Stephen K. Greenholz

Contemporary surgery of tracheomalacia

During the past 7 years, 41 infants were treated for tracheomalacia. The etiology was primary/congenital in 28 patients and secondary/acquired in 16 patients, of which three patients were originally in the primary group. The primary group consisted of patients with pulsatile tracheomalacia but normal vascular anatomy, idiopathic disease, or tracheomalacia associated with tracheoesophageal fistula. The secondary group consisted of patients with tracheal compression due to great vessel abnormalities, or tracheostomy-tracheomalacia. Diagnosis was made by bronchoscopic demonstration of major (greater than 40%) collapse of the trachea. Treatment consisted of conservative therapy, tracheostomy, aortopexy, or tracheal reconstruction. The 15 patients with mild primary tracheomalacia treated conservatively had gradual resolution of symptoms by the age 2 years. All five patients with primary tracheomalacia treated by tracheostomy developed secondary tracheomalacia and/or cicatrix at the tracheostomy site. In the 9 patients with primary tracheomalacia treated by aortopexy, 5 are symptom free, 1 is improved, 1 had recurrent apnea, and 2 died, 1 from unrelated complications. Of the 10 patients in the acquired group treated by aortopexy, 6 were cured, 2 were improved, and 2 failed. Of 6 patients with tracheostomy-tracheomalacia, 3 were eventually extubated, 1 had major reconstruction, and 2 had tracheostomies when lost to follow-up at 1 and 5 years. Our conclusions are that, when feasible, conservative therapy in milder cases is preferred, and no perfect operation currently exists for severe tracheomalacia although aortopexy may have less long-term morbidity than tracheostomy.

Surgical implications of bronchopulmonary dysplasia

Twenty of 30 patients with bronchopulmonary dysplasia (BPD) had major tracheobronchial abnormalities, which in 18 could be incriminated as contributing to their symptoms. There were 15 examples of tracheobronchial stenosis, 13 of tracheomalacia, nine of bronchomalacia, and one of tracheal web. Sixteen patients had operations. Tracheostomy was successful as a temporizing measure in ten patients although there was significant morbidity. Balloon dilatation of tracheobronchial stenosis produced temporary (2) or long-term (2) improvement in four patients. Electroresection of tracheobronchial stenosis was successful in the three instances it was employed. Lobectomy for lobar emphysema was curative in both patients. Aortopexy for tracheomalacia improved all three patients. We conclude that (1) symptomatic major airway lesions are not uncommon manifestations of BPD, (2) many of the lesions are amenable to surgical therapy, and (3) bronchoscopic evaluation should be considered early in the course of infants with BPD.

Meconium obstruction in the markedly premature infant

Markedly premature infants may present with intestinal obstruction and perforation secondary to inspissated meconium in the absence of cystic fibrosis. Between 1990 and 1994, 13 patients were treated for intestinal obstruction secondary to inspissated meconium. The average birth weight was 760 g. Prenatal and postnatal risk factors were identified, and included intrauterine growth retardation, maternal hypertension, prolonged administration of tocolytics, patent ductus arteriosus, hyaline membrane disease, and intraventricular hemorrhage. Stooling was absent or infrequent during the first 2 weeks of life. Surgical presentation consisted of distension and/or perforation between days 2 and 17 of life. Twelve patients required operative intervention. Findings invariably included one or more obstructing meconium plugs with proximal distension and frequent necrosis of the dilated segments. Surgical options consisted of resection or enterotomy, accompanied by primary closure or by distal irrigation and exteriorization. Irrigation led to iatrogenic bowel injury in two patients. One patient was managed successfully with oral and rectal gastrograffin and oral acetylcysteine. Ten patients were discharged, all of whom had normal stooling patterns and tested negatively for cystic fibrosis. Three patients died, two from the primary disease. The markedly premature infant is at risk for obstruction and eventual perforation secondary to meconium plugs, presumably formed in conjunction with intestinal dysmotility. Prompt diagnosis and timely intervention require a high index of suspicion, attention to stooling patterns and abdominal examinations, and screening radiographs when indicated.

Outpatient inguinal herniorrhaphy in premature infants: is it safe?

Because postoperative apnea and bradycardia in premature infants following inguinal herniorrhaphy remains a concern, outpatient repair has not been recommended. We have been performing outpatient inguinal herniorrhaphy in premature infants and the present study reviews our experience. Between 1985 and 1990, 1,294 outpatient inguinal herniorrhaphies were performed. Of this group 124 patients (9.6%) were identified as being premature (less than or equal to 36 weeks gestational age). Average ages were: gestational age 32.7 weeks (range, 24 to 36 weeks); postnatal age 12.6 weeks (range, 3 to 24 weeks); and postconceptional age (gestational plus postnatal) 45.3 weeks (range, 34 to 59 weeks). Twenty-two infants previously required ventilatory support, 11 patients had apnea/bradycardia, and 9 patients developed bronchopulmonary dysplasia. General anesthesia (usually nitrous oxide and fluothane) was used in all patients and 75% underwent endotracheal intubation. The average operating room time was 40 minutes (range, 20 to 115 minutes) and the average recovery room time was 94 minutes (range, 30 to 240 minutes). There were no perioperative deaths. One patient became apneic immediately after extubation in the operating room. No further episodes were noted after 4 hours of observation. Another patient following discharge had a brief apneic episode at home while on an apnea monitor, which was relieved with gentle stimulation. Both patients had no further sequelae. Bradycardia to 80 beats/min was noted in two patients, and resolved spontaneously in the recovery room. Laryngospasm after extubation in the operating room occurred in two patients, one of whom required brief reintubation and the other resolved spontaneously. Two patients required postoperative ventilation: one was extubated in the recovery room and the other was hospitalized for 24 hours.(ABSTRACT TRUNCATED AT 250 WORDS)

