L.T. Nguyen

25 years' experience with lymphangiomas in children.

BACKGROUND/PURPOSE The management of lymphangioma in children is challenging because complete resection is difficult to achieve in some cases, and recurrences are common. The authors reviewed their experience to assess the risk factors for recurrence and the role of nonoperative treatment. METHODS A retrospective study over a period of 25 years was carried out. One hundred eighty-six patients with 191 lesions (five patients with de novo lesions in different sites) were treated. There were 98 boys and 88 girls. The average age at diagnosis was 3.3 years (range, fetal life to 17 years) and the average size 8 cm in diameter. Histocytological confirmation was obtained in all patients. The involved sites were head and neck, 89 patients (48%); trunk and extremities, 78 patients (42%); internal or visceral locations (eg, abdominal and thorax), 19 patients (10%). The treatment consisted of macroscopically complete excision in 145 patients (150 lesions, of which five were recurrences in different sites), partial excision in 10 patients, aspiration in five patients, laser excision in 10 patients, biopsy only in four patients, drainage and biopsy in two patients, and injection of sclerosing agents in 10 patients. RESULTS There were 54 recurrences; 44 underwent excision (five of them more than once), and five regressed spontaneously on follow-up. Five other recurrences were stable and not progressing. Recurrences, (defined as clinically obvious disease), were found to be 100% after aspiration, 100% after injection, 40% after incomplete excision, 40% after laser excision, and 17% after macroscopically complete excision. The recurrence rate in the last group was the highest in the head (33%), the least in the internal locations (0%), and intermediate for the cervical location (13%). There were no significant differences, in terms of outcome, between those who had their surgery immediately at the time of diagnosis (n = 101) and those who had delayed surgery (n = 85). CONCLUSIONS There were fewer recurrences after macroscopically complete excision. Aspiration and injection had the highest recurrence rate. Risk factors for recurrence included location, size, and complexity of lesions. A period of observation may be useful for infants to facilitate complete excision. In the present series, spontaneous regression was infrequent and was seen more often with recurrent lesions.

Appendicitis in children: Current therapeutic recommendations

Wound infection is the most common source of morbidity in appendicitis. Most recent pediatric series use protocols of preoperative antibiotics with aerobic and anaerobic coverage, intraoperative lavage, no peritoneal or wound drains, and continuation of antibiotics postoperatively with complicated appendicitis. There still remains controversy concerning skin closure and the duration of antibiotic therapy. We report the results of a prospective protocol followed over 2 years with 420 children. The protocol was designed to determine whether the skin could be closed primarily in all patients undergoing appendectomy. Preoperatively all patients received triple antibiotics (ampicillin, gentamicin, and clindamycin) that were continued postoperatively for two doses if there was a normal appendix or simple acute appendicitis, for at least 3 days with gangrenous appendicitis, and at least 5 days with perforated appendicitis. Antibiotics were continued if the patient remained febrile or had a white count greater than 10,000. No drains were used and the skin was closed primarily. The overall infectious complication rate was 1.0% (4/420). Among those with a normal appendix or simple acute appendicitis there were no infectious complications. Among those with gangrenous or perforated appendicitis there were 1.7% wound infections (2/117) and 1.7% intraabdominal abscesses (2/117). Duration of hospitalization was 2.1 days (range, 1 to 5 days) after simple acute appendicitis and 6.9 days (range, 3 to 40 days) after gangrenous or perforated appendicitis. These results set new standards in terms of wound management, infectious complications, and length of hospital stay.

Congenital adenomatoid malformation of the lung: current management and prognosis.

A retrospective 10-year review of congenital adenomatoid malformation (CAM) included 10 cases diagnosed in utero by ultrasound and 13 cases that presented postnatally. Two prenatally diagnosed cases were aborted because of associated lethal anomalies. All remaining patients underwent resection. Up to one third of all cases, whether diagnosed prenatally or postnatally, were asymptomatic. Resection is recommended to avoid respiratory distress, infection, or associated malignancy. There were 5 nonsurvivors, including 2 therapeutic abortions and 3 who died postoperatively. All had either polyhydramnios or ascites. All patients who died postoperatively had a respiratory arrest at birth and underwent immediate lobectomy. All died on the first day of life after a brief period and were found to have associated pulmonary hypoplasia. One had undergone a prenatal transthoracic cyst aspiration at 34 weeks gestation in an attempt to allow lung growth and prevent premature labor. Prenatal ultrasound findings suggestive of poor prognosis included polyhydramnios, ascites, mediastinal shift, and noncystic type III CAM. However, there is a spectrum of severity of CAM. The lesion can either regress and be asymptomatic at birth, or it can progress to cause either fetal death from hydrops or neonatal death caused by associated pulmonary hypoplasia. These findings should be considered in prenatal counseling for CAM.

