Double-lung transplantation in children: a report of 20 cases. The Joint Marseille-Montréal Lung Transplant Program.

In the last 3 1/2 years, we have performed 20 double-lung transplantations in children between 7 and 16 years old (mean age, 13 years). One patient had primitive bronchiolitis obliterans and the other 19, cystic fibrosis. Eight patients were operated on in an emergency situation, 7 of them requiring ventilator support before transplantation. The procedures were en bloc double-lung transplantation in the first 11 patients with separate bronchial anastomoses in 10, and sequential bilateral lung transplantation in the later 9 patients. There were no operative deaths. Two patients died in the hospital on postoperative days 37 and 73, and there were four late deaths, which were due to infection, rejection, and bronchiolitis obliterans. The acceptable incidence of airway complications, the improvement in lung function of survivors, and the acceptable midterm survival make double-lung transplantation an acceptable alternative to heart-lung transplantation in children. However, in very small children, heart-lung transplantation may be preferable because of the size of the airway anastomoses at risk.

Esophageal atresia, tracheomalacia and arterial compression: role of aortopexy.

Between 1985 and 1990, we treated 46 cases of esophageal atresia in the pediatric surgery department in Marseille. In 17 of these patients atresia was associated with tracheomalacia. The most common respiratory manifestations were acute apneic attacks, cyanotic spells and recurring pneumopathy. The underlying cause was arterial compression of the airways by the innominate artery in 6 cases, the aortic arch in 2 cases, and a common origin of the innominate and left carotid arteries in 2 cases. Eleven patients underwent surgery to relieve arterial compression: suspension of the aorta from the under surface of the sternum (10 cases) and reimplantation of the innominate artery (1 case). Results in terms of tracheal caliber and symptomatic relief were excellent in 10 cases including 1 case requiring redo, and poor in 1 case. For diagnosis, our experience shows the importance of endoscopy, nuclear magnetic resonance and recognition of gastroesophageal reflux which is often associated (9/11). The role of tracheomalacia is discussed. On the basis of our results and those previously reported, aortopexy appears to be an effective surgical technique in patients with esophageal atresia presenting respiratory complications due to arterial compression.

Management of pediatric laryngotracheal stenosis.

In the past 6 years, 40 children underwent surgery for laryngotracheal stenosis; 32 by the external approach and 8 by endoscopic CO2 laser. Twenty-seven children (67%) were less than 5 years old at the time of treatment and 80% of the stenoses (n = 32) corresponded to an etiology that is secondary to endotracheal intubation and/or tracheotomy. By grading the stenoses according to the amount of narrowing of the lumen, the authors emphasize the interest of conservative treatment (endoscopic for grade I [less than 70%, n = 8], and treatment by external surgical methods for grade II [70% to 90%, n = 13], grade III [90% to 99%, n = 14], and grade IV [total obstruction, n = 5]). At this time, the most commonly used technique is laryngotracheoplasty with costal cartilage interposition. In this series, 88% of the patients were successfully decannulated. As for the treatment of stenosis in infants, the authors describe their recent experience of laryngotracheofissure in 7 patients as an alternative to either tracheotomy in cases of difficult extubation or laryngotracheoplasty when the child is underweight.

[Hirschsprung's disease: a commentary]. / La maladie de Hirschsprung: un commentaire.

Hirschsprung disease has become a neonatal diagnosis. Most cases identified now are children who would previously have died before the diagnosis of their condition which was usually established only after the age of two. Management of the disease should be adjusted to suit this new population of patients. Clinical features consist mainly in evidence of intestinal obstruction and enterocolitis shortly after birth and require the use of several investigative procedures since therapy cannot be initiated before the diagnosis is firmly established. Non-aggressive investigations are now available and include rectomanometry, conventional radiology, and suction mucosal biopsy of the rectum with histologic and cytologic studies of specimens. No single test is diagnostic, but taken in concert results of the different investigations are conclusive. Advances in nursing have made colostomy unnecessary and even harmful since it delays defecation by the natural route. Regardless of the technique selected (Duhamel, Soave or above all, Swenson), surgical treatment can be carried out before two months of age. A review of 90 patients, prove treatment is easy to perform at this age and yields satisfactory immediate and long term results.

