Congenital duodenal obstruction: a 32-year review.

Although survival in infants with congenital intestinal obstruction has improved, duodenal obstruction continues to present unique challenges. One hundred thirty-eight newborns and infants (aged 0 to 30 days) were treated for congenital duodenal obstruction. Sixty-five were boys and 73 were girls. Sixty-one (45%) were premature. Forty-six had an intrinsic defect (atresia, web, stenosis, or duplication), 64 had an extrinsic defect (annular pancreas or malrotation with congenital bands), while 28 had various combinations of these. Presenting signs included vomiting (90%, bilious in 66%), abdominal distention (25%), dehydration (24%), and weight loss (17%). Although plain film abdominal x-ray was diagnostic in 58%, upper and/or lower gastrointestinal contrast studies were obtained in 71% of infants to confirm diagnosis. Thirty-eight percent of patients had associated anomalies, including Down's syndrome (11%), cardiac defects, other atresia, other trisomy syndrome, imperforate anus, and central nervous system anomalies. Fourteen patients (10%) had 3 or more other anomalies, many of which required additional surgical therapy. The operative repair of the various defects included Ladd's procedure for malrotation (31%), duodenoduodenostomy (14%), duodenojejunostomy (22%), gastrojejunostomy or gastroduodenostomy (4%), excision of the web and duodenoplasty (3%), or combination of the above (22%). Gastrostomy was placed in 61%. One hundred twenty-eight patients survived (93%). The causes of death were combinations of sepsis, pneumonia, brain hemorrhage, short bowel, and cardiac anomaly. Eight of 10 (80%) who died had other serious anomalies. Twenty patients (14%) required reoperation 5 days to 4 years postoperatively for obstructing lesions (5), wound dehiscence (3), anastomotic leak or dysfunction (6), other atresias (2), choledochal cyst (1), pyloric stenosis (1), and gastroesophageal reflux (2).(ABSTRACT TRUNCATED AT 250 WORDS)

Complement activation during prolonged extracorporeal membrane oxygenation.

Short-term cardiopulmonary bypass activates the complement system, possibly resulting in pulmonary dysfunction from granulocyte aggregation and pulmonary endothelial damage. These effects may be inhibited by steroids. Prolonged extracorporeal membrane oxygenation (ECMO) is used for newborn respiratory failure, but the effects of ECMO on complement activation are unknown. Twenty-one newborn infants with respiratory failure treated with ECMO were randomly assigned to group I (control, no steroids) or group II (30 mg/kg intravenous methylprednisolone before ECMO). Depletion assays of C3 and C5 were performed in each group at intervals before and during ECMO (declining values indicate complement activation). The groups were compared for complement levels, survival, time on ECMO and on the ventilator, and total hospitalization time. Steroids significantly shortened the time on ECMO and time on the ventilator after ECMO but did not affect survival or total hospitalization time. Steroids also enhanced activation of C3 and C5. Complement activation occurs during ECMO. Steroid administration paradoxically causes earlier complement activation but shortens ECMO and ventilator times. Complement activation during ECMO is of questionable significance. The benefits of steroids during ECMO may be mediated through other mechanisms.

Cardiac injury complicating traumatic asphyxia.

During a 3-year period (1986-1989), 8 patients were seen at St. Louis University Medical Center exhibiting the stigmata of traumatic asphyxia. Fewer than 200 cases of traumatic asphyxia have been reported and there is only a single report of a cardiac injury. In this series, 3 of 8 (37.5%) patients were found to have an injury to the heart: two cardiac contusions and a ventricular rupture. Five patients were crushed in motor vehicle collisions, one by an elevator counterweight, and two patients by river barges. Injuries associated with these patients include pulmonary contusion, hemopneumothorax, traumatic pneumatocele, traumatic retinopathy, bone fractures, mental confusion, and liver contusion. There was one death in the series, a patient with rupture of the right ventricle and severe splenic and liver injuries. The cardiac status of the patients was evaluated by serial serum cardiac enzyme determinations, electrocardiograms, and echocardiography. This report illustrates the importance of complete cardiac evaluation in patients with traumatic asphyxia.

Short-bowel syndrome in children. Quality of life in an era of improved survival.

