Periportal tracking on computed tomography scan diagnostic of extensive liver injury in a neonate.
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Biomedical subjects
Publications and source records attributed to R G Azizkhan.
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A simple and effective method of aspirating residual intrathoracic air is described. This method can be used when air leaks or persistent fluid accumulations are not expected. This technique may be easily adapted for use in older children or adults when similar circumstances are present. Standard postoperative monitoring, including serial roentgenogram of the chest, should be used to verify appropriate pulmonary re-expansion.
A murine endothelial cell line was isolated from hemangiomas induced by expression of the polyoma early region gene in transgenic mice. After two cell sortings using acetylated low-density lipoprotein with a fluorescent label (Dil-Ac-LDL), a pure population of endothelial cells has been carried for more than 60 passages from the animal. The cells retain endothelial cell properties such as a characteristic cobblestone appearance at confluency, contact-inhibited growth, and active uptake of Ac-LDL. Expression analysis shows that the cells express both the polyoma transgene and the von Willebrand factor, an endothelial cell marker. Subcutaneous injection of the cultured endothelial cells into nontransgenic histocompatible mice or nude mice led to hemangioma formation, and endothelial cells were re-isolated by cell sorting from these secondary hemangiomas. This cell line represents a renewable source of murine endothelial cells derived from transgenic mice that can be studied both in vitro and by reintroduction into a host.
Microvillus inclusion disease is an inherited intestinal brush border membrane defect that causes severe fluid and electrolyte malabsorption. In an infant with microvillus inclusion disease (confirmed by electron microscopic evaluation of rectal, jejunal, and gallbladder mucosae), basal stool output was massive (greater than 125 mL . kg-1 . day-1) and was not altered by treatment with clonidine or octreotide. A proximal jejunostomy with mucous fistula was placed, allowing separation of proximal from distal tract outputs (60 mL . kg-1 . day-1 and 100 mL . kg-1 . day-1, respectively). A 10-cm jejunal segment was excised during surgery and mounted in Ussing chambers for determination of transepithelial Na+ and Cl fluxes. Compared with intestine of normal infants, this infant's epithelium showed transmural conductance and unidirectional ion fluxes that were only 30% of normal. With respect to both Na+ and Cl, the excised jejunum was in a net secretory state. Theophylline (5 mmol/L) increased net Cl secretion slightly. In response to mucosal D-glucose (30 mmol/L), jejunal mucosal-to-serosal Na+ flux doubled. In the infant, glucose-electrolyte solution administered intrajejunally did not significantly change stool output, suggesting that all of the solution (40 mL/kg) was absorbed. Subtotal enterocolectomy, in theory, could have decreased purging by 66% in this infant with microvillus inclusion disease, but diarrhea would still have been significant.
The diagnosis and management of a persistent intrapleural-dural cerebrospinal fluid fistula following excision of a large mediastinal ganglioneuroma with intraspinal extension is reported. Use of a vascularized intercostal muscle flap to close the dural fistula was curative in this 4-year-old patient.
Recurrent severe hemorrhage from intestinal vascular malformations, although extremely rare, may be associated with significant morbidity and mortality. The treatment may require extensive surgical resection, vascular embolization, and repeated blood transfusions. An adolescent boy with the Klippel-Trenaunay syndrome involving the pelvis and left leg presented with recurrent life-threatening hematochezia associated with defecation. Endoscopy documented that the bleeding originated from the submucosal venous angiomata in the region of the hemorrhoidal plexus. An argon laser was used to systematically coagulate the venous angiomata involving the distal 7 cm of the anorectal canal. Postoperative minor rectal bleeding and rectal tenesmus resolved in a few days. The patient has had only one brief episode of hematochezia in more than 4 years of follow-up. The use of the argon laser has provided effective palliation of colorectal vascular malformations with minimal morbidity but more importantly has allowed the preservation of normal anorectal function.
Twenty-one consecutive cases of fetal and neonatal gastroschisis were retrospectively reviewed. There was 100% survival if major nonintestinal malformations did not coexist; however, 28.6% of these patients had other major malformations and 66% of them died. There were significantly fewer small for gestational age infants if the defect was diagnosed prenatally (20% versus 75%, p less than 0.003). There was a 60% cesarean delivery rate in prenatally diagnosed infants and 0% if diagnosis occurred at delivery (p less than 0.01).
