Congenital lobar emphysema resulting from bronchial sling around a normal right main pulmonary artery.

A 5-month-old female infant was transferred to our institution for evaluation of recurrent upper respiratory tract symptoms and chest x-ray films showing hyperaeration of the right upper lobe. Preoperative studies including chest fluoroscopy, lung scan, and bronchoscopy suggested congenital lobar emphysema. At thoracotomy, an anomalous right upper lobe bronchus which bifurcated around the main pulmonary artery in a sling fashion was found. This anatomic abnormality has not been described previously and is a unique cause of congenital lobar emphysema.

Extralobar pulmonary sequestration. Unusual presentation and systemic vascular communication in association with a right-sided diaphragmatic hernia.

A 17-day-old infant with a right-sided extralobar pulmonary sequestration associated with an intact diaphragmatic hernia sac presented as an acute respiratory emergency. The diagnosis was obscured by the initial resuscitative treatment of cardiorespiratory arrest necessitating positive-pressure ventilation complicated by bilateral tension pneumothoraces prior to transfer. At operation, unique systemic arterial and venous communications to the sequestration from the dome of the liver through the intact hernia sac were found. A bronchial communication to the right main bronchus was also present. Histologic examination of the sequestration specimen revealed other features common to both intralobar and extralobar sequestration. This case lends support to the hypothesis of a common embryologic basis for both types of sequestration, as well as other congenital pulmonary cystic abnormalities, and the association with diaphragmatic hernia.

Inhibition of interleukin-8 blocks myocardial ischemia-reperfusion injury.

INTRODUCTION: Interleukin-8 is thought to play a role in neutrophil activation and transcapillary migration into the interstitium. Because neutrophils are principal effector cells in acute myocardial ischemia-reperfusion injury, we postulated that the inhibition of interleukin-8 activity with a neutralizing monoclonal antibody directed against rabbit interleukin-8 (ARIL8.2) would attenuate the degree of myocardial injury encountered during reperfusion. METHODS: In New Zealand White rabbits, the large branch of the marginal coronary artery supplying most of the left ventricle was occluded for 45 minutes, followed by 2 hours of reperfusion. Fifteen minutes before reperfusion, animals were given an intravenous bolus of either 2 mg/kg of ARIL8.2 or 2 mg/kg anti-glycoprotein-120, an isotype control antibody that does not recognize interleukin-8. At the completion of the 120-minute reperfusion period, infarct size was determined. RESULTS: In the area at risk for infarction, 44.3% +/- 4% of the myocardium was infarcted in the anti-glycoprotein-120 group compared with 24.8% +/- 9% in the ARIL8.2 group (p < 0.005). In control animals, edema and diffuse infiltration of neutrophils were observed predominantly in the infarct zone and the surrounding area at risk. Tissue myeloperoxidase determinations did not differ significantly between groups, indicating that the cardioprotective effect of ARIL8.2 was independent of an effect on neutrophil infiltration. CONCLUSIONS: A specific monoclonal antibody that neutralizes interleukin-8 significantly reduces the degree of necrosis in a rabbit model of myocardial ischemia-reperfusion injury.

Inhibition of the transcriptional activator protein nuclear factor kappaB prevents hemodynamic instability associated with the whole-body inflammatory response syndrome.