A number of disorders in childhood can result in short-bowel syndrome (small bowel length, less than 100 cm). Improved care has increased survival in patients with short-bowel syndrome, but the quality-of-life factors associated with such improved survival have not been examined, to our knowledge. Sixteen consecutive pediatric patients with short-bowel syndrome (bowel length range, 22 to 98 cm) were followed up for 2 to 10 years. The original diagnoses were as follows: necrotizing enterocolitis (n = 6), multiple intestinal atresias (n = 4), extensive aganglionosis (n = 2), meconium peritonitis (n = 2), and midgut volvulus (n = 2). The range of initial hospitalization was from 62 to 395 days, and 13 of 16 patients have required readmission (two to 14 times). All patients required multiple operations (range, two to 14 operations), including combinations of venous access, adhesiolysis, tapering enteroplasty, reversed intestinal segments, and pull-through procedure. Nine of 16 patients received home total parenteral nutrition, and 12 of 16 patients required home elemental diets, usually via pump feedings. Fifteen patients (94%) survived. Two survivors are deaf, and one of these has mild developmental delay. Seven survivors (age range, 6 to 10 years) attend a regular school, four while receiving total parenteral nutrition or an elemental diet. Ten of 15 survivors are off all nutritional support (including the child with a 22-cm small bowel), with four others weaning. The presence or absence of an ileocecal valve did not affect outcome. Modern nutritional support methods provide excellent survival and quality of life for children with short-bowel syndrome.

Acquired tracheal stenosis in infants and children.

Acquired tracheal stenosis in childhood is frequently difficult to manage because of poor healing, infection, and scarring. In a 10-year period, 62 patients (4 weeks to 14 years of age) were treated for acquired tracheal stenosis. The causes of stenosis were endotracheal intubation (44 patients), caustic aspiration (6 patients), recurrent infection (5 patients), bronchoscopic perforation (4 patients), and gastric aspiration (3 patients). The subglottic or upper trachea was involved in 47 patients, mid portion in 8, and distal or carinal area in 7. Fifty children underwent tracheostomy as part of the therapy, and 12 were managed without tracheostomy. Therapy was individualized, frequently sequentially, utilizing rigid or balloon dilatation (20 patients), bronchoscopic electrocoagulation resection (44 patients), steroid injection (48 patients), T tube stent (8 patients), resection with anastomosis (12 patients), cricoid split (3 patients), and rib cartilage graft (12 patients). Most patients required several techniques and repeated procedures to eventually achieve decannulation. Seven patients (11%) died of unrelated causes. Forty-four of 55 surviving patients (80%) are without tracheostomy, although 14 have required continued endotracheal treatment after tracheostomy removal (dilatation, endotracheal resection). This series demonstrates that acquired tracheal stenosis in childhood is a common, difficult problem, but manageable with the use of a variety of techniques. Resection and grafting procedures should be reserved for cases in which less complex modalities fail.

Congenital tracheal stenosis with unilateral pulmonary agenesis.

Cogenital tracheal stenosis with unilateral pulmonary agenesis is a rate and frequent fetal combination. In an 8-year period, 5 infants (ages 2 to 6 months) with these anomalies were treated. The presenting signs and symptoms consisted of wheezing, stridor, and tachypnea and included frank respiratory failure requiring emergency therapy in several patients. The operative repair consisted of segmental resection and anastomosis in one patient, and rib-cartilage tracheoplasty in the other four. Two infants died, one of cerebral hypoxia, and the other of aortotracheal fistula. Long-term follow-up in the three survivors is satisfactory.

Prognostic determinants in extracorporeal membrane oxygenation for respiratory failure in newborns.

Extracorporeal membrane oxygenation (ECMO) is becoming an accepted therapeutic modality for newborn respiratory failure, but there is little information available regarding the prognostic determinants with this technique. One hundred thirty-five newborns treated with ECMO over a 4-year period were critically analyzed with regard to the influence that birth weight, gestational age, age at initiation of ECMO, best blood gases before ECMO, number of hours on ECMO, renal failure, intracerebral hemorrhage, and long-distance air transport had on survival. Infants with meconium aspiration and those undergoing long-distance transfer showed significant differences in blood gases before ECMO, with survivors having more normal pH and carbon dioxide tension values. Intracerebral hemorrhage and renal failure that developed during ECMO were grave prognostic signs, with few survivors in either group. These data show that ability to ventilate patients before ECMO, giving normal carbon dioxide tension and pH values, is an important prognostic sign in infants with meconium aspiration and undergoing long-distance transfer for ECMO, whereas renal failure and intracerebral hemorrhage are usually lethal complications of ECMO. Each center performing ECMO should continually reevaluate this invasive technique and its results and complications.

Congenital diaphragmatic hernia repair on ECMO.

