Abstracts of Staff Publications
aFall 1999
The use of balloon-expandable metallic stents in the treatment of pediatric tracheomalacia and bronchomalacia
Robert Furman, Carl Backer, Michael Dunham, James Donaldson, Constantine Mavroudis, and Lauren Holinger
From the Department of Otolaryngology—Head and Neck Surgery, University of Illinois (RF); Northwestern University (MD, LH), and the divisions of Cardiovascular-Thoracic Surgery (CB, CM) and Radiology (JD), Children's Memorial Hospital, Chicago.
Archives of Otolaryngology, Head and Neck Surgery 1999;125:203–207
Objectives: To evaluate the use of balloon-expandable metallic stents in the treatment of children with tracheomalacia and bronchomalacia in whom conventional therapy has failed.
Design: Retrospective case series.
Setting: Tertiary pediatric otolaryngology and cardiothoracic surgery referral center.
Patients: Six patients were identified as having undergone bronchoscopic placement of metallic balloon-expandable stents between 1994 and 1997. The age at stent placement, prior surgical interventions, and indications for and sites of stent placement were noted. Also, the complications related to stent placement and the current airway status of the patients were reviewed.
Interventions: Twelve balloon-expandable metallic angioplasty stents (Palmaz; Johnson & Johnson Interventional Systems Co, Warren, NJ) were placed bronchoscopically in 6 patients. Six stents were placed in the lower trachea, and 6 were placed in the main bronchi. The stents were balloon expanded under fluoroscopic guidance.
Main Outcome Measure: Discontinuation of mechanical ventilation.
Results: The age at stent placement ranged from 1.5 to 38 months (mean age at placement, 10 months). The indications for stent placement were (1) tracheomalacia or bronchomalacia, (2) pericardial patch or slide tracheoplasty failure, and (3) bronchomalacia caused by tetralogy of Fallot and large pulmonary arteries. The primary complication of stent placement was postoperative granulation tissue formation. One patient required the removal of 2 tracheal stents because of granulation tissue formation. There were 2 deaths in the series, 1 possibly related to stent placement. Four of the 6 patients were weaned from mechanical ventilation, and 3 experienced prolonged relief of airway obstruction.
Conclusions: Metallic balloon-expandable stents are effective in relieving lower tracheomalacia and bronchomalacia in select patients. Only patients in whom conventional therapy has failed should be considered for stent placement.
Exercise studies in tetralogy of Fallot: A review
Hans Wessel, Milton Paul
From Children's Memorial Hospital, Chicago.
Pediatric Cardiology 1999;20:39–47
Exercise evaluation of patients with congenital heart disease in general and tetralogy of Fallot in particular has had much less impact on clinical care than that of adults with coronary heart disease. Of over 4000 references on tetralogy of Fallot in the literature since 1965, only 87 involve exercise. These studies were reported from 56 institutions, with 39 originating from the United states and Canada, 40 from Europe, and 8 from Japan. They involved 3157 patients, studied mostly after intracardiac repair (ICR). In reviewing these data one is struck by the wide range of different approaches to exercise testing. This is in part related to the different purposes of each exercise study, but it also relates to the lack of uniform standards for exercise testing in patients with congenital heart disease. The studies involved maximal treadmill exercise and maximal or submaximal cycle ergometer work, in the upright or supine position, utilizing 52 different work protocols, the most common being the Bruce treadmill protocol (employed 27 times). Despite this lack of standardization, exercise evaluation has significantly contributed understanding of the natural history and pathophysiology of surgically corrected tetralogy of Fallot.
Child and adolescent injury and death from urban firearm assaults: Association with age, race, and poverty
Elizabeth Powell and Robert Tanz
From the divisions of Pediatric Emergency Medicine (EP) and General Academic Pediatrics and the Violent Injury Prevention Center (RT); Department of Pediatrics, Northwestern University Medical School, Chicago.
Injury Prevention 1999;5:41–47
Objective: To describe rates and trends in the incidence of non-fatal and fatal firearm assault among children (16 years old or younger) over an 11 year period in Chicago, Illinois, and to identify the socioeconomic characteristics of community areas where assaults are common.
Methods: The Chicago Police Department (CPD) records from 1986 through 1996 were reviewed for children assaulted with a firearm. United States census data for 1990 for Chicago were used to calculate incidence rates; census data were also used for community area (defined by census tract) socioeconomic descriptions.
Results: The CPD recorded 11,163 pediatric firearm assaults during the study period: 10,571 non-fatal and 592 (5%) fatal. From 1986 through 1996 non-fatal assaults more than doubled, with the highest rates in 1994; fatal assaults tripled, with rates peaking in 1993–94. Significant increases in non-fatal firearm assaults occurred among black and Hispanic males and females. In 1994, compared with white males, the relative risk of non-fatal assault was 7.0 (95% confidence interval (CI) 5.3 to 9.1) for black males and 3.3 (95% CI 2.5 to 4.4) for Hispanic males; the relative risk was 1.5 (95% CI 1.1 to 2.1) for black females. A handgun was the firearm used in most assaults (88% of non-fatal and 84% of fatal).
