Helene Flageole

Outcome of the Prenatally Diagnosed Congenital Cystic Adenomatoid Lung Malformation: A Canadian Experience

Congenital cystic adenomatoid malformation of the lung (CCAM) is diagnosed by prenatal ultrasonography with an increasing frequency but controversy persists as to its prognosis and prenatal management. Method: A multi-institutional study of cases of CCAM diagnosed antenatally identified by ultrasonographers and by a review of hospital charts. Results: We obtained 48 cases from five centers. We estimate the incidence of CCAM at 1:25,000 to 1:35,000 pregnancies. The incidence of voluntary abortions was 15% (7/48), of spontaneous abortions 2% (1/41) and of postnatal death 10% (4/40). One of the postnatal deaths was from trisomy 18. Of the 7 aborted fetuses, 2 had multiple malformations and 1 had severe hydrops and oligohydramnios; the other 4 had a large mass with mediastinal displacement but without hydrops. When pregnancy was allowed to continue, 56% of the lesions regressed spontaneously, even though one third of these had initial progression. In 17 cases (42%) the mediastinal shift corrected itself, sometimes by simple growth of the fetus but most often by a decrease in the size of the lung mass. In 1 fetus, repeated needle decompressions followed by double-pigtail catheter drainage of large cysts allowed regression of hydrops. Despite this, neonatal death occurred from pulmonary hypoplasia. Conclusion: CCAM can lead to fetal or neonatal demise from hydrops, lung hypoplasia, prematurity or severe associated malformations, but has a good prognosis in the majority of cases.

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.

The management of asymptomatic congenital lung malformations

Congenital Pulmonary Airway Malformation (CPAM) and lung sequestration are often discovered on routine prenatal sonography. While some lesions may be large and cause serious complications in the fetus or newborn, many will be asymptomatic at birth. Some authors advocate simple observation because of the lack of data on the incidence of long-term complications. However, there are very few described cases where CPAM and intralobar sequestration (ILS) have remained asymptomatic throughout life; complications eventually develop in virtually all patients. The most common complication is pneumonia, which may respond poorly to medical treatment. Other complications include the development of malignancies (carcinomas and pleuropulmonary blastomas), pneumothorax and hemoptysis or hemothorax. Since lung resection will be required sooner or later for CPAM and ILS, it is best not to wait for complications to occur. We recommend surgery at 3 to 6 months of life at the latest, to allow compensatory lung growth to occur. At this age the postoperative course is usually smooth and long-term follow- up has shown normal respiratory function. The management of small non- communicating extralobar sequestrations is more controversial; it is known that these can remain asymptomatic throughout life but complications may develop and they are sometimes difficult to differentiate from neuroblastoma.

Tracheoscopic endoluminal plugging using an inflatable device in the fetal lamb model

OBJECTIVE Intra-uterine tracheal occlusion has been proposed to reverse pulmonary hypoplasia, an important prognostic factor in congenital diaphragmatic hernia. We aimed to evaluate the feasibility and pulmonary effects of tracheoscopic tracheal obstruction with a detachable balloon. STUDY DESIGN Fourteen mid-trimester fetuses out of 24 in 13 ewes underwent tracheoscopic balloon obstruction. Ten non-operated fetuses served as controls. Plugging was performed under fiber-tracheoscopy using a detachable balloon. Outcome measures consisted of: total operating time, tracheoscopy time, fetal survival, efficiency of plugging, and pulmonary effects. The Mann-Whitney test and linear regression were used for statistical analysis. RESULTS Mean operating time and tracheoscopy time were 65+/-12 and 6.6+/-3.9 min, respectively. One intra-operative death occurred in each group. The post-operative mortality was 2/13 for cases and 2/9 for controls. In all 14 fetuses, the trachea was successfully obstructed. In the 11 treated animals born alive, the lung-to-body-weight ratio was 0.060+/-0.01, while in controls it was 0.031+/-0.01 (P = 0.0001). In a subset of six fetuses obstructed for 14-18 days, mean-terminal-bronchial density was 0.95+/-0.59, compared to 2.06+/-0.80 for controls (P = 0.046). CONCLUSIONS Using fetal tracheoscopy, the trachea can successfully be obstructed with an inflatable balloon. Pulmonary hyperplasia is achieved when the obstruction lasts 2 weeks.

Tracheoscopic endotracheal occlusion in the ovine model: technique and pulmonary effects.

BACKGROUND/PURPOSE The purpose of this study was to assess the tracheoscopic placement of a detachable balloon to obstruct the trachea in the fetal lamb model and to study the pulmonary effects 2 weeks later. METHODS Thirteen pregnant ewes carrying 27 fetuses were used in this trial. Thirteen fetuses underwent endotracheal plugging at a mean gestational age of 95 (90 to 100) days (term, 145). Fourteen nonoperated fetuses served as controls. The intended duration of obstruction was 15 days. Study parameters consisted of operating time, fetal survival, performance of the balloon, and pulmonary changes. RESULTS Hysterotomy time was 41 +/- 25 minutes and tracheoscopy time was 7.2 +/- 3.4 minutes. There were no intraoperative deaths in this series. Postoperative fatalities occurred in 2 of 13 plugged fetuses and 1 of 14 controls. At completion of the experiment, the balloon was recovered intact in the trachea of 12 of 13 (92.3%) fetuses. The lung weight/body weight ratio was 76 +/- 16 mg/kg for plugged animals, while in controls it was 34 +/- 8 mg/kg (P = .0001). The lung volume/body weight ratio was 101 +/- 17 mL/kg in plugged fetuses, compared with 47 +/- 4 mL/kg for controls (P = .0002). Mean-terminal-bronchial-density was 0.937 +/- 0.303 for plugged animals, compared with 1.911 +/- 0.441 for controls (P = .0002). Mean linear intercept was 60.9 +/- 5.2 microm in treated fetuses, and 46.3 +/- 5.8 microm for controls (P = .0004). CONCLUSIONS Fetal endotracheal occlusion, using a tracheoscopically placed detachable balloon, is reliable, and results in pulmonary hyperplasia after 2 weeks of obstruction. This technique may be beneficial in treating the pulmonary hypoplasia seen in congenital diaphragmatic hernia. Further studies using this balloon device are now required to corroborate these findings.