Congenital diaphragmatic hernia (CDH) with severe respiratory failure in the first few hours of life continues to be associated with significant mortality. Extracorporeal membrane oxygenation (ECMO) has been successfully used postoperatively to reverse the effects of severe pulmonary hypertension. Since 1984, ECMO has been required in 27 of the patients we treated with CDH. This report describes our experience with six very high-risk patients placed on ECMO prior to the operation who subsequently underwent repair of their diaphragmatic hernias while on ECMO. Two patients presented in extremis, unlikely to survive initial operative repair, and were placed on ECMO prior to the operation. All six patients had immediate respiratory distress after birth with mean Apgars of 2.3 and 3.7. The best pre-ECMO arterial blood gas (postductal) showed mean +/- SEM values of 6.97 +/- 0.1; PO2 = 54.8 +/- 5.9; PCO2 = 79.5 +/- 16.9. Immediately prior to ECMO, the mean +/- SEM ventilatory index (VI = rate x mean airway pressure) was 1,233 +/- 44, with a mean pH of 7.17 +/- 0.05; PO2 = 32 +/- 2.9; PCO2 = 59 +/- 5.3 and a mean AaDO2 of 622 +/- 4.8. The timing of the operative repair averaged 25 hours following initiation of ECMO. Three right-sided and three left-sided hernias were treated. Four were repaired through an abdominal approach, and two via thoracotomy; four required a Gortex patch closure. Postoperative bleeding was not a major problem in these heparinized patients. Four of these six patients survived, and follow-up of 2 months to 3 years shows no significant respiratory compromise.(ABSTRACT TRUNCATED AT 250 WORDS)

Extracorporeal membrane oxygenation for hydrocarbon aspiration.

Extracorporeal membrane oxygenation is a pulmonary bypass procedure that has been employed in adults to provide temporary treatment for reversible acute pulmonary and cardiac insufficiency. The technology of membrane oxygenation has been used since 1977 in neonates with predictably fatal pulmonary failure due to respiratory distress syndrome, persistent fetal circulation or persistent pulmonary hypertension of the newborn, meconium aspiration syndrome, and congenital diaphragmatic hernia. The use of extracorporeal membrane oxygenation in older children with other pulmonary disorders has been limited. We report two cases of hydrocarbon aspiration involving petroleum-based products, both successfully treated with extracorporeal membrane oxygenation. A 15-month-old male infant who aspirated baby oil (light mineral oil) is particularly unusual owing to the generally expected low risk of aspiration with a hydrocarbon of such viscosity (greater than 60 Saybolt Universal Seconds). The second patient is a 16-month-old male infant who aspirated furniture polish (mineral seal oil). In both children severe intractable hypoxemia developed despite intensive ventilatory support, and they became candidates for alternative therapy. Extracorporeal membrane oxygenation provides a potentially life-saving option when a patient fails to respond to conventional therapy for hydrocarbon aspiration.

Complex hemangiomas of infants and children. Individualized management in 22 cases.

Large hemangiomas in infants and children are rare but can be life-threatening if they involve vital structures or produce thrombocytopenia or congestive heart failure. During a 6-year period, 22 infants and children, aged newborn to 7 years, were treated for complex, symptomatic hemangiomas. The lesions were located in the liver in seven, face or parotid gland in five, neck in four, extremity in two, and mediastinum, chest wall-spinal cord, trachea, and retroperitoneum in one patient each. The diagnosis was suggested by physical examination in all patients and was confirmed by radiologic examination in most patients. The treatment was individualized, usually progressed from less to more invasive, and included observation, prednisone therapy, arterial ligation, and resection. All children were eventually cured, with minimal morbidity. Children with life-threatening hemangiomas can be successfully managed with the use of a variety of techniques.

Congenital bronchopulmonary malformations. Diagnostic and therapeutic considerations.

