B.J. Hancock

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.

Childhood primary pulmonary neoplasms

Nine children (6 boys, 3 girls) were diagnosed with a primary endobronchial or pulmonary parenchymal neoplasm. The average age at diagnosis was 9 years. Presenting complaints included cough (7), fever (5), pulmonary infection (3), respiratory distress (3), weight loss (2), pain (2), and hemoptysis (1). Pulmonary x-rays showed persistent atelectasis, pneumonic infiltrates or mass lesions. A computed tomography scan was performed in 8. Five of six endobronchial tumors were diagnosed with bronchoscopy and biopsy. Treatment consisted of thoracotomy and pulmonary resection in 7 cases and laser resection in 2. The pathologic diagnoses were bronchial carcinoid (3), bronchial mucoepidermoid carcinoma (1), inflammatory pseudotumor (plasma cell granuloma) of the bronchus (2) and of the lung parenchyma (1), fibrosarcoma (1), and rhabdomyosarcoma (1). Postoperative chemotherapy was given only to the patient with pulmonary rhabdomyosarcoma; this child died. One child has developed a local recurrence while 7 children are alive and free of disease at an average of 2.4 years postresection. Pulmonary neoplasms are unusual in the pediatric age group and represent a wide spectrum of pathology. Including the present series, 383 tumors have been described. Seventy-six percent were malignant. Early investigation and surgical intervention are essential in children with persistent pulmonary symptoms or x-ray abnormalities. In most cases, the prognosis is excellent with complete surgical resection; however, malignancies other than bronchial adenoma are associated with significantly mortality.

Early Analgesia for Children With Acute Abdominal Pain

Objectives. The objectives of this study were to determine whether the administration of morphine to children with acute abdominal pain would impede the diagnosis of appendicitis and to determine the efficacy of morphine in relieving the pain. Methods. This was a double-blind, randomized, placebo-controlled trial involving 5- to 16-year-old children who presented to the emergency department of a children’s hospital with a chief complaint of acute abdominal pain that was thought by the pediatric emergency attending physician to require a surgical consultation. Subjects were randomized to receive intravenously administered morphine or normal saline solution. Clinical data and the emergency physician’s confidence in his or her clinical diagnosis (0–100%) were recorded systematically with a standardized form. This was repeated 15 minutes after administration of the study medication. The surgeon assessed the child within 1 hour and completed a similar data collection sheet. Pain was assessed, with a color analog scale, before and after study medication administration. Each subject was monitored for 2 weeks after enrollment. Results. One hundred eight children were enrolled; 52 received morphine and 56 received a placebo saline solution. There were no differences between groups in demographic variables or the degree of pain. There were no differences between groups in the diagnoses of appendicitis or perforated appendicitis or the number of children who were observed and then underwent laparotomy. The reduction in the mean pain score was significantly greater in the morphine group (2.2 vs 1.2 cm). The emergency physicians’ and surgeons’ confidence in their diagnoses was not affected by the administration of morphine. Conclusions. Our data show that morphine effectively reduces the intensity of pain among children with acute abdominal pain and morphine does not seem to impede the diagnosis of appendicitis.

Tracheobronchial injuries in children.

Five patients with tracheobronchial injuries secondary to blunt thoracic trauma were reviewed over a 9-year period. Bronchial disruption occurred in four cases and tracheal disruption in one. Of the four patients with bronchial disruption, a major airway injury was suspected early because of a large air leak or persistent pulmonary atelectasis. However, definitive diagnosis by bronchoscopy was delayed from 4 to 16 days due to initial response to conservative management. Bronchial repair was achieved in every case: additional lobectomy was required in only one instance. Postoperative bronchial stenosis occurred in one patient and responded well to dilatation. The child with a blowout perforation of the trachea was diagnosed early by bronchoscopy and was successfully managed without surgery. Tracheobronchial injury is one of the most severe injuries caused by blunt trauma and requires a high index of suspicion for early diagnosis and surgery. Bronchial repair is successful in most instances.

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.

