chance for complete cure. Sclerotherapy with 50% dextrose is not beneficial in the management of recurrent disease or postoperative seromas. Local drains and perioperative antibiotics do not appear to diminish the incidence of seromas and infectious complications, respectively.

Meckel's Diverticulum in Children: A 20-Year Review

Meckels diverticulum occurs in 2% of the population and may present at any age. Its management, when found incidentally at laparotomy, remains controversial, particularly in the pediatric population. From 1970 to 1989, a Meckels diverticulum was discovered in 164 children at laparotomy. There were 120 boys and 44 girls with a mean age of 5.2 years (range, 0 to 18 years). Forty-seven cases were asymptomatic, representing an incidental finding at laparotomy, 25 were resected, and ectopic gastric mucosa was present in 7 specimens (28%). Three postoperative deaths (6%) that were not related to the resection and 2 complications (4%) (postoperative leak and wound infection) occurred in this group. Of the 117 symptomatic patients, 49 (42%) presented with bowel obstruction, 45 (38%) had rectal bleeding, 16 (14%) had diverticulitis, and 7 (6%) had umbilical pathology. Volvulus (20) and intussusception (19) were the most common causes of obstruction. Predisposing factors for bowel obstruction were fibrous bands to umbilicus or mesentery (37%) and ectopic mucosa (35%). Severe painless rectal bleeding occurred in 45 patients, 30 of whom (67%) required blood transfusion. A nuclear medicine Meckel scan was positive in 32 of 37 patients (85%). Contrast studies were not diagnostic; colonoscopy and gastroscopy ruled out other causes of bleeding. Patients with diverticulitis (16) presented with acute abdominal pain compatible with appendicitis. In the symptomatic group, ectopic mucosa was present in 61% of the resected specimens. Gastric (88%), pancreatic (7%), and gastric with pancreatic (3%) were the most common ectopic tissue. Postoperative morbidity and mortality for symptomatic patients was 8.5% and 0%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

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)

Airbags and children: A spectrum of C-spine injuries☆

Over 30 children who were improperly restrained or in rear facing safety seats have been reported killed in motor vehicle accidents (MVA) involving airbags. The authors report one minor and two major injuries in properly restrained children in the front passenger seat. In case 1, A 10-year-old seat-belted boy was involved in an MVA (40 km/h) with deployment of both airbags. Physical examination findings showed right hyphema with corneal abrasion, right cheek abrasion and minimal cervical tenderness. C-spine x-ray was normal. He was treated for whiplash and facial burns resulting from contact with hot gas released by the airbags and discharged. In case 2, a 4-year-old boy wearing a lapbelt was in a MVA (20 km/h) with airbag deployment. On arrival, his Glasgow coma score was 3 and he was hemodynamically unstable. Secondary survey after stabilization showed left neck abrasions and ecchymoses, quadraplegia, priapism, and absent rectal tone. C-spine x-ray showed atlanto-occipital dislocation with possible complete spinal cord transection at C1. Aggressive maneuvers were withheld, and the patient was pronounced dead. Autopsy findings confirmed the clinical diagnosis. In case 3, a 3-year-old boy in a forward-facing safety seat was in a MVA (60 km/h) with air bag deployment. The patient was fully awake. C-spine x-rays were normal. Because of fluctuating level of consciousness, he underwent head computed tomography (CT) scan, which demonstrated a posterior fossa subarachnoid hemorrhage and a hematoma posterior to the odontoid, suggesting a ligamentous tear. He remained asymptomatic and was discharged on day 6. A head CT scan at 1 month showed a periosteal reaction in the area of the alar ligament suggestive of partial ligamentous avulsion; this injury was the forerunner of atlanto-occipital dislocation. Airbags deploy by releasing a hot effluent at 300 km/h. Mechanisms of injury include direct contact of hot gas with facial skin and energy transmitted directly from the airbag system to the childs head and neck. These cases illustrate a spectrum of C-spine injuries caused by airbag deployment and support the recommendation that children under 12 years of age travelling in a car equipped with dual airbags be seated in the back.

