Advancement flap umbilicoplasty after abdominal wall closure: postoperative results compared with normal umbilical anatomy.

BACKGROUND/PURPOSE: Umbilical reconstruction frequently is an adjunct used after repair of congenital abdominal wall defects. The authors describe a new technique of umbilicoplasty and compare their results with normal neonatal umbilical anatomy. METHODS: After fascial closure, 6 newborn infants with either gastroschisis (n = 3) or omphalocele (n = 3) underwent umbilicoplasty performed by advancing bilateral skin flaps including creation of an umbilical skin collar. The umbilical collar height, circumference, and distance from the xiphoid with respect to the pubis (ratio of xiphoid-umbilicus distance to xiphoid-pubis distance or XU:XP) were recorded and referenced to normal umbilical anatomy in additional 28 infants (24 to 40 weeks gestation [mean, 33.2 weeks] and age 2 to 131 days [mean, 26.5 days]). RESULTS: After umbilicoplasty, all infants had a near-normal-appearing umbilicus (collar height, 0.75 +/- 0.25 cm and circumference, 5.24 +/- 1.20 cm) compared with normal umbilical anatomy (collar height, 4.36 +/- 1.32 cm and circumference, 1.03 +/- 0.38 cm). However, after umbilicoplasty, the neoumbilicus was positioned more cephalad (XU:XP = 0.53 +/-.05) compared with normal (mean XU:XP, 0.67 +/- 0.07). The only complication encountered was a small dehiscence of the superior aspect of the abdominal wound. CONCLUSIONS: Closure of abdominal wall defects with simultaneous umbilicoplasty provides a cosmetically pleasing result. When performing umbilicoplasty, attempt should be made to position the neoumbilicus as inferior as possible, optimally at two thirds the distance from the xiphoid to the pubis.

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.

Reconstruction of the toddler diaphragm in severe anterolateral congenital diaphragmatic hernia with the reverse latissimus dorsi flap.

The management of infants with severe congenital diaphragmatic hernia (CDH) continues to evolve. When a prosthetic patch is placed in the neonatal period for pleuroperitoneal separation, it ultimately will require a subsequent reconstruction for progressive pulmonary or abdominal symptomatology. The reverse latissimus dorsi (RLD) flap has been used for reconstruction in only several reports in the last 12 years. In this paper, a patient with severe anterolateral CDH is reconstructed with the RLD flap on an elective basis at 2 years of age. Elective repair was performed for the particular indication of chest wall restriction imposed by the nonpliable Gore-Tex patch. In this case, use of the RLD flap alone without the use of synthetic mesh has resulted in satisfactory results with 17 months of follow up.

Congenital diaphragmatic hernia: an overview.

Understanding the complex multisystem dysfunction in the infant with a congenital hernia of the posterolateral diaphragm is still evolving and has changed radically during the last decade. The reduction in lung mass, in conjunction with surfactant deficiency and diminished compliance, leads to initial deficiencies in oxygenation and carbon dioxide (CO2) removal. This may then be potentiated by an extremely reactive hypoplastic pulmonary arterial system. Treatment no longer is focused on the operative repair but rather on the components of the pathophysiological process that are potentially reversible. Thus, extracorporeal membrane oxygenation and delay of repair until resolution of pulmonary artery hypertension have become mainstays of therapy and are probably responsible for increasing the survival rate in the patient who presents early with respiratory distress from 50% to 65%. Still far from acceptable, these results are giving impetus to new approaches to therapy including drugs such as nitric oxide, fetal intervention including open repair, and lung transplantation.

Meconium obstruction in 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)

The surgery of biliary atresia.

