Potentially lethal complications of central venous catheter placement.

BACKGROUND: Placement of central venous catheters, although often considered to be a relatively safe and "junior"-level procedure, may be associated with life-threatening complications. METHODS: A recent surgical death associated with placement of a central venous catheter at this Institution led to submission of a questionnaire to pediatric surgeons referenced through the American Pediatric Surgical Association directory regarding knowledge of similar incidents and information regarding catheter placement-related complications. RESULTS: Results to this response, although anecdotal, provided data regarding complications of an acute nature, which fell into the categories of pneumothorax, hydrothorax, cardiac tamponade, and hemothorax. Of 10 children with cardiac tamponade, 7 were infants, and most complications were associated with needle stick for access, with symptoms developing within minutes up to 12 hours after the procedure. Drainage of the tamponade was performed by aspiration alone in 3 cases; surgical drainage in 6 children resulted in survival in 9 of the 10 patients. Hemothorax was described in 19 patients and appeared to be more common in children in the 1- to 6-year age group, usually associated with percutaneous access techniques. Thoracotomy for hemothorax was performed in 16 children with 11 survivors. Vascular injury to subclavian artery, vein, or superior vena caval were noted in most at operation. CONCLUSIONS: Although data included in this review are entirely anecdotal and not subject to scientific scrutiny or analysis, certain conclusions appear evident. Inherent risks of central venous catheters are intrinsic and should be discussed with the family in obtaining preoperative consent, including life-threatening risks that may necessitate urgent surgical intervention (by thoracotomy or other means). Certain technical aspects of the procedure should be rigidly followed with an experienced surgeon in attendance throughout the procedure. Rapid evaluation should be performed for any unexplained problems that occur in the operating theatre or during the early postoperative period.

Management of persistent pediatric tracheostomal fistulas.

Decannulation of a tracheostomy generally results in spontaneous closure. Occasionally, epithelialization results in persistence of the fistula, which may be initially treated by local curettage or cautery. Failure of these methods constitutes an indication for surgical closure. Dissection of the entire tracheocutaneous tract permits fistula closure in juxtaposition to but outside the trachea and prevents any iatrogenic airway narrowing. Twelve patients have been so managed over the last 10 years, and there have been no immediate or long-term complications.

Parathyroid cysts: report of the sixth and youngest pediatric case.

A nonfunctioning parathyroid cyst occurred in an 8-year-old girl, the youngest patient reported to have this diagnosis. Most cysts are asymptomatic; a few are associated with signs and symptoms of hyperparathyroidism. In asymptomatic patients with a lateral neck cyst, aspiration of clear fluid with an elevated parathyroid hormone level is diagnostic of a parathyroid cyst. If this results in disappearance of the cyst, without recurrence, no further treatment is necessary. If the cyst recurs, aspiration may be repeated. However, persistence of the cyst despite aspiration or recurrence after the second aspiration should prompt surgical removal, with intraoperative identification of all parathyroid glands, because functioning parathyroid cysts are associated with a high risk of hyperplasia or adenoma.

Use of polymorphic DNA probes to test the lymph nodes of infants and fetuses for spontaneous chimerism.

Spontaneous chimerism is rare, but it has been observed in infants who have received intrauterine transfusions, or in those infants who have severe combined immunodeficiency disease. The site of maternal cell engraftment has not been identified in these cases, but it is apparently not the bone marrow. Lymph nodes have not been investigated. Based on recent experimental data that showed that foreign cells grow in the lymph nodes, but not in the marrows of perinatally inoculated mice, the decision was made to use highly specific polymorphic DNA probes to assay for the presence of maternal DNA in the nodes that were removed from three infants and two fetuses. No evidence for the engraftment of maternal cells was detected at a 5% level of sensitivity. These results were in accord with findings from previous work that has shown that the placenta and host-resistance mechanisms combine to make spontaneous chimerism a rare event. The results also showed that similar tests for maternal cell engraftment in lymph nodes and other sites, not previously evaluated, are feasible and perhaps indicated in infants with congenital and acquired immunodeficiencies, such as severe combined immunodeficiency disease and acquired immunodeficiency syndrome.

Use of intraoperative ultrasound during hepatic resection in pediatric patients.

Five pediatric patients with primary liver tumors were evaluated preoperatively with ultrasound (US), computerized tomography (CT), and angiography, and tentative operative plans were formulated. Intraoperative US was subsequently used to examine these children, resulting in changes in operative strategy of all five patients despite their extensive preoperative evaluations. Intraoperative ultrasound appears to provide the most accurate assessment of both the extent of tumor and its vascular relationships. Thus, operative strategies may be precisely tailored on the basis of such information, allowing rational resection where appropriate, while futile attempts at removal of inoperable lesions may be averted.