[Laryngotracheal stenosis in children. Current aspects of the surgical treatment and prevention]. / Les sténoses laryngo-trachéales de l'enfant. Aspects actuels du traitement chirurgical et de leur prévention.

In the past 6 years, 40 children underwent surgery for laryngotracheal stenosis; 32 by the external approach, 8 by endoscopic CO2 laser. Twenty-seven children (67%) were less than 5 years old at the moment of treatment and 80% of the stenoses (n = 32) corresponded to an etiology that is secondary to endotracheal intubation and/or tracheotomy. By grading the stenoses according to the amount of narrowing of the lumen, the authors emphasize the interest of conservative treatment (endoscopic) for grade I (less than 70%, n = 8), and treatment by external surgical methods for grade II (70%-90%, n = 13), grade III (90%-99%, n = 14), and grade IV (total obstruction, n = 5). At this time, the most commonly used technique is laryngotracheoplasty with costal cartilage interposition. In this series, 88% of the patients were successfully decannulated. As for the treatment of stenosis in infants, the authors describe their recent experience of laryngotracheofissure in 7 patients as an alternative to either tracheotomy in cases of difficult extubation or laryngotracheoplasty when the child is underweight.

Management of Hirschsprung's disease: curative surgery before 3 months of age.

Curative surgery of Hirschsprung's disease (HD) was performed in 32 infants younger than 3 months of age from January 1, 1977, December 31, 1986. There were 24 males, seven of whom presented with total colonic aganglionosis. Preoperative relief of obstruction was carried out by only enemas in 25 patients (81%), with addition of total parenteral nutrition of 8 to 27 days in case of severe enterocolitis. Colostomy was never performed as a routine procedure. Preoperative morbidity occurred in three cases: one enterocolitis and two sigmoid performations that were successfully treated by colostomy. Weight at operation ranged from 3.3 to 6.0 kg (mean, 4.9 kg). Swenson's (25), Duhamel's (5), and Soave's procedures (2) were performed. There was no mortality. Follow-up varied from 2 to 10 years. Postoperative morbidity occurred in five patients (one anastomotic leak, two stenosis, one volvulus, and one recurrent enterocolitis). All other patients are considered as definitively cured. Comparison with data in the literature permits one to advocate primary corrective treatment of HD without decompression in infants less than 3 months of age.

A 40-year multinational retrospective study of 880 Swenson procedures.

This report reviews the experience of pediatric surgeons in seven cities in North American and Western Europe where the Swenson procedure was performed on 880 patients. Information on the diagnosis, treatment, complications, and long-term results was collected by reviewing the hospital records, the treating physicians' office records, and by interviewing the patients in person or by telephone. A follow-up evaluation was obtained on 814 patients. The patients' ages at the time of the resection ranged from four days to 50 years. The length of follow-up averaged 10.3 years, while the longest follow-up was 39.5 years. The overall postoperative mortality was 2.4% during the entire 40 years of the study. The postoperative mortality has decreased to 1.25% for the last 20 years. Significant factors influencing postoperative mortality included Down's syndrome, the patient's age at the time of the operation, and leak of the distal colonic anastomosis. Most of the patients followed for over 5 years have normal bowel habits, report one to three bowel movements per day, and have no soiling. No patient has urinary incontinence or impotence.

[Double lung transplantation. Report of the 1st French case and comments on the 5 subsequent cases]. / Les transplantations bi-pulmonaires. Rapport de la première observation française et commentaires des cinq suivantes.

Using a modified version of the technique described by the Toronto group, the Marseille group has performed 6 double lung transplantation procedures. In 6 cases the underlying disease was cystic fibrosis. Four patients are currently alive. The technique was modified in two ways. First anastomosis was made on the two main stem bronchi in order to rule out the risk of ischemic complications. Second a special postoperative care technique in which the patient is regularly turned from one side to the other was applied to avoid lymph stasis during the first postoperative weeks.

[Cancer of the thyroid in children]. / Le cancer de la thyroïde chez l'enfant.

