Predictive capabilities of preoperative and postoperative pulmonary function tests in delayed repair of congenital diaphragmatic hernia.

To improve the survival of newborns with congenital diaphragmatic hernia (CHD), preoperative stabilization with conventional ventilatory therapy and extracorporeal membrane oxygenation (ECMO) have been used. Measurements that quantify pulmonary function may allow an accurate assessment of lethal pulmonary hypoplasia and predict outcome. Pulmonary function tests (PFTs) were obtained in 20 infants preoperatively and postoperatively; these included measurements of compliance, dynamic compliance, and tidal volume. Overall survival was 75%. Six surviving infants were initially managed with ventilator therapy alone, followed by repair (group 1). The remaining 14 patients, who were moribund at presentation or whose initial ventilator therapy failed, were placed on ECMO and received repair during bypass; nine survived (group 2), and five died (group 3). Compliance, dynamic compliance, and tidal volume obtained at initial presentation and immediately preoperatively were significantly higher for group 1 as compared with groups 2 and 3. Infants whose initial compliance was greater than 0.25 mL/cm H2O/kg and initial tidal volume was greater than 3.5 mL/kg did not require ECMO. Ultimate improvement in compliance was noted in 5 of 6 patients in group 1, 8 of 8 patients in group 2, and 5 of 5 in group 3. This improvement followed an initial decline in compliance in 9 of 14 survivors, from 15% to 76%. All six patients in group 1 had tidal volumes of more than 4 mL/kg, as did 7 of 9 patients in group 2. Only one patient among the ECMO nonsurvivors (group 3) had a postoperative tidal volume of this magnitude. These data suggest that initial PFTs may predict which infants will require ECMO.(ABSTRACT TRUNCATED AT 250 WORDS)

Congenital hemangiopericytoma: an unusual vascular neoplasm of infancy.

BACKGROUND: Hemangiopericytoma is an uncommon tumor of infants, which originates from the vascular pericytes. Although generally considered to benign, metastases can occur. METHODS: Five cases of congenital hemangiopericytoma were seen in infants; all were found in females. The mean age at diagnosis was 7 weeks (range, birth to 10 weeks). The lesions were located in the neck, the parotid, the axilla, and the retroperitoneum. One neck lesion was detected prenatally by ultrasonography. Each lesion was resected. The diagnosis of congenital hemangiopericytoma was established only after histologic examination. RESULTS: No evidence of recurrence has been found in four of the children. However, intrathoracic and intracranial metastases developed in one child with a neck lesion 28 months after the original resection, and the child was treated with chemotherapy, but she died of progressive disease. CONCLUSIONS: We advocate the consideration of congenital hemangiopericytoma in the newborn infant with a vascular mass. Because congenital hemangiopericytoma is unresponsive to steroid therapy, unlike other vascular malformations, resection is the treatment of choice. Long-term postoperative follow-up is essential for the early detection of metastases.

Growth-related gene expression in early cholestatic liver injury.

BACKGROUND: Extrahepatic biliary obstruction initiates cholestasis, bile duct proliferation, periportal fibrosis, and, eventually, lethal biliary cirrhosis. Little is known about the genetic regulation of the cellular proliferation and differentiation that begins with the onset of bile duct obstruction. To focus this and future gene expression studies, we sought to determine the time frame for growth-related gene expression and questioned whether the in vivo expression of the protooncogenes H-ras and c-myc was altered after bile duct obstruction. METHODS: Female Fisher rats underwent ligation and division of the common bile duct or sham laparotomy. RESULTS: After obstruction, serum bilirubin and gamma-glutamyl transpeptidase rose to 24% and 30%, respectively, of maximum levels by 10 days after ligation. Morphologic evidence of proliferation of bile duct epithelial cells was first evident after 3 days. After hybridization to c-DNA probes, densitometry for H-ras and beta-actin revealed an immediate and parallel increase in steady-state levels of expression after 24 hours of cholestasis. Levels of c-myc messenger RNA were elevated during the first 3 days of cholestasis; however, at 7 and 10 days c-myc expression was depressed 16% and 60%, respectively. CONCLUSIONS: These profiles of expression show an oncogene response induced by early cholestasis. These data showed that elevations in H-ras and c-myc steady-state expression accompany the proliferative response of bile duct epithelial cells. Decreased levels of c-myc after initial elevation infer that ductal proliferation may continue independently of its steady-state expression, a response usually seen in vitro rather than in in vivo proliferation.

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)

Congenital diaphragmatic hernia beyond infancy.

Congenital diaphragmatic hernia (CDH) is a common cause of severe respiratory distress in the newborn. However, the presentation of CDH in older children and adults is rare, and, therefore, little is known concerning its symptoms, operative management, and postoperative complications. Thirteen patients (age range: 2 months to 26 years; 5 males, 8 females) presented with CDH. Four patients had right-sided hernias, eight left-sided hernias, and one bilateral hernias. Symptoms included chronic respiratory tract infections in 6 patients, vomiting in 5, weight loss in 1, severe failure to thrive in 2, and severe respiratory distress in 3; one patient was asymptomatic. Physical signs included the absence of breathing sounds or bowel sounds in the chest in eight patients, hyperresonance in one, and cachexia in two. The diagnosis was confirmed in each patient by chest roentgenogram or gastrointestinal contrast radiograph. All patients underwent immediate repair. After reduction of the viscera, 12 of 13 patients underwent primary diaphragm repair, whereas one patient required a prosthetic diaphragm patch. Twelve of 13 patients (92%) survived. Postoperatively, 7 of the 12 survivors (58%) developed severe gastric atony, and four required further operative therapy. In contrast to newborns, CDH in the older child and adult is frequently seen on the right side, rarely presents with severe respiratory distress, and is occasionally asymptomatic. Postoperative gastric atony is a major cause of morbidity, making transabdominal repair with simultaneous pyloroplasty and/or feeding jejunostomy the preferred operative approach.