One hundred thirty-one consecutive infants with biliary atresia were operated on during the 15-year period between 1973 and 1988. Six patients did not have biliary reconstruction because of advanced cirrhosis or transplant preference. The other 125 infants had excision of all nonpatent extrahepatic bile ducts; biliary drainage was provided by a gallbladder-common bile duct conduit in 14 patients and by a Roux-en-Y portoenterostomy in 111 infants (including the seven patients with correctable biliary atresia). The bilioenteric conduit was temporarily exteriorized and, for the past 2 years, a conduit intussusception valve was incorporated. Immediate postsurgical bile drainage was achieved in 103 infants (82%). Reoperation during the first 6 postoperative weeks restored bile flow in 14 of 18 infants who had shut down. Seventy-two patients (57%) had sustained (more than 1 year) relief of biliary obstruction. Postoperative morbidity was substantial. The six children not having corrective surgery died within 19 months. Three patients were lost to follow-up. Sixty-eight patients having Kasai's operation died, 55 from complications of liver disease, 1 from a coexisting malformation, and 12 after liver transplantation. Fifty-seven patients are alive, 13 by virtue of liver replacement, 9 with mild-to-moderate hepatic sequelae, and 35 (28%) with normal to near-normal liver function. Although none is considered "cured," the 35 children are anicteric, have normal growth and development, and participate in full school activities (including contact sports). Average follow-up is 85.8 months (range 1 to 15 years).

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.

Development of a program for planned cesarean delivery and immediate pediatric surgical repair of ventral wall defects.

The development of a program for timed elective delivery and immediate repair of ventral wall defects is described. Our experience with 3 cases of immediate surgical repair of gastroschisis shows that this program can be applied in tertiary care centers to shorten pre- and postoperative stay and to present the neonate to surgery in optimal condition.

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.

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.

Biliary atresia in the newborn.

A prenatal sonographic diagnosis of extrahepatic biliary atresia was made and, 76 hours after birth, operatively confirmed. A standard Kasai operation was performed, with the exception of the use of an ancillary appendiceal conduit to provide biliary drainage of an independent bile duct draining the right anterior hepatic segment. Hepatic and ductal histology were identical to those usually found in biliary atresia in a 6- to 8-week-old infant. The child is well at 16 months.

Traumatic bilateral renal artery occlusion: successful outcome without surgical intervention.

Traumatic bilateral renal artery thrombosis is rare, and attempts to reestablish life-sustaining renal function have had dismal results. This case describes a successful outcome of such an injury, the gradual return of adequate renal function without surgical intervention. This experience raises an argument against both strict limitation of the acceptable time interval between injury and revascularization, and early nephrectomy.

Natural history of untreated inferior vena cava injury and assessment of venous access.

Conventional policy mandates exploration of retroperitoneal hematomas presumed to contain penetrating injuries of the inferior vena cava (IVC). In clinical practice this maneuver frequently results in torrential blood loss, suggesting the injury may be best managed without exploration and IVC suturing. The present study was undertaken to define the natural history of untreated IVC perforations, and to assess venous access with such injuries. Twelve adolescent Hampshire pigs (20-25 kg) underwent halothane anesthesia and laparotomy. The infrarenal IVC was isolated temporarily and a posterior cruciate incision, equivalent to 50% of the IVC circumference, made via anterior venotomy. Animals were resuscitated with Ringer's lactate. Technetium sulfur colloid was injected 20 min postlaparotomy closure via either a jugular or femoral venous cannula, and count activity measured over the IVC injury site using a mobile gamma camera. The procedure was repeated via the counterpart limb. Background from the first injection was subtracted, and count ratios integrated from 16 to 19 min postinjection. Extravasation following lower extremity infusion was not significantly different from that following central venous administration. One pig succumbed to cryptogenic peritonitis one wk postinjury. The remaining animals were killed at periods ranging from 3 to 12 wk. No animal had clinical evidence of delayed bleeding, pulmonary embolus, or other venous sequelae during the observation period. At autopsy all vena cavae were normal caliber without evidence or pseudoaneurysm of thrombosis. This swine study confirms the efficacy of lower extremity venous access with contained IVC injury, and suggests that isolated retroperitoneal IVC injuries may be treated nonoperatively in very selected patients.

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.