BACKGROUND: The transcription factor nuclear factor kappaB mediates the expression of a number of inflammatory genes involved in the whole-body inflammatory response to injury. We and others have found that dithiocarbamates specifically inhibit nuclear factor kappaB-mediated transcriptional activation in vitro. OBJECTIVE: We hypothesized that inhibition of nuclear factor kappaB with dithiocarbamate treatment in vivo would attenuate interleukin 1 alpha-mediated hypotension in a rabbit model of systemic inflammation. METHODS: New Zealand White rabbits were anesthetized and cannulated for continuous hemodynamic monitoring during 240 minutes. Rabbits were treated intravenously with either phosphate-buffered saline solution or 15 mg/kg of a dithiocarbamate, either pyrrolidine dithiocarbamate or proline dithiocarbamate, 60 minutes before the intravenous infusion of 5 micrograms/kg interleukin 1 alpha. Nuclear factor kappaB activation was evaluated by electrophoretic gel mobility shift assay of whole-tissue homogenates. RESULTS: Infusion of interleukin 1 alpha resulted in significant decreases in mean arterial pressure and systemic vascular resistance, both of which were prevented by treatment with dithiocarbamate. Pyrrolidine dithiocarbamate induced a significant metabolic acidosis, whereas proline dithiocarbamate did not. Nuclear factor kappaB-binding activity was increased within heart, lung, and liver tissue 4 hours after interleukin 1 alpha infusion. Treatment with dithiocarbamate resulted in decreased nuclear factor kappaB activation in lung and liver tissue with respect to that in control animals. CONCLUSIONS: These results demonstrate that nuclear factor kappaB is systemically activated during whole-body inflammation and that inhibition of nuclear factor kappaB in vivo attenuates interleukin 1 alpha-induced hypotension. Nuclear factor kappaB thus represents a potential therapeutic target in the treatment of hemodynamic instability associated with the whole-body inflammatory response.

Hepatic vein and retrohepatic vena caval injuries in children. Sternotomy first?

Major hepatic vein and retrohepatic vena caval injuries are often fatal because of massive uncontrollable hemorrhage. Children with these injuries can be identified by their unique and dramatic clinical presentation and the selective use of computed tomographic imaging. Volume resuscitation promotes abdominal wall tamponade and hemodynamic stability until the abdomen is opened, at which point there may be sudden exsanguination before vascular control can be obtained. An alternative approach is to open the sternum before opening the abdomen. Management in this sequence provides rapid vascular control and improves the efficiency of hepatic exclusion. To date, five children with major hepatic vascular injuries have been treated with the sternotomy-first approach and four have survived; an atriocaval shunt was used on two occasions. Although sternotomy before laparotomy improves the efficiency of hepatic exclusion and may offer improved survival, accurate preoperative case selection limits its routine use.

An essential role for NF-kappaB in the cardioadaptive response to ischemia.

BACKGROUND: Ischemic preconditioning (IP) is the phenomenon whereby brief episodes of ischemia protect the heart against a subsequent ischemic stress. We hypothesize that activation of the transcription factor NF-kappaB mediates IP. METHODS: Rabbits were randomly allocated to one of three groups: (1) 45 minutes of myocardial ischemia followed by 2 hours of reperfusion (I/R); (2) three cycles of 5-minute ischemia and 5 minutes of reperfusion followed by I/R (IP + I/R); or (3) IP in the presence of ProDTC, a specific NF-kappaB inhibitor, followed by I/R (IPProDTC + I/R). Infarct size, indices of regional contractility, and NF-kappaB activation were determined. RESULTS: In preconditioned rabbits (IP + I/R), infarct size was reduced 83% compared with both I/R alone and IPProDTC + I/R groups (p < 0.05). Throughout reperfusion, preconditioned myocardium showed enhanced regional contractile function compared with I/R and IPProDTC + I/R groups (p < 0.05). Gel shift analysis showed NF-kappaB activation with IP that was blocked by ProDTC. I/R and IPProDTC + I/R groups showed NF-kappaB activation with I/R that was absent in preconditioned animals. CONCLUSIONS: The cytoprotective effects induced by IP require activation of NF-kappaB.

Treating myocardial ischemia-reperfusion injury by targeting endothelial cell transcription.