Laparoscopic cholecystectomy : a treatment option for gallbladder disease in children

Laparoscopic cholecystectomy was performed safely in two children ages 7 and 15 years. Special considerations were required in the younger child because of the discrepancy between the length of the instruments and the size of the peritoneal cavity. Both children remained in hospital less than 24 hours and were able to resume normal diet and activity quickly. Pediatric surgeons with special training in laparoscopic surgery may want to use this treatment modality for children with biliary tract disease.

Recurrent intussusception: Safe use of hydrostatic enema

Recurrent intussusception (RI) occurs in 5% to 8% of patients with intussusception. The authors reviewed their 15-year experience to better define the management of RI. Among 258 patients, 28 (10.8%) had episodes of RI (37 episodes altogether). Twenty-two patients had a single recurrence, four had double recurrence, one had triple recurrence, and one had quadruple recurrence. RI and non-RI patients were compared with respect to symptoms. The incidence of vomiting and bloody stools was significantly lower in the RI group. Ten percent of RI patients had lead points. Thirty percent of RI episodes occurred within 24 hours, and 74% occurred within 6 months. The success rate of hydrostatic enema reduction for recurrent episodes was 62.8%, which is comparable to that for the initial episode (68.9%). Among patients with previous surgery, 36.3% of enemas were successful; after previous enema reduction, 75% were successful. There was no complication related to the enema. Enema reduction is safe and has a good success rate in cases of RI, even after previous surgical reduction.

Spontaneous deep vein thrombosis in childhood and adolescence

Deep vein thrombophlebitis (DVT), unrelated to intravenous catheters, is unusual in children. During the period between 1970 to 1984, 15 cases of spontaneous DVT were seen at The Montreal Childrens Hospital. The female to male ratio was 2:1. Age ranged from 10 to 17 years. Venogram was positive in the 14 patients in whom it was done. Significant factors were related to the DVT in 12 patients: oral contraceptives, pelvic fracture, Ewings sarcoma and chemotherapy, brain tumor, nephrotic syndrome, and ulcerative colitis (2, one of whom developed the DVT 3 months before the onset of gastrointestinal symptoms). The sites of thrombophlebitis encountered were left iliofemoral vein, right iliofemoral, right tibial, and left subclavian. Three patients developed pulmonary emboli, which were recurrent in two and required iliac vein ligation or inferior vena cava clipping. Treatment usually consisted of intravenous heparin followed by oral anticoagulants for 3 to 6 months. Two patients received streptokinase with good results. All the patients recovered. With a limited follow-up (6 months to 5 years), we have not seen any postphlebitic sequelae.

Diaphragmatic pacing in children with congenital central alveolar hypoventilation syndrome

BACKGROUND The purposes of this study were to study congenital central alveolar hypoventilation syndrome (CCAHS), to report the experience at our institution with this disorder, and to describe the surgical technique of diaphragmatic pacing. Three patients are in our diaphragmatic pacing program. They all have CCAHS, documented by means of lack of respiratory drive to hypercapnia and normal peripheral nerve and muscle studies. Two patients have associated Hirschsprungs disease. METHODS The ages of patients at insertion of bilateral phrenic nerve pacemakers were 1, 2, and 5 years. A bilateral axillary thoracotomy in the third interspace was used in two patients, and anterior thoracotomy was used in one for insertion of electrodes on the phrenic nerves. Counterincisions in both flanks were used in all patients for insertion of the receivers in subcutaneous pockets. RESULTS In all patients pacing was commenced within 1 week of the surgical procedure, because no primary pacemaker failures occurred. One patient has experienced no failure of the equipment and no infectious complications at 4 years. A second patient has had the pacemakers for 6 months without complications. The third patient underwent placement of bilateral pacemakers in 1984. In this patient a 10-year experience has resulted in subcutaneous implant failure on two separate occasions; also the presence of a staphylococcal empyema necessitated the temporary removal of one phrenic nerve electrode for 6 months, with successful reinsertion. All patients now receive mechanical assisted ventilation when sleeping and phrenic nerve pacing when awake. CONCLUSIONS Pediatric surgeons should be aware of CCAHS because it may be treated with surgically implanted electrodes that allow for pacing of the diaphragm. The technique has an acceptable complication rate, and it can greatly decrease the impact of the disease on the lifestyle and activity of the patient. CCAHS also may be associated with Hirschsprungs disease.