Outcome of laparoscopic appendectomy for perforated appendicitis in children.

BACKGROUND The aim of this study was to evaluate the outcome of laparoscopic (LA) vs open appendectomy (OA) in patients with perforated appendicitis in our center. METHODS Retrospective review from July 2002 to April 2007 (institutional review board-approved), evaluating 281 patients with perforated appendicitis based on surgical approach. We compared demographics, mean operative time, length of stay (LOS), infectious complications, and follow-up in patients with OA (n = 213) and LA (n = 68). RESULTS Laparoscopic appendectomy patients were significantly older (12 vs 9.4 years), heavier (51.8 vs 36.6 kg) and more frequently girls (47.8% vs 34.3%). Mean operative time was longer in LA (72.6 vs 50.2 minutes). Median LOS was 5 days in LA and 6 days in OA. Few patients in each group required a drainage procedure for a persistent abscess (LA 4.4%, OA 4.7%; P = 1.000). Laparoscopic appendectomy patients had fewer wound infections (1.5% vs 9.5%; P = .034), and less follow-up visits were needed (>2 clinic visits 4.5% vs 16.4%; P = .013). CONCLUSION Laparoscopic appendectomy has a shorter median LOS, a trend toward less postoperative infectious complications, and fewer clinic visits than OA, which makes it a safe and effective procedure for patients with perforated appendicitis.

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.

Sclerotherapy for lymphatic malformations in children: a scoping review

PURPOSE This scoping review assesses the literature and summarizes the current evidence on sclerotherapy for the treatment of lymphatic malformations in pediatric patients. METHODS A comprehensive search of published and unpublished literature was conducted using multiple databases. Title, abstract, and full-text screening was conducted by 2 independent clinicians. All discrepancies were resolved during consensus meetings. RESULTS A total of 182 articles were retrieved. Forty-four articles were removed as duplicates, and 11 articles were added after reviewing prominent studies. After full-text abstraction, 44 articles and 2 conference proceedings (N = 882 patients) were included in the final results. Twelve articles were classified as level II and 34 articles as level IV evidence. Picibanil (OK-432) was the primary agent used in most included studies. Postinjection symptoms with OK-432 were primarily fever, swelling, and erythema at the site. Life-threatening complications were uncommon and involved postinjection swelling of cervical lesions causing airway compromise. CONCLUSIONS The literature regarding sclerotherapy for lymphatic malformations is of a low level of evidence and suffers from a lack of standardization. Randomized clinical trials focused on OK-432, bleomycin, or alcoholic solution of zein; standardized dosing protocols; and consistent and reliable outcome reporting will be necessary for further development of treatment guidelines.

A contemporary evaluation of surgical outcome in neonates and infants undergoing lung resection

BACKGROUND/PURPOSE The timing and need of resection of asymptomatic congenital lung lesions are controversial. The morbidity of such surgery needs to be considered in the decision analysis. We analyzed the contemporary outcome of infants and neonates undergoing lung resection. METHODS With institutional review board approval, all patients 12 months or younger undergoing lung resection between 1995 and 2004 in 2 hospitals were reviewed. Demographic data, indications for surgery, operative procedure, complications, use of regional anesthesia, length of stay (LOS), and follow-up were assessed. RESULTS Forty-five patients (28 male, 17 female) with a median age of 4 months (2 days-12 months) were evaluated. Congenital lesions (42) were the most frequent indication for surgery. Twenty-two (48.9%) patients had cardiorespiratory symptoms or infection preoperatively. Lobectomy was the most common operation (40/45). Three patients had intraoperative difficulty (bleeding, hypotension, desaturation). Significant postoperative complications occurred in 7 patients: prolonged air leak or chest tube drainage (4), anemia or bleeding (2), respiratory distress requiring reintubation (1). Fewer complications occurred in asymptomatic vs symptomatic patients (1/23 vs 6/22). Of 12 patients, 7 (58%) requiring 24 hours of ventilation or longer were 3 months or younger. Increasing age did significantly influence the chance of successful extubation (P = .01; odds ratio, 1.5; 95% confidence interval, 1.0-2.0), as did the use of epidural anesthesia (P < .001). Median LOS was 6 days (2-89 days). Asymptomatic patients had shorter LOS (median, 4 days; range, 2-20 days; P = .024) vs symptomatic patients (median, 8 days; range, 4-89 days). The only death occurred from underlying heart disease. Mean follow-up at 35 months (12-132 months) revealed no subjective reduction in cardiopulmonary function. CONCLUSIONS Lung resection is safe and well tolerated in infancy. Surgery should be scheduled before the development of symptoms but likely after 3 months of age to improve the chances of postoperative extubation. The use of regional anesthesia may facilitate this.

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.