Surgical management of Wolff-Parkinson-White syndrome in infants and small children

Surgical ablation of accessory conduction pathways has rarely been reported in infants and small children with Wolff-Parkinson-White syndrome. In the interval January 1985 to September 1988, 19 infants and children aged 5 or younger have undergone surgical ablation of accessory conduction pathways because of recurrent supraventricular tachycardia. There were 12 (63%) boys and seven (37%) girls. Age ranged from 4 to 66 months (mean 33.8 months). Nine infants were less than 24 months old. Weight ranged from 5.5 to 2.16 kg (mean 13.2 kg). All 19 patients had symptoms, with duration of symptoms ranging from 3 to 63 months (mean 21 months). Accessory conduction pathways were classified preoperatively as left free wall in four (21%), right free wall in nine (47%), and posterior septal in six (32%). No multiple pathways were recognized preoperatively. Left ventricular function was abnormal in four (21%) preoperatively. Free wall pathways (n = 13) were surgically dissected and septal pathways (n = 6) were cryoablated at -70 degrees C. Mean cardiopulmonary bypass time was 60 +/- 4 minutes. Mean crossclamp time was 42 +/- 2 minutes in those undergoing surgical dissection. Mean postoperative stay was 6.4 +/- 0.2 days. There were no deaths, no significant postoperative complications, and no instance of complete heart block. All patients were considered cured at the time of discharge. At a mean follow-up of 12.7 months, 18 (94.7%) remain cured. One patient with Ebstein's anomaly and a right free wall pathway had a recurrent supraventricular tachycardia 3 months postoperatively, and repeat electrophysiologic study has shown a previously unsuspected anterior septal pathway. Ventricular function returned to normal in all four patients who had abnormal function preoperatively. Surgical ablation of accessory conduction pathways can be safely done in infants and small children with results equal to those obtained in adults.     

Surgical management of congenital lymphedema in infants and children.

Of 67 children and infants with lymphedema, 28 had the congenital type. Congenital lymphedema appears during the first few weeks of life, frequently involves more than one extremity, and enlarges at a slower rate than general body growth. The swelling usually becomes less pronounced with age, and no specific therapy is required in two thirds of the patients. Seven of the 28 children had swelling of the upper extremities and a generalized lymphangiopathy syndrome. Subcutaneous lymphangiectomy was performed on ten of 28 patients who had moderate to severe swelling. Those with hand and arm involvement were particularly benefited; however, operations on the dorsum of the foot produced hypertrophic scars in one third of the cases. The operation is deferred until after age 2 years to permit optimal technical repair and to identify those patients whose conditions will improve spontaneously.     

Surgical aspects of airway management in infants and children.

The surgical management of the upper airway in infants and children varies in many important details from the principles of airway management usually applied to adults. Recent technical adaptations and innovations have improved our ability to deal with upper airway obstruction effectively. Advances in respiratory support for sick infants have saved thousands of lives and at the same time produced a new spectrum of airway problems which must be dealt with effectively. The surgeon who takes on such problems should be specially trained and dedicated to the unique requirements of infants and children.     

Surgical treatment of truncus arteriosus, with emphasis on infants and small children.

Two of 10 children with truncus arteriosus, Type I, II, less than 2 years of age, 6 of 7 between 2 and 5 years of age, and all 5 older than 5 years survived after complete repair. Five of the nine hospital deaths are believed related to inadequate intra- and postoperative management. One late death has occurred 42 months postoperatively. The low hospital mortality rates in those 2 years of age and older, good long-term results to date, and the poor prognosis of children not operated upon indicate the advisability of elective repair at age 2 to 3 years. Although results in infants less than 2 years old have not been good, current improvements of intra- and postoperative care and the lack of a better alternative suggest that prompt primary repair is indicated for infants with intractable heart failure or increasing pulmonary vascular resistance.     

Treatment of hypoglycemia in infants and children. Surgical considerations

Pancreatic resection was required in 11 pediatric patients over a 14 year period for control of persistent hypoglycemia. A standard surgical approach based on pathologic considerations was utilized. A subtotal distal pancreatectomy, liver biopsy, and removal of additional adenomatous tissue when found was performed in each patient. This approach precluded the need for routine preoperative visceral angiography, which is currently reserved for patients who require a second exploration. Surgery was curative in patients with adenomatous disease, although patients with diffuse pancreatic lesions usually required continued pharmacologic therapy postoperatively. Two patients thus far have had a total pancreatectomy performed at a second laparatomy due to persistent intractable disease. Seizure disorders, mental retardation, and behavioral problems frequently were unresponsive to improved control of hypoglycemia. An aggressive approach to management of this disorder is required if permanent neurologic damage is to be minimized.     

Surgical management of ameloblastoma in children.