Exacerbation of, rather than improvement in, a hypoxic injury after reperfusion of ischemic tissues is recognized as the specific clinicopathologic entity referred to as ischemia/reperfusion (I/R) injury. Arguably, one of the most common forms of I/R injury occurs during cardiac surgery, which has a mandatory period of myocardial ischemia required to allow surgery in a bloodless, motionless field, followed by coronary artery reperfusion after removal of the aortic cross-clamp. In this review, we examine the endothelial cell activation phenotype that initiates and propagates myocardial I/R injury. Emphasis is given to the biology of one transcription factor, NF-kappaB, that has the principal role in the regulation of many endothelial cell genes expressed in activated endothelium. NF-kappaB-dependent transcription of endothelial cell genes that are transcribed in response to I/R injury may be a favorable approach to preventing tissue injury in the setting of I/R. Elucidating safe and effective therapy to inhibit transcription of endothelial cell genes involved in promoting injury after I/R injury may have wide applicability to the patients with heart disease and other forms of I/R injury.

Encouraging results with a modified Sawaguchi hepatoportoenterostomy for biliary atresia.

Since 1974, 25 patients with biliary atresia underwent a modified Sawaguchi hepatoportoenterostomy. Seventeen of 18 patients operated on before the age of 10 weeks became free of jaundice within 2 to 4 months and had biliary intestinal continuity established by 10 months of age. Two of these patients experienced sudden diminution of bile flow with return of jaundice. Both responded to transconduit repeat resection of the portal fibrous tissue with resolution of jaundice, which allowed take-down of the biliostomy. At last follow-up 1 to 13 years postoperatively, all 17 patients were free of jaundice (94 percent of total). One patient in this group never had drainage of bile and eventually died from sepsis. In contrast, five of seven patients operated on after 10 weeks never had drainage of bile. Four of these patients died from hepatic failure and one from bleeding varices. Two patients initially had bile drainage and became anicteric, but thereafter, acute cessation of bile flow and reccurrence of jaundice occurred. Reoperation resulted in a definitive increase in bile flow in both of these patients. The modified Sawaguchi procedure is definitely preferable to liver transplantation as primary treatment in infants with biliary atresia, especially if operation is performed before the age of 10 weeks.

Retrograde esophageal dilatation in children: an alternative to the indwelling string.

Repeated esophageal dilatation for chronic strictures secondary to corrosive burns or anastomotic scarring is commonly performed by the retrograde technic in children. This requires the presence of an indwelling string, tied in a circle, entering the nose, and exiting the gastrostomy site. Long-term presence of this string frequently leads to nasal and gastrostomy excoriations and resultant patient discomfort. We advocate and describe a technic using a piece of Silastic tubing as an alternative to the indwelling string. This has met with increased patient acceptance and comfort as compared with the previous "heavysilk" or other "string" materials.

Biliary obstruction from hepatic regeneration following extended right hepatectomy for tumor.

Two patients, aged 2 and 6 months, underwent extended right hepatectomy for hepatoblastoma. Tumor resection was complete in both and postoperative chemotherapy was begun. One patient became markedly jaundiced 4 months postoperatively. Imaging evaluation showed marked nodular liver enlargement and intrahepatic ductal dilatation. At laparotomy an obstructed distal common duct was draped over a huge regenerating liver nodule. A high Roux-En-Y choledochojejunostomy was followed by a decrease in bilirubin to normal within 4 days. Chemotherapy was restarted and the patient remains anicteric and healthy. No evidence of tumor recurrence was found. A second patient became jaundiced 2 weeks after resection. Imaging evaluation suggested tumor recurrence. At laparotomy biopsy failed to confirm recurrent tumor but the area of the porta was not explored. Jaundice persisted and the patient died of liver failure thought secondary to tumor recurrence 4 months postoperation. Postmortem examination showed a completely obstructed common duct draped over a liver nodule and no evidence of tumor. These two patients illustrate the heretofore unrecorded entity of biliary obstruction from regenerating liver following resection for tumor. The sudden appearance of jaundice following a successful liver resection for tumor should raise the suspicion of benign repairable biliary obstruction rather than unresectable tumor recurrence. The definitive evaluation is reexploration rather than various imaging modalities, which may not only be misleading, but may delay life-saving intervention.