Esophageal Endosclerosis in Children

During the past 6 years, 25 consecutive patients with esophageal variceal hemorrhage were treated by esophageal endosclerosis (direct injection of varices with a sclerosing agent). The primary disease in the 25 children was portal vein thrombosis (11 patients), biliary atresia (nine patients), and hepatic cirrhosis from cystic fibrosis (three patients), alpha 1-antitrypsin deficiency (one patient), and neonatal hepatitis (one patient). Thirteen patients were treated during acute, major variceal hemorrhage. Esophageal endosclerosis was repeated at regular intervals until all esophageal varices were obliterated. Twenty-one patients completed therapy. Four patients died: one of a complication of therapy and three of the primary disease. Other than the one death, complications were minor. Recurrent esophageal variceal hemorrhage has not been encountered in follow-up from 9 months to 6 years after completion of therapy.

Balloon dilatation of postoperative tracheal stenosis.

Three patients who had previously undergone linear augmentation tracheoplasty developed postoperative stenosis within the grafted segment. In each patient, balloon catheter dilatation successfully enlarged the stenotic airway obviating secondary reconstruction in two.

Infants undergoing pyloromyotomy are not at risk for postoperative apnea

Thirty term infants undergoing general anesthesia and pyloromyotomy had pre‐ and postoperative sleep studies to determine whether these infants were at risk for postoperative apnea.

Biliary obstruction in infants with cystic fibrosis requiring Kasai portoenterostomy

Cystic fibrosis is associated with an inspissated bile syndrome producing cholestasis secondary to plugging of macroscopically normal bile ducts. In extreme neonatal forms, with early profound intrahepatic cholestasis, the process can be associated with a marked decrease in ductal diameter, varying from hypoplasia to atresia. From 1990 to 1995 three infants were identified with cystic fibrosis, persistent jaundice, and complete absence of biliary excretion despite expectant and conservative treatment including choleretics and surgical biliary irrigation. Abdominal ultrasounds showed contracted gallbladders and no evidence of dilated ducts. Liver biopsy results in two infants showed portal fibrosis, paucity of bile ducts, and minimal inflammation. The third infant had moderate inflammation, bile duct replication, and plugging. Two infants had undergone intestinal resection followed by hyperalimenation for complications of meconium ileus in the newborn period. Surgical exploration was undertaken at 7 to 12 weeks of age. Gross findings were typical of biliary atresia with microgallbladders and nonpatency of the cystic duct. Cholangiograms failed to document ductal patency in two patients who were then treated with a Kasai portoenterostomy. The third infant had patent hypoplastic ducts and underwent only biliary irrigation. Although bile flow was transiently achieved, jaundice recurred, and at reexploration at 16 weeks of age a Kasai poroenterostomy was performed. Histological review of the biliary specimens showed microscopically patent ducts in two patients and proximal patency and distal atresia in the third. All the ducts had mural fibrosis with cystic changes. Bile drainage was achieved in each instance, although in one patient with hypoplastic ducts scant output of highly concentrated bile proved insufficient to arrest progressive liver failure. The subsequent two patients responded with resolution of hyperbilirubinemia and normalization of liver function. They remain free of biliary complications at 30 and 40 months postoperatively. This manifestation of cystic fibrosis in infants is suggested by prolonged jaundice unresponsive to choleretics, nondilated bile ducts and gallbladder on ultrasound, absent biliary excretion on nuclear scan, and characteristic liver biopsy. Exploration is warranted, and discovery of atrophic bile ducts may be best managed with reconstruction.

Manometric and pH consequences of esophageal endosclerosis in children

Twenty-seven manometric and 22 18-hour pH monitoring studies were done in 17 consecutive patients undergoing esophageal endosclerosis. Prior to endosclerosis, esophageal manometry was normal in eight of nine patients. Peristaltic dysfunction was observed in all 13 postendosclerosis patients and consisted of (1) decreased mean peristaltic amplitude pressures. Pressures were 61.4 and 74.7 mmHg at 0 to 2 cm and 3 to 4 cm above the lower esophageal sphincter (LES), respectively, prior to treatment. After endosclerosis, values fell to 30.2 and 43.3 mmHg; (2) a drop in mean resting LES pressure from 22.3 mmHg before endosclerosis to 17.1 mmHg afterward and (3) an increase in the rate of peristaltic propagation failure from 12% to 26% after endosclerosis. Esophageal pH monitoring demonstrated gastroesophageal reflux (GER) in three of seven patients before endosclerosis and in five of 11 patients afterward. Because of the major incidence of GER before endosclerosis, the procedure could not be causally incriminated. However, GER was roughly correlated with the severity of the manometric dysfunction. There was no correlation of GER or manometric abnormality with the number of endosclerosis treatment nor the interval between endosclerosis and pH and manometric studies. The remote sequelae of the abnormalities are conjectural.

Multifocal small bowel obstruction in an infant

This is a case report of an acute small bowel obstruction in a 17-month-old girl with no significant past medical history. Computerized tomography (CT) of the abdomen demonstrated complete obstruction at the proximal jejunum as well as perplexing dilation and air-fluid levels within the distal jejunum beyond the initial obstruction. Imaging demonstrated no evidence of malrotation, volvulus, or intussusception, but a clear diagnosis was not evident. Exploratory laparotomy was performed and multiple foreign bodies that appeared to be rubber balls were found within the small bowel. This case study identifies important clinical and radiologic considerations when assessing infants for small bowel obstruction.