Within community areas, the correlation between non-fatal
and fatal assault incidence was strong (r=0.80, p<0.001). The proportion of families with income below the 1989 poverty level ($12,674) and the percent black race in the community area
together accounted for 70% of the variance in assault rates.
Conclusions: From 1986 to 1994 there were significant increases in both non-fatal and fatal firearm assaults, usually by handguns; thereafter, rates declined. Urban children who were victims of non-fatal firearm assault appear to come from the same population as those who suffer fatal assaults. Black and Hispanic youth living in poverty were at particular risk.
Features of Alagille syndrome in 92 patients: Frequency and relation to prognosis
Karan Emerick, Elizabeth Rand, Elizabeth Goldmuntz, Ian Krantz, Nancy Spinner, and David Piccoli
From the Department of Pediatrics and the divisions of Gastroenterology and Nutrition, Cardiology, and Genetics at the Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia. (Dr. Emerick is now at Children's Memorial Hospital.)
Hepatology 1999;29:822–829
We have studied 92 patients with Alagille syndrome (AGS) to determine the frequency of clinical manifestations and to correlate the clinical findings with outcome. Liver biopsy specimens showed paucity of the interlobular ducts in 85% of patients. Cholestasis was seen in 96%, cardiac murmur in 97%, butterfly vertebrae in 51%, posterior embryotoxon in 78%, and characteristic facies in 96% of patients. Renal disease was present in 40% and intracranial bleeding or stroke occurred in 14% of patients. The presence of intracardiac congenital heart disease was the only clinical feature statistically associated with increased mortality (P<.001). Initial measures of hepatic function in infancy including absence of scintiscan excretion were not predictive of risk for transplantation or increased mortality. The hepatic histology of these AGS patients showed a significant increase in the prevalence of bile duct paucity (P=.002) and fibrosis (P<.001) with increasing age. Liver transplantation for hepatic decompensation was necessary in 21% (19 of 92) of patients with 79% survival 1-year post-transplantation. Current mortality is 17% (16 of 92). The factors that contributed significantly to mortality were complex congenital heart disease (15%), intracranial bleeding (25%), and hepatic disease or hepatic disease or hepatic transplantation (25%). The 20-year predicted life expectancy is 75% for all patients, 80% for those not requiring liver transplantation, and 60% for those who required liver transplantation.
Air embolism during tunneled central catheter placement performed without general anesthesia in children: A potentially serious complication
Frank Morello, James Donaldson, Martha Saker, and Jackson Norman
From Children's Memorial Hospital, Northwestern University Medical School, Chicago.
Journal of Vascular and Interventional Radiology 1999;10:781–784
Central venous catheters have had an increasingly important role in a variety of patient care situations, including long-term antibiotic therapy, chemotherapy, and nutritional support. The recent past has seen a gradual transition from placement of vascular access catheters by surgeons to placement by interventional radiologists. The interventional radiology service places a majority of the vascular access devices at our children's hospital, including peripherally inserted central catheters, tunneled central venous catheters, temporary and permanent hemodialysis catheters, and subcutaneous ports. Most procedures performed by our interventional radiology service in children can be successfully completed with use of intravenous (IV) sedation, and a few require general anesthesia (GA). Key advantages GA over IV sedation include the ability to have positive pressure ventilation (PPV) or controlled apnea during the procedure. We report our experience of venous air embolism in three small children during placement of tunneled central venous catheters when GA was not used.
Modification of Cotrel-Dubousset's original hook constructs for idiopathic scoliosis
Brian Grossman, John Sarwark, Richard Lim, Michael Schafer, Stuart Montgomery, Vincenzo DeRosa, and Kellen Choi
From the Department of Orthopaedic Surgery, Northwestern University Medical School, and the division of Pediatric Orthopedic Surgery, Children's Memorial Hospital, Chicago.
Journal of Pediatric Orthopaedics 1999;19:500–503
We performed a retrospective review of 41 patients (ages 9–18 years) who underwent posterior spinal fusion with either Isola or Cotrel-Dubousset (CD) instrumentation to determine whether the presence of an apical hook on the thoracic convexity affected initial and long-term sagittal and coronal correction in adolescent idiopathic scoliosis surgery. A study group of 38 female and three male patients was evaluated (2–5 years of follow-up). Twenty-three patients (Group A) were treated with an up-going hook at the convex apex of the thoracic curve, and 18 patients (Group B) with similar curves were instrumented without an apical hook. Results showed that Group A's average preoperative coronal curve of 48° decreased to 17° whereas Group B's preoperative average of 52° decreased to 25°.
At follow-up, no statistical significance was noted in either coronal curve correction (p=0.203)or sagittal kyphosis (p=0.38) between Groups A and B. We concluded that omission of the up-going hook at the apex of the thoracic covexity can reduce postoperative discomfort in patients undergoing posterior spinal fusion, without sacrificing curve correction or balance.
Treatment of mercury intoxication
Carl Baum
From the Division of Emergency Medicine, Children's Memorial Hospital, Chicago.