Modified Duhamel procedure for treatment of Hirschsprung's disease in infancy and childhood: review of 41 consecutive cases.

The original Duhamel procedure has undergone several important modifications over the years by Martin, Ravitch, Soper, Talbert, and others. Combining the best features of these various modifications, a modified Duhamel procedure has been developed and utilized in 41 consecutive infants and children with all types of Hirschsprung's disease, including three with long segment involvement. The procedure includes the use of a formal upper and lower anastomosis and division of the spur by the GIA stapling device. There were no deaths and no early complications. At 2-7 yr all children are toilet-trained, continent, and growing well. With appropriate modifications, the Duhamel operation is a very satisfactory definitive procedure for Hirschsprung's disease in infancy and childhood.

Extensive multi-organ necrosis secondary to sulfuric acid ingestion.

This is a report of a case in which a significant quantity of sulfuric acid was ingested, causing life threatening respiratory distress, secondary to glottic injury and edema, and cardiovascular collapse. Vigorous resuscitive efforts allowed survival through the first 48 hours and two major operations. Persistent and complex surgical management eventually restored oropharyngeal competence and gastrointestinal integrity. Survival of a child from such an overwhelming injury has not been previously reported.

Treatment of pancreatic duct disruption in children by an endoscopically placed stent.

BACKGROUND: Injuries to the pancreas from blunt abdominal trauma in children are rare. Most are minor and are best treated conservatively. The mainstay for treatment of major ductal injuries has been prompt surgical resection. Diagnostic imaging modalities are the key to the accurate classification of these injuries and planning appropriate treatment. Computed tomography (CT) scan has been the major imaging modality in blunt abdominal trauma for children, but has shortcomings in the diagnosis of pancreatic ductal injury. Endoscopic retrograde cholangiopancreatography (ERCP) has been shown recently to be superior in diagnostic accuracy. The therapeutic placement of stents in the trauma setting has not been described in children. METHODS: Two children sustained major ductal injuries from blunt abdominal trauma that were suspected, but not conclusively noted, on initial CT scan. Both underwent ERCP within hours of injury. In case 1, a stent was threaded through the disruption into the distal duct. In case 2, a similar injury, the stent could only be placed through the ampulla, thereby reducing ductal pressure. In both cases, clinical improvement was rapid with complete resolution of clinical and chemical pancreatitis, resumption of a normal diet, and discharge from the hospital. The stents were removed at 10 and 12 days postinjury, and both children have remained well. Follow-up ERCP and CT scans show complete healing of the ducts and no evidence of pseudocyst formation 1 year post injury. CONCLUSIONS: Acute ERCP should be the imaging modality of choice in suspected major pancreatic ductal injury. Successful treatment by placement of an intrapancreatic ductal stent may be possible at the same time. Surgical resection or reconstruction can then be reserved for cases in which stenting is impossible or fails.

One-stage esophagectomy and in situ colon interposition for esophageal replacement in children.

PURPOSE: Esophageal replacement has been achieved using stomach, gastric tube, small intestine, and colon in various positions and in single or multiple stages. Long-term functional results are of prime importance in children with benign disease. The aim of this study is to present the immediate and long-term results of one-stage esophagectomy and in situ colon interposition esophageal replacement in children. METHODS: Seven children have undergone one-stage esophagectomy and in situ colon interposition esophageal replacement for stricture secondary to caustic ingestion (n = 4), battery ingestion (n = 2), and epidermolysis bullosa (n = 1). TECHNIQUE: Via thoracoabdominal and cervical incisions, the transverse colon, isolated on the left colic artery, is pulled through the esophageal hiatus and normal esophageal bed into the neck at the time of simultaneous esophagectomy. The stomach is partially wrapped around the colon as an antireflux procedure and a pyloroplasty are performed. RESULTS: There was no immediate postoperative morbidity or mortality. All patients were discharged taking a soft diet by mouth. There were no anastomotic leaks or strictures, and functional swallowing is excellent. Contrast studies show no dilation of the colon in the chest. Growth and development have been normal up to 7 years postoperatively. CONCLUSION: The one-stage esophagectomy and in situ colon interposition is an excellent technique for esophageal replacement in children. Continued evaluation of this technique will be necessary to confirm these preliminary results.