Cholestasis without cirrhosis alters regulatory liver gene expression and inhibits hepatic regeneration.

Partial hepatectomy (PH) initiates cellular signals for regeneration. Sequential expression of nuclear and cytosolic protooncogenes accompanies the restoration of normal liver function and architecture. Although cirrhosis is known to inhibit liver regeneration, the effects of noncirrhotic cholestasis on hepatocellular proliferation, differentiation, and regulatory gene expression are unknown. To examine this, 25 male Fisher rats underwent common bile duct ligation and division. A 47% +/- 5% PH was performed 10 days after common bile duct ligation and division when histologic analysis revealed cholestasis without cirrhosis. Despite early elevations of total hepatic DNA and RNA values, cholestatic livers demonstrated a significant threefold suppression of expected hepatocyte mitotic indexes 48 and 72 hours after PH, compared with livers after PH alone. Weight restoration in cholestatic livers was 11% +/- 5.2% compared with 40% +/- 4.3% in control livers (+/- SEM; p less than 0.001) 5 days after PH. Analysis of regenerating liver messenger RNA with complementary DNA probes revealed an abnormal, sustained elevation of K-ras expression in cholestatic livers through all time points. Cholestasis blunted but did not obliterate normal sequential elevations in H-ras found in control livers. The expression of c-myc was inhibited threefold with cholestasis 72 hours after PH. These results are the first indication that cholestasis alone inhibits hepatocyte proliferation and the expression of c-myc that normally precedes the first wave of mitosis. This implies that cholestasis without cirrhosis may alter programmed liver gene expression, inhibiting normal hepatic regeneration.

Laparoscopic cholecystectomy in anticoagulated patients.

Although minimally surgically invasive, laparoscopic surgery has yet to be proven safe in patients receiving anticoagulants. Retrospectively, the laparoscopic management of four patients requiring anticoagulation for cardiac valvular prostheses or chronic atrial fibrillation was reviewed with regard to potential hemorrhagic complications. Warfarin was discontinued preoperatively in all cases. Heparin anticoagulation was individualized according to each patient's risk for thrombosis. Laparoscopic cholecystectomy and intraoperative cholangiography were completed in each patient without resulting hemorrhagic complications. The operative management of patients exhibiting cholecystitis may be complicated by anticoagulation therapy required for preexisting conditions/diseases such as cardiac valve prostheses, chronic atrial fibrillation, deep venous thrombosis, and pulmonary embolism. The minimally invasive nature of laparoscopic surgery lends itself well to cholecystectomy required in the face of anticoagulation treatment. This limited initial series of selected patients demonstrates the feasibility and efficacy of laparoscopic cholecystectomy in patients receiving anticoagulants.

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)

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.

Mucosal proliferation characteristics in ureterosigmoidostomy: effect of calcium supplement.

Ureterosigmoidostomy, frequently performed in children for urinary diversion, is a known premalignant condition. Dietary calcium supplements have been shown to normalize mucosal proliferative patterns in other human premalignant colonic conditions, which might decrease the risk of cancer. However, calcium supplementation has not been investigated in ureterosigmoidostomy. We used a rat model to study the effects of increased dietary calcium on the progression to carcinoma in ureterosigmoidostomy. Twenty-five Wister rats underwent ureterosigmoidostomy by anastomosis of bladder trigone and ureters to sigmoid, and were divided into two groups: group 1, regular diet (14); and group 2, calcium supplemented (2%) diet (11). All animals received the diet for 9 months. At death the anastomosis was weighed and examined histologically. In addition, ornithine decarboxylase (ODC), an enzyme involved in polyamine synthesis that becomes elevated in neoplastic and proliferative disorders, was determined at the anastomosis and 8 cm proximal (normal colon). All animals developed proliferative metaplastic polyposis at the anastomosis. Tumor weights were 2.6 +/- 1.1 g for group 1 and 4.8 +/- 1.2 g for group 2 (P less than .05). The ODC levels were: group 1, normal colon 78.2 +/- 15.6 and tumor 321.3 +/- 58.8 (P less than .002); and group 2, normal colon 425.8 +/- 65.4 and tumor 568.0 +/- 40.9 (P = NS). The difference between group 1 normal colon and group 2 normal colon was significant (P less than .001), as was group 1 tumor and group 2 tumor (P less than .02).(ABSTRACT TRUNCATED AT 250 WORDS)

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.

A bacteriologically occlusive clothing system for use in the operating room.

A comparison was made in a laminar-flow operating room between total-body exhaust gowns and a clothing system made from Fabric 450. This disposable clothing was found to be much more comfortable and convenient than the total-body exhaust gowns. The average airborne bacterial counts obtained during total hip replacement operations from each of the clothing systems were identical when the downflow method of ventilation was used (0.7 per cubic metre) and no significant difference could be demonstrated when the crossflow system was used (2.2 per cubic metre with the total-body exhaust gowns and 3.1 per cubic metre with the disposable clothing). Tests in a dispersal chamber were carried out to find the effectiveness of each item of the disposable clothing in reducing bacterial dispersion. These tests demonstrated the relative ineffectiveness of wearing a surgical gown as compared with wearing the complete system. It was confirmed bacteriologically that the downflow system of ventilation was more efficient than the crossflow type; the importance of this observation with respect to clothing and sepsis is discussed in this paper.