Congenital duodenal obstruction: The impact of an antenatal diagnosis

In a series of 34 infants with congenital duodenal obstruction, 15 were diagnosed by antenatal ultrasound between the fifth month of gestation and term. Fourteen patients were available for follow-up. In 10 of 14 cases, patients were available for follow-up. In 10 of 14 cases, parents were informed of the diagnosis antenatally and, in 9, were advised of the possibility of surgery in the neonatal period. Parents felt the information helped them prepare for the surgical and medical interventions necessary in the postnatal management of their infants. The mean birth weight and gestational age of these infants were comparable with those diagnosed postnatally. Associated congenital anomalies were more common in the patients with a postnatal diagnosis. In the total group of 34 infants with duodenal obstruction, 32 had surgical repair with a survival rate of 94%. Surgical procedures were comparable between the two groups. Surgical intervention occurred earlier in the neonatal period when an antenatal diagnosis was made. Overall survival in the entire series was 88% with four deaths attributed to severe associated congenital anomalies. We conclude that (1) the antenatal diagnosis of duodenal obstruction influences parents positively in coping with the anomaly, and (2) although surgery was performed sooner, the outcome of infants with duodenal obstruction was not changed by providing an antenatal diagnosis.

Incarcerated congenital lumbar hernia associated with the lumbocostovertebral syndrome

Congenital lumbar hernias are uncommon, with only thirty-three reported cases. They have been associated with the lumbocostovertebral syndrome. This report describes a patient with a congenital lumbar hernia and manifestations of the lumbocostovertebral syndrome. The diagnosis of the hernia was delayed due to its juxtaposition to a posterior meningomyelocele, resulting in incarceration. The patients course was also complicated by recurrence of the lumbar hernia requiring prosthetic repair.

Lethal short-bowel syndrome

Infants with short-bowel syndrome are difficult to manage. Despite supportive measures with parenteral nutrition and surgery to lengthen remaining bowel or increase functional absorptive surface area, the outcome for many of these infants is poor. We have reviewed a series of seven infants diagnosed with severe short bowel. Causes included volvulus (3), multiple atresias (2), and total intestinal aganglionosis (2). Survival time ranged from 15 days to 8 months. During the hospital course, each infant underwent one to three operative procedures to diagnose and manage the short bowel and all received total parenteral nutrition (TPN) ranging from 10 days to 6 months. One infant died of liver failure and two others developed significant liver dysfunction secondary to TPN. Most infants remained hospitalized until their death. Death occurred at an average of 9 weeks following the diagnosis of short-bowel syndrome. This review suggests that infants with less than 6 cm of small bowel beyond the Ligament of Treitz will inevitably die of their disease or treatment complications. Until bowel transplant becomes a viable alternative, operative intervention and nutritional support may prolong survival but will not change the outcome of these infants and will only contribute to additional morbidity. A decision to withhold further therapy would be reasonable at the time the diagnosis is established.

Surgical and metabolic aspects of liver transplantation for tyrosinemia.

Tyrosinemia represents a very small percentage of patients undergoing liver transplantation world-wide. This disease is endemic within our referral area however, one-third of the liver transplantations at our institution are done for this disease. Since 1986, 16 patients with tyrosinemia and 34 patients with various other indications (non-tyrosinemic) have undergone a total of 55 liver transplantation. The survival rate for tyrosinemic patients is 87%, compared to 75% for non-tyrosinemic patients. Liver transplantation for hereditary tyrosinemia and other metabolic disorders without portal hypertension or previous portohepatic operations is notably easier to perform. Intraoperative blood loss was less, length of hospital stay was shorter and incidence of infections was lower in tyrosinemic than in non-tyrosinemic patients. Less than 10% of tyrosinemic patients had foci of hepatocellular carcinoma at the time of transplantation. For this reason, and while most patients with tyrosinemia will eventually require liver transplantation, our results do not support systematic early transplantation.

The development of a national pediatric trauma curriculum

Background: Educational programs dedicated to pediatric trauma are either not available or comprehensive. Pediatric trauma is thus managed by a range of specialists with training in a variety of related fields. Post-certification fellowships in pediatric medicine all mandate education in the assessment and management of the injured child. The purpose of this study was to develop a blueprint for a national pediatric trauma training curriculum. Methods: A team of four experts developed content for a national pediatric trauma curriculum and disseminated it to 11 pediatric trauma sites across Canada. The objectives contained both knowledge and skill sets related to the management of the pediatric trauma patients. A multi-tiered Delphi process was used to develop the final content. Results: All the 11 pediatric teaching centers across the country participated. A final list, representing a consensus of views, was developed in 10 domains through the iterative process of the Delphi technique. The domains for the curriculum included introduction to pediatric trauma and epidemiology, initial management, pediatric airway, shock, thoracic injuries, abdominal and pelvic injuries, spinal and neurological injuries, pediatric head injuries, burns and electrical injuries, and orthopedic injuries. Conclusion: The Delphi process is an invaluable tool in developing curricula. The pediatric trauma curriculum can be used in teaching hospitals for house staff education and meeting core competencies. The blueprint can be validated further in the future.