Hepatic artery ligation for exsanguinating liver injuries in children.

Selective hepatic dearterialization for exsanguinating liver injury has been performed in two young children without untoward sequelae. It appears to be a safe, simple, life-saving maneuver, as has been shown in adults. Simultaneous cholecystectomy and drainage of the extrahepatic biliary tree does not seem necessary. Postoperative studies in these two patients showed normal liver functions, normal liver scintiscans, and in one case, early rearterialization of the devascularized lobe by translobar collaterals. We would urge the use of this relatively simple procedure in place of major hepatic resection for selected severe hepatic injuries.

Primary fascial closure in infants with gastroschisis and omphalocele: a superior approach.

The optimal surgical management of neonates with gastroschisis and omphalocele remains controversial. Early attempts at primary closure were limited by respiratory and vena cava compromise and were gradually replaced by skin closure only, staged reduction of implanted prostheses, and escharification of the intact omphalocele. Advances in surgical technique, neonatal intensive care, and mechanical ventilation have made primary closure a viable alternative once again. We wish to report a consecutive series of 73 infants, including 54 with gastroschisis and 19 with omphalocele, over a 16-year period. Fifty gastroschisis patients were closed primarily, with four deaths (8%), and four with staged prostheses, with one death (25%). Sixteen omphaloceles were closed primarily, with three deaths (19%); two closed with a staged prosthesis, and one patient, closed with skin flaps only, died (100%). Overall survival for primary closure was 59/66 (89%) and for staged reduction, 5/7 (43%). In the past 5 years all patients have been closed primarily with no deaths (100% survival). All survivors were alive and well 1 to 15 years postoperatively. Our current operative technique involves vigorous manual stretching of the abdominal cavity, manual emptying of the entire Gl tract, and primary fascial closure, with no gastrostomy. Key points in postoperative management include total paralysis and mechanical ventilation, as the initially tense abdominal wall softens over 1 to 2 days, followed by weaning from the ventilator, and vigorous fluid support. Gl function returns more rapidly after primary closure (average of 3 days) and hospital stay is not prolonged. Other complications (intestinal obstruction, two patients; fistulae, one patient, etc) are much less frequent.(ABSTRACT TRUNCATED AT 250 WORDS)

The anterior cricoid split procedure for the management of subglottic stenosis in infants and children.

Severe acquired subglottic stenosis occurs most commonly in infants and children who require long-term ventilatory support with indwelling endotracheal tubes for underlying respiratory disease. A variety of operative and endoscopic procedures have been advocated for this problem including endoscopic fulguration, cryotherapy, direct incision or excision, and several types of extensive direct laryngotracheoplasties. The failure rate with these procedures is high. We have treated 22 patients including 12 neonates and 10 older children with acquired airway injury and obstruction with a simple cricoid split. In 15 patients the airway obstruction was completely relieved and these youngsters were extubated without difficulty. An additional 3 patients failed initial extubation which was subsequently successful, however, after repeat intubation for a short period of time. Endoscopic follow-up shows complete healing of the incised area. In 2 neonates the procedure was unsuccessful, and tracheotomy was needed. One of these had additional severe airway injury in the distal tracheobronchial tree secondary to selective bronchial intubation in the newborn period. Two older children also required tracheotomy because of severe airway scarring unrelieved by this procedure. The cricoid split is a simple and successful way of dealing with subglottic stenosis, especially in the newborn premature infant. A significant advantage is that it disturbs the anatomy very little, allowing for more extensive laryngotracheoplasty in the future should it fail.

Experimental portal hypertension in the rat.