Congenital bronchopulmonary malformations are uncommon but potentially life-threatening anomalies of infants and children. Between 1970 and 1988, 45 patients from birth to 13 years of age (23 boys and 22 girls) underwent evaluation and treatment for bronchopulmonary malformations. Thirty-seven had solitary lesions: bronchogenic cyst (n = 13), cystic adenomatoid malformation (n = 9), congenital lobar emphysema (n = 6), pulmonary sequestration (n = 6), arteriovenous malformation (n = 2), and bronchial atresia (n = 1). Eight additional patients had two simultaneous abnormalities and three patients had congenital diaphragmatic hernias. Twenty-one patients had respiratory symptoms, which were severe in seven. Twelve had pulmonary infection and 10 patients were completely free of symptoms. Plain chest roentgenogram was the only diagnostic imaging performed in 11 patients. Thirteen patients underwent computed tomographic scan, but in only four was it essential for diagnosis. Prenatal ultrasonography in three patients demonstrated cystic adenomatoid malformation in two, with one false negative study. Postnatally, ultrasonography was also useful in establishing the diagnoses of cystic adenomatoid malformation and pulmonary sequestration. Thoracotomy with excision of the lesion by lobectomy or pneumonectomy resulted in survival of 42 patients (93%). Three deaths in neonates were due to pulmonary hypoplasia and hypertension. Two of them had concomitant diaphragmatic hernia; the other had a cystic adenomatoid malformation and died despite the use of postoperative extracorporeal membrane oxygenation. These data demonstrate that congenital bronchopulmonary malformations usually can be diagnosed by plain chest x-ray films. Ancillary studies such as ultrasonography or computed tomography may occasionally be necessary. Combinations of the different types of bronchopulmonary malformations occurred frequently. All lesions, including symptomatic lesions in neonates, can be managed surgically soon after diagnosis.

Changing spectrum of cholelithiasis and cholecystitis in infants and children.

Cholecystitis and cholelithiasis are infrequent in children and have been historically associated with adolescent pregnancy or hemolytic disorders; however, the incidence and spectrum of cholelithiasis seem to be changing. Between 1970 and 1988, 47 children 17 years of age or less underwent cholecystectomy for cholecystitis or cholelithiasis in our hospital. The patients were divided into chronologic groups: Group 1 encompassed 1970 through 1979 (15 patients) and group 2, 1980 through 1988 (32 patients). The groups were compared for age, sex, pregnancy, blood dyscrasia, family history, obesity, use of total parenteral nutrition (TPN), and incidence of choledocholithiasis with its sequelae. A significant increase in the number of patients with cholelithiasis was found. Infants and young children were affected more frequently in group 2, and many of these young patients had a history of TPN. Choledocholithiasis was also more common in group 2 and presented with life-threatening sequelae. Calculous biliary tract disease should be considered as a possible cause of abdominal pain in children. Timely operative intervention can prevent the increasingly common sequelae of childhood cholelithiasis.

A critical analysis of extracorporeal membrane oxygenation for congenital diaphragmatic hernia.

Despite advances made in the care of infants with congenital diaphragmatic hernia (CDH), survival remains poor. New therapy, such as extracorporeal membrane oxygenation (ECMO), is controversial but may improve survival. Thirty-two newborns with CDH were treated. Thirteen infants were treated with jugular vein-carotid artery ECMO after CDH repair elsewhere; six (46%) survived. Three of the remaining 19 were moribund shortly after birth and first received ECMO, then underwent repair; two (67%) survived. The other 16 underwent CDH repair; 8 of 9 (89%) recovered with conventional therapy, and 4 of 7 (57%) survived when ECMO was used after conventional therapy failed. Overall survival was 63%. Parameters with which physicians may attempt to predict survival or the need for ECMO after repair--such as A-aDO2, ventilatory index versus PCO2, presence of a "honeymoon period" (PaO2 greater than 100 mm Hg after repair), or oxygenation index--were unreliable. ECMO can improve survival in infants with CDH, probably through reversal of pulmonary hypertension. Presently available methods of predicting survival after CDH repair with or without ECMO are not accurate, and thus no infant should be excluded from repair or ECMO support.

Malignancy associated with ureterosigmoidostomy: detection by mucosa ornithine decarboxylase.

Urinary diversion into the gastrointestinal tract (ureterosigmoidostomy) is associated with stepwise malignant degeneration of colonic mucosa. Early detection of such malignancy can be difficult. Ornithine decarboxylase (ODC) is an enzyme that initiates polyamine synthesis that is elevated in malignant colonic mucosa, but its level in premalignant mucosa after ureterosigmoidostomy is unknown. Ten Wistar rats underwent urinary diversion (bladder trigone to sigmoid colon), and were maintained on a regular diet with antibiotics for 6 months, then killed. All animals developed metaplastic changes histologically at the anastomosis. Mean ODC levels of colonic mucosa at the anastomosis v normal colon 8 cm proximal were 515 +/- 177 pmole v 24.5 +/- 4.4 (P less than .01). These data show that premalignant changes in colonic mucosa after ureterosigmoidostomy can be detected by elevated colonic biopsy ODC levels. Periodic sigmoidoscopy with colon mucosa biopsy for histology and ODC levels in children with ureterosigmoidostomy is recommended.

Exposure of the anterior spine. Technique, complications, and results in 85 patients.