OBJECTIVE: The purpose of this study was to report experience in the treatment of ameloblastoma in Taiwanese children and to discuss the treatment regimen. STUDY DESIGN: Fifteen patients, all younger than 18 years of age, with a diagnosis of ameloblastoma in our department between January 1991 and December 2004 were selected for study. Data included sex, age at surgery, tumor type, size and site of lesion, primary surgical methods, use of adjuvant therapy, complications, recurrences, and course of follow-up. RESULTS: Ages ranged from 9 to 17 (average was 13.7 years). Nine were male, 6 were female; the sex ratio was 1.5:1. Fourteen lesions were in the mandible (1 in the symphysis, 13 in the mandibular posterior area) and only 1 was in the maxilla. The size of the lesions ranged from 4 x 2.5 cm to 8 x 8 cm. Clinical typing included 8 unicystic type, 3 multicystic type, and 4 solid types. Of the histologic types, 5 were plexiform, 3 were mixed type, and 3 were acanthomatous type. Complications included numbness of the lower lip and contour defect of the face. Three cases (20%) were recurrent, and 1 patient had 2 recurrences. CONCLUSIONS: Good results can be achieved in the treatment of ameloblastoma in children using conservative surgery. In the event of recurrence, a second surgery can be successful. Patient compliance and careful follow-up are important.     

Postoperative small bowel obstruction in infants and children.

In the Pediatric Surgical Department of The St Radboud Hospital, Nijmegen, The Netherlands, between January 1970 and December 1980, 1476 laparotomies were performed on neonates, infants, and children. In 33 of these patient the abdominal surgery was complicated by a postoperative small bowel obstruction (SBO), for which a second laparotomy had to be performed. In 80% of patients this SBO developed within three months of the prior operation. The risk of developing an adhesive SBO was greater when there was more than one prior peritoneal procedure, and when, during this prior procedure, there was already a peritonitis. There was no obvious relation with the nature of the original operation. In more than 70% of patients a single adhesion caused the obstruction, while in many of these cases there were already circulatory disturbances, even by early reintervention. The mortality was 6%.     

Hemodialysis in infants and small children

Hemodialysis in infants and small children requires specialized nursing staff, equipment and adequate access. The techniques, requirements and available equipment for this population are discussed.     

Surgical treatment of airway obstruction associated with congenital heart disease in infants and small children

In the last 12 years, 21 patients age 1 month to 5 years (median, 7 months) underwent surgical treatment for severe airway obstruction associated with congenital heart disease. Plico-suspension of the pulmonary artery was carried out in 14 patients with documented severe left bronchial compression by a distended pulmonary artery together with repair of ventricular septal defects (11 patients), repair of large coronary artery fistula (1), division of ductus (1) and pulmonary artery banding (1), with subsequent significant relief of compression. Aortopexy was used in 2 patients with documented severe tracheal compression by a right aortic arch. Five patients had congenital tracheal stenoses (3 extensive, 2 localized). Two of 3 patients with associated complex intracardiac anomalies underwent concomitant repair of both lesions with the use of cardiopulmonary bypass and 1 had a tracheoplasty and pulmonary artery banding. Tracheal reconstruction with a cartilaginous graft together with repair of pulmonary artery sling was carried out in 2 infants. Nineteen patients are alive and well, at a mean follow-up of 87 months. In conclusion, we advocate early aggressive surgical intervention to both lesions to obtain a better result in the management of infants and small children with this difficult and often fatal combination.     

Surgical management of Crohn's disease in children.

Crohn's disease is more common in children than has generally been appreciated. Based on a 12 year review of cases from the UCLA Hospital, approximately 57 per cent of children with this disease require operation for the management of its complications.In 28 of 50 children undergoing operation, the disease was confined to the terminal ileum and ascending colon. Each of these patients underwent localized resection with ileocolostomy, although two children had preliminary cutaneous ileostomy; 25 per cent had mild recurrence within 5 years but only two required reoperation. Obstruction is the major symptom in this group, whereas perianal fistulas and abscesses are uncommon.Approximately one third of the patients (17 of 50) had primary Crohn's disease of the colon and rectum. Diarrhea and growth failure are common with this form of the disease. Eighty-two per cent had anal fistulas and abscesses requiring surgical treatment. Although intestinal diversion or intestinal resection, or both, with reanastomosis were tried in the majority of these patients, 14 of the 17 eventually required proctocolectomy for relief of severe symptoms. Recurrent disease in the terminal ileum occurred in 64 per cent of these patients.Only a rare patient with granulomatous disease of the small intestine alone will benefit from surgical resection.Bowel rest and total parenteral nutrition before and after resectional surgery appear to minimize the likelihood of postoperative obstruction or anastomotic leak and also to reduce the length of intestine required for resection.     