Double aortic arch associated with esophageal atresia and tracheoesophageal fistula.

Esophageal atresia (EA) with tracheoesophageal fistula (TEF) is commonly associated with significant cardiovascular anomalies. Mortality is higher with cardiovascular abnormalities, especially those undiagnosed prior to surgical repair of the EA/TEF. Advances in cardiac imaging techniques, most notably echocardiography, have made noninvasive evaluation of the heart and great vessels easy to perform and reliable. Thus, the preoperative cardiovascular anatomy of these infants should be routinely assessed prior to surgical correction. A case of EA/TEF associated with an unsuspected double aortic arch is described. Although a wide variety of cardiovascular lesions have been associated with EA/TEF, coexistence of a double aortic arch has not been previously reported. Preoperative recognition of this anomaly would have altered the surgical approach, and perhaps reduced the postoperative morbidity and mortality.

Transthoracic Nissen fundoplication for gastroesophageal reflux in patients with severe kypho-roto-scoliosis.

Transthoracic Nissen fundoplication was used for the correction of gastroesophageal reflux in five mentally retarded patients with severe kypho-roto-scoliosis. This deformity may result in a gastroesophageal junction so high above the left costal margin that transabdominal fundoplication is extremely difficult. Operative morbidity was minimal. One wrap disruption occurred that required reoperation. Results have been satisfactory 6 to 24 months postoperatively.

Principles in the management of pediatric intestinal leiomyosarcomas.

Primary intestinal leiomyosarcomas in children are unusual tumors with little known about their natural history. Two patients (aged 9 and 10 years) with leiomyosarcoma of the jejunum treated at our institution are reported and added to the 20 other patients reported in the literature. The first patient had a grade 1 leiomyosarcoma completely resected, received no additional therapy, and remains alive with no evidence of disease 14 years later. The second patient presented with a perforated grade 2 leiomyosarcoma which was incompletely resected; he received combination chemotherapy with an initial good response, but eventually died from sarcomatosis 7 years after initial diagnosis. The 22 cases of pediatric intestinal leiomyosarcoma presented more commonly with obstruction or perforation, were able to be completely resected more often, and appear to have a better prognosis than in adults. Thus, intestinal leiomyosarcomas in children appear to have a natural history different from that of the same tumor arising in adults.

Transforming growth factor beta (TGF-beta) induces fibrosis in a fetal wound model.

The adult cellular response to tissue injury is characterized by acute inflammation followed eventually by fibroblast proliferation and collagen synthesis. Fetal tissue responses to injury differ markedly from those of the adult; an early acute inflammatory response is absent, few fibroblasts participate, and no collagen is deposited. The object of the present study was to analyze the effects of transforming growth factor beta (TGF-beta), an important regulatory molecule in adult healing events, on the fetal tissue response following wounding. Fetal cellular and extracellular matrix responses to injury were evaluated by placing subcutaneous wound implants containing TGF-beta (0.01 to 10 ng) in fetal rabbits at 24 days gestation (term = 31 days). Histologic responses one to seven days later were compared with fetal and adult control implants without TGF-beta. The histology of the adult implant was characterized by an early acute inflammatory response: by day 7 fibroblasts and collagen were predominant. In contrast, control implants removed from fetal rabbits had no histologic evidence of acute inflammation or fibroblast penetration and no collagen was deposited. When implants containing 1.0 ng TGF-beta were removed from fetal rabbits at seven days, a grossly fibrotic reaction was observed: histology confirmed marked fibroblast penetration with collagen deposition. Fetal implants containing 0.01 ng or 10 ng TGF-beta showed few fibroblasts but had increased numbers of inflammatory cells compared with controls. These observations demonstrate that the fetal response becomes adultlike with fibroblast proliferation and collagen accumulation when TGF-beta is added, thus documenting the responsiveness of the fetal system to adult repair signals.(ABSTRACT TRUNCATED AT 250 WORDS)

Use of the Greenfield filter in adolescents for deep vein thrombosis and pulmonary embolism.