Pediatric spontaneous pneumothorax

The aim of this study was to review pediatric spontaneous pneumothorax and describe diagnostic and therapeutic approaches and to review our institutional experience with spontaneous pneumothorax. A medline search using keywords pneumothorax and pediatric in both French and English from 1988 through May 2002 was performed, and pertinent papers regarding the natural history, diagnosis, imaging, and therapy were reviewed. A 10-year chart review of spontaneous pneumothorax recording data on demographics, diagnostic imaging, therapeutic approach, and outcomes was performed. Only 4 case series and one review article were produced by the medline search involving exclusively pediatric spontaneous pneumothrax. One hundred ninety-six other articles involved adult series with a minority of patients under 18 years of age. A subset of articles from this adult-based group were examined to assist in formulating recommendations for diagnosis, imaging, treatment, and surgical management of pediatric spontaneous pneumothorax. Few articles on this topic report randomized studies in the adult literature and none in the pediatric literature. The chart review yielded 53 cases of spontaneous pneumothorax in 40 patients with important data presented in the current review. Although few articles are able to provide best-evidence information, the diagnosis, imaging, treatment, and surgical management of patients with primary spontaneous pneumothorax is achieved with good results. Stronger data specific to pediatric patients are needed.

Hydrometrocolpos in neonate due to distal vaginal atresia

Hydrocolpos is the result of vaginal obstruction and can become an emergency in the newborn period. The treatment of imperforate hymen is well defined, but the treatment of vaginal atresia is more complex. We encountered two cases of hydrocolpos secondary to distal vaginal atresia, that were operated on in the first days of life. One baby had distal atresia without persistance of urogenital sinus. Surgery combining abdominal perineal approaches and a posterior vaginoplasty was carried out. The second baby had hydrocolpos with persistance of urogenital sinus. Drainage through the sinus was unsuccessful because the baby developed sepsis by trapping urine in the uterus. Finally an abdominoperineal vaginal pull-through was successfully done. The embryology and literature are reviewed. The classification, indications and surgical technique are discussed.

Repair of congenital diaphragmatic hernia after weaning from extracorporeal membrane oxygenation

Stabilization and delayed operation for patients with congenital diaphragmatic hernia (CDH) is now widely accepted. When preoperative extracorporeal membrane oxygenation (ECMO) is needed, most centers have CDH repaired on ECMO to minimize the risk of postoperative deterioration. The authors adopted a policy of weaning from ECMO before repair in an effort to avoid hemorrhagic risks. They reviewed their experience with CDH patients who required ECMO for stabilization before repair but for whom post-ECMO repair was planned. The records of all high-risk CDH patients with a gestational age of at least 34 weeks were reviewed. Eighteen patients were identified. None of the eight who were stabilized and operated on without ECMO required bypass postoperatively; all survived. Ten were placed on bypass, nine for stabilization before repair. Of the nine, seven (78%) were weaned from ECMO to conventional ventilation. Repair of the diaphragmatic defect was performed an average of 3.8 days later; none of these patients had severe pulmonary hypertension postoperatively, and all survived. Two could not be weaned before repair, one of whom had a complex congenital heart defect. This patient died. The other patient had repair on ECMO because of intrathoracic gastric volvulus. Severe blood loss prompted decannulation, and the patient died. One patient who was placed on bypass was transferred 10 days after having had repair elsewhere (at 4 hours of age). Pulmonary hypertension did not resolve, and the postmortem examination showed alveolar capillary dysplasia, with focal misalignment of the pulmonary vessels.(ABSTRACT TRUNCATED AT 250 WORDS)