Mesenteric cysts in children

Mesenteric cysts are rare intraabdominal lesions of childhood that may vary in presentation from an asymptomatic mass to an acute abdomen. From 1970 to 1990, 15 children were diagnosed and treated for mesenteric cysts at Ste Justine Hospital in Montreal. The ages ranged from birth to 18 years (average age, 6 years). There were 9 boys and 6 girls. Ten patients required emergency surgery and five underwent elective surgery. The main presenting symptom was abdominal pain. Ten patients had preoperative ultrasounds that were diagnostic for a cystic mass in all patients. The second most frequent preoperative diagnosis was appendicitis. The cysts were located in the small bowel mesentery in 5 cases, the base of the mesentery with retroperitoneal extension in 4 cases, the transverse mesocolon in 4 cases, and the gastrocolic ligament in 2 cases. Operative procedures performed included complete cyst excision (9 patients), complete excision with intestinal resection (5 patients), and drainage of the cyst (1 patient). The only recurrence in this series occurred after drainage. One other patient had recurrence of a mesenteric cyst following resection performed elsewhere. Mesenteric cysts are rare in children, are usually symptomatic, and are most commonly misdiagnosed as acute appendicitis. Accurate preoperative diagnosis is possible with current ultrasonographic imaging techniques. Complete cyst resection is the procedure of choice and results in an excellent outcome.

Cholelithiasis in newborns and infants

Cholelithiasis in infants is rare, and has usually been associated with hemolysis, ileal disease, congenital anomalies of the biliary tree, hyperalimentation, and prolonged fasting. With the increased use of abdominal ultrasonography (US), more cases of cholelithiasis are being discovered. We report our experience with 13 infants diagnosed on abdominal US to have gallstones. There were 9 boys and 4 girls with an average age at diagnosis of 2.6 months (range, 0 to 9 months). Predisposing factors could be identified in only 6 of the 13 patients. Two patients with obstructive jaundice underwent cholecystectomy and common bile duct exploration. One patient with choledocolithiasis and common bile duct dilatation was observed. His stone passed spontaneously, with resolution of symptoms. Ten patients without cholestasis remained asymptomatic, with disappearance of lithiasis in five of them. Neonatal cholelithiasis is more common than previously suspected; it seems to affect males more often than females and is usually not associated with known predisposing factors. It appears to be a temporary, self-limiting phenomenon, and an aggressive approach is not warranted in the asymptomatic infant. Surgical or radiological intervention should be reserved for the symptomatic patients or those with underlying lithogenic disorders.

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.

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)

Type A esophageal atresia: a critical review of management strategies at a single center

PURPOSE The purpose of was to study the short- and long-term outcomes in the management of isolated esophageal atresia with different operative strategies. METHODS All patients undergoing type A atresia repair over a 15-year period were included. Demographic data, birth weight, gestational age, incidence of associated anomalies, management, and long-term outcomes were studied. RESULTS Fifteen patients with type A atresia (9 male) were treated in the study period. The mean gestational age was 35.5 weeks (range, 27-39 weeks), and the mean birth weight was 2179 g (range, 670-3520 g). Eight babies had associated anomalies. Thirteen patients underwent gastrostomy as the initial procedure, and 2 underwent the Foker procedure. In the delayed management group, 9 patients underwent primary anastomosis, with 2 patients needing proximal pouch myotomy. Two patients underwent a Collis gastroplasty. Two patients underwent a cervical esophagostomy and a gastric tube replacement at 4 months and 1 year, respectively. Eight patients (60%) in this group had anastomotic leaks. All patients are currently on prokinetics and proton pump inhibitors. Seven required antireflux surgery. The median length of hospital admission was 4 months (range, 3-19 months). The native esophagus was preserved in 13 (85%) of 15 babies. All patients are alive, and 14 of 15 are capable of feeding orally. CONCLUSIONS Type A esophageal atresia continues to be associated with significant morbidity despite advances in surgical technique and intensive care.

Scrotoschisis Associated With Contralateral Meconium Periorchitis

Scrotoschisis, a congenital defect of the scrotal wall associated with extracorporeal testicular ectopy, has been previously reported only twice. Meconium periorchitis is another rare scrotal anomaly indicative of an antenatally healed gastrointestinal perforation. The authors present a third case of scrotoschisis and the first associated with meconium periorchitis. Several hours after birth of an otherwise-normal term baby boy, a scrotal exploration was performed with orchidopexy and primary closure of the scrotal wall defect. At 4 months of age the baby underwent a contralateral inguino-scrotal exploration with excision of a paratesticular mass of calcified meconium. The role of a normally developed scrotum in testicular descent and causes of calcified scrotal masses in infants are discussed.