The Pediatric Trauma Score (PTS) has been identified as the only accurate and adequate means of predicting outcome in pediatric trauma. In answer to the increasing number of trauma patients arriving at local hospitals, the ability of the adult Trauma Score (TS) to predict pediatric trauma outcome was tested. Of the total 2,604 pediatric trauma cases in the North Carolina State Trauma Registry, 441 had both a PTS and TS available for analysis. The primary measures of outcome were emergency department and hospital dispositions. Logistic regression demonstrated that TS (R2 = 0.50) was a stronger predictor of pediatric outcome and PTS (R2 = 0.35) for emergency department disposition and TS (R2 = 0.63) with PTS (R2 = 0.51) for hospital disposition. The correlation between TS and PTS was high (R = 0.8). Stepwise discriminant analysis demonstrated that TS was the stronger predictor of outcome and the PTS added only 9% (partial R2 = 0.09) more accuracy to TS for emergency department disposition and only 6% (partial R2 = 0.06) for hospital disposition. The results of this research demonstrate that TS is a useful method of predicting outcome in pediatric trauma. The use of both scores for each patient does not increase the predictive value of the scores.
Ultrathin flexible bronchoscopes with controlled distal angulation allow the conventional diagnostic examination of the lower airways of even the smallest infants. These instruments may be passed through small endotracheal or tracheostomy tubes while ventilation is maintained. It is thus possible, under direct visualization, to control the manipulation of surgical instruments where they could not otherwise be seen, or to study airway dynamics and anatomy intraoperatively without extubating the patient.
Congenital hernias in the lumbar region are very uncommon. Approximately 10 per cent of all lumbar hernias are congenital and the vast majority are unilateral. We report our experience with seven congenital lumbar hernias in six infants treated during a five year period. All patients presented in the first year of life with abnormal protrusions in the lumbar region. In addition to the lumbar hernia, major associated malformations, including caudal regression anomalies, diaphragmatic hernia, ureteropelvic junction obstruction, cloacal exstrophy and lipomeningocele, were observed in 66 per cent of the infants and were consistent with lumbocostovertebral syndrome. In five patients, unilateral defects were repaired primarily. One patient with bilateral lumbar hernia underwent staged repair. The larger right-sided defect was repaired using a polytetrafluoroethylene prosthesis. At a second operation two months later, the small left-sided hernia was closed primarily. All patients have done well without recurrence with a follow-up period ranging from four to 48 months. Early repair of congenital lumbar hernias in infants after correction of other life-threatening conditions is advocated. Unlike the acquired variety, congenital lumbar hernia may include a more extensive deficiency of the entire lateral abdominal wall extending to the rectus sheath and inguinal ligament and satisfactory closure of the defect without prosthetic material may be difficult or impossible.
In infants and children requiring prolonged and multiple central venous catheterizations, conventional cannulation sites may become thrombosed or stenotic, making inability to gain vascular access a life-threatening problem. The technique we use for the percutaneous placement of inferior vena caval tunneled silastic catheters via the translumbar and transhepatic approaches is described. Three translumbar placements and one transhepatic placement in three children without immediate complications have been performed. We conclude that percutaneous inferior vena caval cannulation via the translumbar or transhepatic routes offers a viable alternative in these patients with difficult vascular access.