Serious venous thromboembolic disease is now recognized more frequently in the pediatric age group. Caval interruption is indicated most commonly for prophylaxis against life-threatening or recurrent pulmonary embolism (PE) when anticoagulation is ineffective or contraindicated. Greenfield vena caval filters have been utilized locally in 415 adult patients with 97% long-term patency and 5% recurrent embolization. Its application in adolescents is reported herein. Standard adult (30-mm) vena caval filters were placed in ten patients, ages 13 to 18. Four filters were required following PE, six were used for deep venous thrombosis (DVT) when anticoagulation was contraindicated, and one was inserted prophylactically. In eight patients, filter insertion was accomplished with local anesthesia, while two underwent filter placement under general anesthesia administered for other procedures. One filter was misplaced into the right renal vein, requiring a second filter insertion. All patients have been followed from 1 to 11 years with yearly vascular duplex imaging and radionuclide venograms documenting caval patency without clinical embolic sequelae. This duplicates the adult experience in safety and efficacy. As recognition of venous thromboembolism becomes more frequent in the pediatric age group, safe caval interruption may be necessary for those excluded from or not responsive to anticoagulation. This technique may be extended to smaller patients with miniaturization of both filter and carrier.

Cricoid split for subglottic stenosis in infancy.

Historically, tracheostomy has been used for infants with airway obstruction caused by congenital or acquired subglottic stenosis. Postoperative morbidity and mortality with this provisional operation led Cotton, in 1980, to substitute anterior cricoid split as the primary definitive procedure. Within the past three years, anterior cricoid split has been performed in 4 infants, aged 3 to 9 months, with acquired (3 patients) or congenital (1 patient) subglottic stenosis requiring ventilation through an endotracheal tube. Following cricoid split, the trachea is stented for 12 to 14 days by a nasotracheal tube, with extubation and rigid bronchoscopy in the operating room with the patient under anesthesia to confirm healing and patency. During an 18- to 24-month follow-up in these 4 patients, morbidity has been minimal, patency has persisted, and stridor has not recurred. Accordingly, a conclusive operation, cricoid split, rather than a temporizing tracheostomy may be employed for certain obstructive tracheal lesions early in life.

Multifocal ganglioneuroblastoma coexistent with total colonic aganglionosis.

Neuroblastoma and Hirschsprung's disease are considered aberrations of neural crest cell growth, migration, or differentiation. Their coexistence, however, is rare. We present the case of an only child with total colon Hirschsprung's disease diagnosed shortly after birth, who was found to have noncontiguous ganglioneuroblastomas without metastases at age 16 months. The spectrum of neural crest anomalies, long segment Hirschsprung's disease and multifocal neuroblastoma, in this child is unique and previously unreported.

Mortality with extracorporeal membrane oxygenation following repair of congenital diaphragmatic hernia in 93 infants.

The results of extracorporeal membrane oxygenation (ECMO) following repair of congenital diaphragmatic hernia (CDH) are analyzed for 93 neonates reported to the Neonatal ECMO Registry. Each infant was symptomatic at birth and developed life-threatening hypoxemia after herniorrhaphy. ECMO was used after neonates met criteria predictive of death in individual centers. These included (1) failure of medical therapy to reverse hypoxemia (35 neonates); (2) acute clinical deterioration making death appear likely (37 neonates); (3) AaDO2 greater than 600 for 8 hours (13 neonates); (4) oxygen index score of 51 for 4.8 hours (5 neonates); and (5) cardiac arrest (1 neonate). With ECMO, 52 infants (58%) survived and were discharged. Pre-ECMO arterial blood gas analysis was not predictive of outcome. Mortality was higher in small premature infants. ECMO corrected the hypoxemia, which usually causes death following congenital diaphragmatic herniorrhaphy. Unfortunately, 47 major bleeding complications occurred with 29 deaths. Therefore, improved survival may depend on new methods designed to prevent bleeding.

Fetal response to injury in the rabbit.

Fetal, neonatal, and adult tissue response to a standardized injury was studied using subcutaneous wound implants, linear incisions, and punch wounds in New Zealand white rabbits. In the fetus, sutured incisions healed by primary intention without antecedent inflammation. However, neither contraction nor healing by secondary intention was seen in punch or unsutured wounds. Healing both by primary and secondary intention following inflammatory infiltration was observed uniformly in neonatal and adult rabbits. Wound implants were extensively infiltrated with collagen in the adults studied; however, no collagen was seen in fetal implants and collagen hydroxyproline content could not even be detected by high performance liquid chromatography techniques; rather, a matrix rich in hyaluronic acid was found. The fetal tissue response to injury differs from the adult, proceeding in the absence of a classical inflammatory stimulus and lacking contractile capabilities. The deposition of extracellular matrix rich in hyaluronic acid but devoid of collagen suggests that the fetal response to injury may be a process more closely resembling regeneration or growth rather than repair by scar deposition.