The anterior approach to the spine is necessary for correction of some congenital spinal deformities and other conditions, including spinal trauma, infection, and tumor. The morbidity associated with this procedure has not been extensively reviewed in the literature. Between 1981 and 1986, 85 patients (41 male and 44 female) aged 1 to 77 years underwent anterior spinal fusion by an orthopedic or general surgery team (33 pediatric patients and 52 adult patients). Thirty-four patients had scoliosis, 8 had kyphosis, 24 had spinal trauma, 9 had tumor, and 10 had infection. Fifteen patients had restrictive lung disease diagnosed by pulmonary function testing (10 children and 5 adults). The thoracoabdominal approach was used in 50 patients, thoracotomy in 22 patients, and the lumbar approach in 10 patients. Two incisions were used in three patients. Correction was accomplished by interbody fusion in 36 patients (17 with instrumentation) and strut graft in 49 patients (6 with instrumentation). Twelve strut grafts were vascularized ribs and 37 were free ribs. Eighty-two patients survived (96 percent). Seventy-four complications occurring in 50 patients all resolved prior to discharge. These included 28 pulmonary complications, 27 urinary complications, and 5 gastrointestinal complications. Three patients required prolonged mechanical ventilation. Solid fusion was seen in 78 of 85 patients, whereas pseudoarthrosis developed in 7.

The effects of perfluorochemicals on tumor growth and chemotherapy response.

Hypoxia, leading to cells in resting or slow replication phases may be a cause of chemotherapy and radiation therapy resistance in some tumors. Perfluorochemicals (PFC) may potentiate response to these therapies by increasing oxygen delivery to the tumor, forcing cells into more therapy-responsive replicating phases. To assess the effects of PFC on tumor growth and chemotherapy response, 91 ACI rats bearing 1 cc flank Morris hepatoma tumors were divided into groups: Group I, control; Group II, Adriamycin (ADR) 10 mg/kg intraperitoneally (IP); Group III, Cytoxan (CTX) 100 mg/kg IP; Group IV, PFC 20 mL/kg IV; Group V, ADR and PFC; Group VI, CTX and PFC. Animals were kept in 0.5 FiO2 for 24 hours after treatment, and mortality and tumor volumes determined 2 weeks later. Tumor DNA turnover was measured using opposing pathways assay of 14C-thymidine uptake and degradation. In a separate group, tumor tissue pO2 was measured polarographically with an oxygen microelectrode before and after injection of PFC (20 mL/kg). The survival was significantly reduced in group IV (4%) compared with group I, control (73%). Both ADR and CTX slowed the growth of the tumor, while PFC alone significantly accelerated tumor growth. The tumor response to ADR was potentiated by the addition of PFC. These results were confirmed by DNA synthesis evaluation. The mean pO2 level prior to injection was 6.6 +/- 1.96 mmHg compared with 18.92 +/- 1.00 mmHg after PFC injection (P less than or equal to .01).(ABSTRACT TRUNCATED AT 250 WORDS)

Neonatal diaphragmatic hernia. An improving outlook with extracorporeal membrane oxygenation.

In a 15-year period, 89 newborns were treated for congenital diaphragmatic hernia. The patients were divided into three groups, depending on postoperative therapeutic support available: group 1, ventilator therapy only; group 2, ventilator therapy plus pulmonary vasodilators (tolazoline hydrochloride); and group 3, ventilators, tolazoline, and/or extracorporeal membrane oxygenation (ECMO). The three groups were identical for presenting symptoms, signs, and preoperative blood gas determinations. The survival for each group was as follows: group 1, 17 (40%) of 42; group 2, 14 (45%) of 31; and group 3, 12 (75%) of 16. Complications requiring further operations were identical. All survivors in groups 1 and 2 are normal developmentally, while one of five group 3 ECMO survivors has developmental delay and another has long-term ventilator dependence. These data suggest that ECMO, an invasive technique for newborn respiratory failure, improves survival in congenital diaphragmatic hernia.

Operative technique and results of subtotal splenectomy for Gaucher disease.

Patients with type 1 (adult form) Gaucher disease complicated by massive splenomegaly and hypersplenism have previously been treated by total splenectomy. Subtotal splenectomy in Gaucher disease will retain splenic tissue which may protect the patient from the risk of overwhelming sepsis. Removal of at least 85 per cent of the splenic mass is necessary to eliminate hypersplenism and to decrease splenic size. The surgical technique and outcome of four patients undergoing subtotal splenectomy for massive splenomegaly due to type 1 Gaucher disease are discussed.