Acquired bronchial stenosis following prolonged endotracheal intubation is uncommon, but in infants it is associated with significant morbidity. A variety of endobronchial techniques including forceps or cautery resection and balloon dilatation have been used with inconsistent results. Laser therapy seems attractive, but pediatric applications have been very limited. We report the first series of infants with life-threatening acquired bronchial stenosis treated with an argon laser. Eight infants, age 3 weeks to 2 years, presented with symptomatic bronchial obstruction following prolonged intubation. Seven of these patients had at least 90% obstruction of a lobar or mainstem bronchus. Under general anesthesia a 300 or 600 micron quartz laser fiber was passed through the suction channel of a 3.5-mm flexible or 3-mm rigid bronchoscope. The laser was operated at 2.5 to 3.5 W in 0.5-second pulses, to ablate the obstructing tissue. Multiple procedures, spaced no closer than 10 days, were required in three of eight infants. Follow-up bronchoscopy after 2 to 30 months revealed normal findings in five of eight infants. All but one child, who has persistent collapse of the bronchus intermedius due to bronchomalacia at the site of the obstruction, had satisfactory results. The only complication was a pneumothorax in a 1,300 g infant, which developed eight hours after treatment. Our experience suggests that the argon laser is effective in the management of endobronchial lesions in infants and is superior to the CO2 and Neo-dymium-yttrium aluminum garnet (Nd-YAG) lasers for this purpose.(ABSTRACT TRUNCATED AT 250 WORDS)
Xanthogranulomas larger than 2 cm are rarely reported variants of this otherwise common, benign lesion usually measuring 1 mm to 1 cm in diameter. The natural history of such macronodular xanthogranulomas is uncertain, but appears to parallel the generally predictable course toward involution seen in micronodular lesions. Two patients with macronodular xanthogranuloma illustrate exophytic and endophytic variants, as well as the problems of managing this unusual tumor. Nonoperative observation is advisable if possible, but complicating factors may necessitate surgical excision in some cases.
Bleomycin (BLEO), an antitumor antibiotic effective against a variety of malignancies, has been associated classically with a pulmonary toxic reaction producing diffuse interstitial fibrosis. However, BLEO-related pulmonary nodules have been reported recently, mostly in children and young adults treated for germ cell tumors. A different, apparent hypersensitivity reaction with prominent eosinophilic infiltrates has been seen in other patients. This report details the clinical history, radiographic features, and histopathologic condition of three patients with osteogenic sarcoma in whom pulmonary nodules developed during the course of their multiagent, BLEO-containing chemotherapy. The predominant histopathologic lesion was bronchiolitis obliterans-organizing pneumonia (BOOP); one patient had a significant eosinophilic infiltrate also. Pulmonary lesions developed in all of these patients after relatively low doses of BLEO (less than 200 mg). All of these patients underwent open lung biopsy to establish the diagnosis. Reported cases of BLEO-induced pulmonary injury other than diffuse fibrosis are reviewed and comparisons are made with those in the current report. Also, suggestions are made for the management of these patients.
In patients requiring long term central venous access, common venous access routes may become occluded. Surgical placement of central venous catheters may not always be possible or optimal. Percutaneous translumbar inferior vena cava central line placement offers an additional access route. The technique of translumbar inferior vena cava central venous access is described in a critically ill child.
We report a case of renal salvage after total right ureteral loss using the isolated vermiform appendix with preservation of the blood supply. The appendix was used to bridge the gap between the right renal pelvis and left ureter. Followup revealed satisfactory function of the appendiceal conduit with preservation of renal function. The appendix, used in this fashion, is an attractive alternative to other surgical modalities after total or near total ureteral loss. The excellent contractility combined with the relatively small size eliminates the problems associated with urinary stasis and electrolyte reabsorption. However, its use is limited by its availability and length.
The placement of multipurpose silicone right atrial catheters for continuous venous access in children with complex urological diseases has become increasingly necessary. Between September 1985 and September 1987, 26 children with a variety of urological diseases required long-term central venous access, primarily for delivery of chemotherapy and blood products in 13 patients with malignancies, hemodialysis access in 7 undergoing renal transplantation, total parenteral nutrition in 5 (2 of whom were born with cloacal exstrophy), and fluid and antibiotic administration in 1 with dermatomyositis and acute pyelonephritis. Patient age at catheter placement ranged from 1 day to 15 years. A total of 11 complications was encountered during 4,500 catheter days: 10 were mechanical and 1 was infectious in nature. These complications led to replacement of the catheter in 3 patients and the remainder were managed successfully nonoperatively. The surgical techniques involved in placement of these catheters are discussed. The catheters not only allowed many of these patients to receive treatment on an outpatient basis but also ensured their survival. Our favorable experience with prolonged venous access in children should encourage urologists to include these techniques in their armamentarium.
An extremely rare case of a basioccipital meningocele causing obstruction of the upper airway in a neonate is described. A transoral approach was used to remove the meningocele and repair the dural fistula. Bilateral posterior lateral pharyngeal releasing incisions were made to allow mucosal and muscle coverage over the dural repair. This report demonstrates and emphasizes the use of lateral pharyngeal releasing incisions to cover midline full thickness defects in the posterior oropharyngeal wall that might be encountered during transoral transclival operations.