Study of a personal series of 10 cases of thyroid cancer in children and a review of the literature reveals what we consider to be the 3 problems which are posed by this pathology in children: 1. The frequency of isolated cervical adenopathy in the initial symptomatology may be confused with congenital swelling or inflammatory adenopathy. 2. The surgical management depends on the histological nature: total thyroidectomy, lymph node excision, risk to the parathyroid gland and IRAtherapy all depend on the histological data. 3. Finally, the long term prognosis of these cancers is unknown. Schematically, differentiated cancers including papillary carcinomas have a good or indeed excellent prognosis, while medullary carcinomas have a prognosis which will imminently depend on the extrathyroid involvement, the quality of thyroidectomy and lymph node clearance. This highlights the problem of screening for subclinical disease in familial forms of the disease.

[Surgical treatment of benign laryngotracheal stenosis. Apropos of 12 cases]. / Traitement chirurgical des sténoses laryngo-trachéales bénignes. A propos de douze observations.

The authors report a series of twelve benign laryngotracheal strictures. In almost every case, these lesions were secondary to tracheal intubation and consisted of extensive, circumferential and almost complete stenosis. All were treated surgically. The authors obtained enlargement of the airway be means of an interposed cartilaginous graft and an intraluminal prosthesis which was left in place. In nine cases, this procedure was combined with posterior cricotomy. One eight month old infant died during the postoperative period. Ten patients had an uneventful postoperative course with a satisfactory anatomical and functional result.

[Pediatric organ transplantation]. / La transplantation d'organes pédiatrique.

Since we started our pediatric kidney transplant program in 1970, we advocate children's transplantation to be performed in pediatric surgery units. Recent progress in immuno-suppression with ciclosporine and in operative procedures lead us to extend the program to liver transplantations in 1986, then to heart and lung transplantations in 1988. The Pediatric Transplant Unit was designed to assume the pre-operative evaluation of the recipients and the post-operative course of transplanted patients, closely connected to all specialists dealing with medical and surgical diseases of children. 29 patients were transplanted (kidney: 8, liver: 14, heart: 1, lungs: 6) with a 83% overall survival rate. The goal of this paper is not to discuss and compare indications or results with others series. Through our experience of pediatric organ transplantation, we shall try to point out the main advantages of a Pediatric Transplantation Unit: it optimizes the management of the rare pediatric donnors, and allows better skill and efficiency of the numerous specialities concerned by organ transplantation, such as intensive care, infectiology, immunology, radiology... The common medical and para-medical staff, common operative theater, and common use of equipment in the same department for transplantation of different organs is also an important matter to be considered now in term of cost-effectiveness.

Combined approach in the treatment of anorectal malformations: the MOPE procedure.

No single major procedure is able to achieve all necessary goals without problems in the treatment of anorectal malformations. The Mollard-Pena (MOPE) procedure adds the benefits of an anoplasty based on the original perineal flap described by Mollard, but slightly modified, and the precise recognition of all muscle structures as reported by Pena and De Vries. Cosmetic as well as functional early results are encouraging.

Evaluation of the role of surgery in 130 patients with neuroblastoma.

The role of surgery for children with neuroblastoma was evaluated by using a recently proposed TNM staging system. One-hundred thirty patients were retrospectively assigned a TNM clinical stage (CS) preoperatively and a pathologic stage (PS) postoperatively. Patients with CS 4 were separated into CS 4A and CS 4B according to their age and pattern of metastases. Patient survival was analyzed according to CS, age, location of primary, and PS. Actuarial survival of patients was as follows: CS 1, 100%; CS 2, 82%; CS 3, 63%; CS 4A, 50%; and CS 4B, 5%. For all stages, patients younger than 1 year old survived longer than those older than 1 year (72% v 32%). Prognosis for CS 1 was the same regardless of age. For CS 2 and CS 3, patients younger than 1 year old lived longer. CS 4A had better survival than CS 4B. Survival by site was 100% for cervical, 62% for mediastinal, 45% for pelvic, and 36% for retroperitoneal primaries. The role of surgery was evaluated by analyzing survival according to the postoperative PS. PS 1-2-3 A were regarded as satisfactory resections since all macroscopic tumor was removed. PS 3B as a debulking procedure, and PS 3C as an unresectable lesion which was biopsied. Patients with nonmetastatic disease (CS 1-3) with PS 1 and PS 2 disease had a 100% survival rate; PS 3A, 93%; PS 3B, 58%; and PS 3C, 21%. This proves the value of total resection in nonmetastatic disease. The role of surgery could also be proven in metastatic disease.(ABSTRACT TRUNCATED AT 250 WORDS)