Previous attempts to produce sustained portal hypertension in experimental animals by portal venous obstruction have not been successful. Experiments were designed to study the effects of gradual portal venous occlusion plus hepatic lymphatic ligation in the rat. Adult male Lewis rats were divided into three groups: (A) nonoperated or sham-operated; (B) operated, with hepatic lymphatic ligation only; and (C) operated, with placement of a portal vein ameroid constrictor and hepatic lymphatic ligation. There were no differences noted in portal venous pressures (control 9.6 +/- 0.4 cm H2O) and portovenograms between groups A and B throughout the study. In group C there was greater than doubling of portal venous pressure by 1 wk which remained for 2 mo. Portal pressure then fell but remained elevated at greater than 30% above control values for 1 yr. Three systems of collateral circulation developed rapidly in response to this extrahepatic portal venous occlusion: (A) veins directly bridging across the occluding ameroid reestablishing hepatopetal flow which steadily increased; (B) spontaneous portosystemic shunts at the splenorenal area that occur early and then regress; and (C) retroperitoneal, paraesophageal and submucosal esophageal veins that develop late and remain stable. Quantitative assessment of the lumen size of the submucosal esophageal veins revealed a twofold increase in these veins in group B when compared to controls by 4 wk and a fivefold increase in these veins in group C by 8 wk that persisted for 1 yr. This model appears useful for studying the course and effects of extrahepatic portal hypertension and its gradual alteration by the spontaneous development of portosystemic collaterals in the rat. This model may have enough similarities to the extrahepatic portal hypertension seen in children that its use for future studies may be fruitful.

The lateral reservoir technique of ileal endorectal pull-through for ulcerative colitis and familial polyposis in children.

Total abdominal colectomy and endorectal ileal pull-through have been widely used in children as a definitive continence-preserving procedure for ulcerative colitis (UC) and familial polyposis (FP). Controversy exists in regards to the results achieved with the various techniques utilized to accomplish this procedure, including straight ileal pull-through, pull-through with balloon dilatation of the lower ileal segment, and the construction of a variety of ileal reservoirs: S-shaped, J-shaped, or lateral. We have utilized the two-stage lateral reservoir approach advocated by Fonkalsrud et al, on a total of 12 patients, aged 1 to 17 years, including ten patients with UC and two with FP. Twelve patients have undergone stage 1 and seven of these stage 2. Numerous complications of stage 1 occurred early in the series, including breakdown of the ileoanal anastomosis, 3 patients; cuff abscess, 2 patients; postoperative intestinal obstruction, 2 patients; and intraabdominal abscess, 1 patient. All have fully recovered. The last six stage 1 procedures have been without complications. Five of the seven stage 2 patients are now well, continent, and having four to six controlled bowel movements a day 1 to 2 years postoperatively. Two patients are using small amounts of Immodium and Metamucil. One patient has developed marked dilatation of the pouch at 8 months postoperatively with episodic "pouchitis" requiring operative revision and diversion. One stage 2 patient suffered numerous complications including spontaneous perforation of the reservoir 2 months postoperatively, eventually leading to re-creation of a permanent ileostomy and removal of the pouch. Five patients await further treatment. The lateral reservoir technique is capable of producing a satisfied and continent patient; however, the potential technical problems and early complications are significant. The occurrence of delayed "pouchitis" is worrisome. Although the initial results with the lateral reservoir technique appear encouraging, further longterm evaluation is necessary.

Nonvisualization of the intravenous pyelogram--a poor prognostic sign in Wilms' tumor?

In a series of 49 consecutive cases of Wilms' tumor from a single institution, there was a 20% incidence of nonvisualization on the initial intravenous pyelogram. Even though nonvisualization was secondary to either gross or microscopic invasion of the renal vein or renal pelvis, this did not forecast a grim progosis. Seven of the nine patients with nonvisualization are presently alive and tumor-free at least 5 yr after resection. The only two deaths in this particular group were unrelated to the Wilms' tumor.