Surgical management of infective endocarditis in children.

Infective endocarditis occurs infrequently in the general pediatric population, occurring mostly in patients with congenital heart disease. This study reviews our surgical experience with infective endocarditis based on a policy of aggressive intervention, conservative operative debridement, and creative reconstruction options using pericardium and prosthetic heart valves. From 1982 to 1989, 16 patients, 3 weeks to 16 years of age, underwent 19 intracardiac operations for infective endocarditis therapy at Kosair Children's Hospital. Eight (42%) were for resection of vegetations alone; an additional 11 operations (58%) involved more extensive debridements requiring either valve replacement or valvuloplasty using pericardium for exclusion of an abscess cavity, closure of a fistula, or for valve repair. Operative mortality was 25% (4 patients) and related to preoperative disease severity. There was one late death. Offending organisms included Staphylococcus species (31%), Haemophilus influenzae (13%), pneumococcus (5%), gram-negative organisms (13%), and Candida (13%); no organism grew on culture in 25%. We conclude that aggressive surgical exploration in patients with infective endocarditis is indicated and often requires resection of vegetations alone. More extensive procedures should preserve as much valvular tissue as possible. Pericardium is useful for reconstruction after debridement.     

Surgical management of hiatal hernia in children.

Hiatal hernia should be included in the differential diagnosis of all children with emesis and failure to thrive, since early diagnosis is imperative to prevent the irreversible esophageal damage from long-standing peptic esophagitis. The Nissen fundoplication as described in this paper appears to be far superior to gastropexy in preventing recurrence of gastroesophageal reflux. Colon interposition should be reserved for those cases in which hiatal herniorrhaphy is technically impossible. Successful repair of the hiatal hernia results in rapid improvement in the nutritional status of these children.     

Epilepsy and focal lesions in children. Surgical management

Epilepsy surgery has gained a large role in the treatment of intractable seizures in the last few decades because of the development of operative techniques and better identification of the cerebral anomalies using electrophysiological recordings and neuroimaging. A series of 419 children, aged from five months to 15 years, with epilepsy (medically refractory in 85.5% of them) associated with focal cortical lesions, who underwent surgery between 1986 and 2006 was analyzed to identify the factors that correlated with outcome. Mean follow-up was 5.2 years. According to Engel's classification; 75.8% of the children were seizure-free. When the lesion was well defined, correlations between clinical data, radiological features and electrophysiological features, suggesting a zone of seizure onset around (or even in) the lesion, was the best guarantee of achieving good seizure control by lesionectomy. Nevertheless, seizure outcome was also determined by other factors: the duration of the epilepsy and the surgery. Persistence of seizures was found to be significantly associated with the preoperative duration of epilepsy, the completeness of the lesional resection and de novo brain damage induced by the surgical procedure itself. Early surgery must be considered in children because of the benefits of seizure control on the developing brain and the risk of secondary epileptogenesis.     

Surgical management of children with hemoglobinopathies.

Sickle hemoglobinopathies include sickle cell disease, sickle-C disease, and sickle-beta thalassemia. Patients with these disorders commonly suffer a multitude of destructive events to vital organs, especially to the central nervous system, the spleen, the kidney, the lung, and the heart as a result of microvascular plugging by the sickled erythrocytes. Thoughtful preparation for anesthesia and operation, especially when directed by experienced individuals, can greatly reduce the hazard of inducing the sickle crises that formerly plagued individuals with sickle hemoglobinopathies who faced major operations under general anesthesia. The patient must be free of any acute illness, especially one involving the respiratory system. Adequate hydration preoperatively combined with avoiding perioperative hypoxia, hypothermia, and acidosis, the triggers for sickling, will go far toward avoiding sickle-induced complications. Modern transfusion therapy, consisting of multiple small transfusions of Hb A erythrocytes administered over several weeks prior to the operation, not only corrects the chronic anemia but suppresses erythropoiesis of cells containing Hb S in the patient's bone marrow and leaves him or her with a majority of cells containing Hb A. This provides a safety net in case a sickle-inducing insult occurs despite the best efforts to avoid one. Individuals with sickle hemoglobinopathies may require any of the operations common to all children, for example, herniorrhaphy, appendectomy, tonsillectomy, and circumcision, but a significant number will develop calcium bilirubinate cholelithiasis and possibly cholecystitis as a result of the continual increased load of bile salts resulting from the shortened lifespan of the cells containing Hb S. Also, although most individuals with Hb S will gradually suffer splenic infarction by late childhood, a significant number of infants will experience acute splenic sequestration crisis, a life-threatening entity, the recurrence of which is prevented by splenectomy. Several publications have demonstrated that such surgical procedures can be performed in large numbers of patients with sickle hemoglobinopathies without deaths and with minimal morbidity.