Current Opinion in Pediatrics 1999;11:265–268
The element mercury exists as inorganic, elemental, or organic species. Routes of exposure and toxicity in humans vary according to the species of mercury involved. Treatment of mercury poisoning generally requires the use of sulfhydryl bond-containing chelation agents, including the parenterally administered dimercaprol and its oral congeners. These oral chelators, meso-2,3 dimercaptosuccinic acid and sodium 2,3-dimercapto-1- propanesulfonate, have numerous advantages over dimercaprol, including less toxicity. Although dimercaprol is contraindicated in organic mercury exposures, meso-2,3-dimercaptosuccinic acid and sodium 2,3-dimercapto-1-propanesulfonate may be used to chelate all species of mercury. Recent evidence suggests that their efficacy in organic mercury poisoning is uncertain.
Histopathology of congenital subglottic stenosis
Lauren Holinger
From the Division of Pediatric Otolaryngology, Children's Memorial Hospital and the Department of Otolaryngology—Head and Neck Surgery, Northwestern University Medical School, Chicago.
Annals of Otology, Rhinology and Laryngology 1999;108:101–111
A precise delineation of each laryngeal abnormality is critical to planning rational therapy. Since congenital cricoid cartilage anomalies are easily overlooked, a systematic approach to their identification is as important as a clear concept of their classification. The purpose of this communication is to present the pathologic findings and classification of congenital cricoid cartilage deformities and to summarize updated information derived by the whole organ macrosection technique. The classification of congenital subglottic stenosis is based on study of the English-language literature and the collection in the Laryngeal Development Laboratory at the Children's Memorial Hospital in Chicago. Twenty-nine specimens with congenital cricoid abnormalities have been identified. Fourteen have an elliptical cricoid cartilage; 12, a laryngeal cleft; 5, a flattened shape; 1, a large anterior lamina; and 3, generalized thickening. Some specimens have more than 1 anomaly. Eight cases of fragmented or distorted cricoid cartialges are thought to be acquired lesions. An anterior submucous cricoid cleft has been identified and is presented for the first time.
Infantile acropustulosis revisited: History of scabies and response to topical corticosteroids
Anthony Mancini, Ilona Frieden, and Amy Paller
From the departments of Pediatrics and Dermatology, Northwestern University Medical School and Children's Memorial Hospital, Chicago, and the departments of Pediatrics and Dermatology, University of California, San Francisco.
Pediatric Dermatology 1998;15:337–341
Infantile acropustulosis (IA) is a condition of young children characterized by recurrent episodes of pruritic vesicles and pustules in an acral distribution. Several reports describe patients with scabies infestation prior to the diagnosis of IA, although the relationship between the two remains unclear. Furthermore, optimal therapy is controversial. We reviewed the history of scabies and response to therapy in 21 patients diagnosed with IA at two institutions between 1983 and 1997. A history of prior treatment for scabies was noted in 14 patients, although only two had mites, feces, or ova detected on microcospic examination for diagnostic verification. All patients were treated with topical corticosteroids (4 with class I, 12 with class II, 3 with class III, 1 with class IV, and 1 with class VI).
All 18 patients who returned for follow-up experienced significant improvement or cleared completely with treatment. There were no observed cutaneous or systemic side effects from corticosteroid therapy. We conclude that a history of preceding scabies is common in patients with IA, but often this diagnosis is made without microscopic confirmation. We also demonstrate that mid- to high-potency topical corticosteroids are a safe and effective first-line therapy for patients with IA.
The favorable impact of arrhythmia surgery on total cavopulmonary artery fontan conversion
Constantine Mavroudis, Barbara Deal, Carl Backer, and Christopher Johnsrude
From the divisions of Cardiovascular-Thoracic Surgery and Pediatric Cardiology, Children's Memorial Hospital, Northwestern University Medical School, Chicago.
Pediatric Cardiac Surgery Annual of the Seminars in Thoracic and Cardiovascular Surgery 1999;2:143–156
Patients who have undergone atriopulmonary Fontan procedures are at risk for significant atrial arrhythmias and obstructive connections, which have been related to intra-atrial suture lines, atrial enlargement, and somatic growth. Twenty patients (mean age,
17.3±6.8 years) had conversion to total cavopulmonary artery connection 8.9±2.1 years after the previous Fontan procedure (for atrial arrhythmias in 19 patients and for obstructive lesions in one). Arrhythmia ablative surgery evolved over the study period from "arrhythmia circuit cryoablation" (cryoablation lesions completing lines of block) to the more standard approaches of modified right-sided Maze and Maze-Cox III procedures. Preoperative functional New York Heart Association class was IV in nine patients, III in nine, and II in two. All patients survived. Two patients had prolonged chest drainage (there was pericardial effusion in one).
The average length of hospital stay was 11.3 ± 5.4 days; chest tubes were removed on day 8.5±5.4. There were no long-term deaths (mean follow-up period, 20.3±14.9 months; range, 2 months to 4 years). Late postoperative arrhythmias occurred in two patients who are receiving long-term antiarrhythmic medications. All patients have improved to New York Heart Association class I or II. Total cavopulmonary artery Fontan conversion in association with modified right-sided Maze or Maze-Cox III procedures and pacemaker placement can be accomplished with low morbidity and mortality, and results in functional class improvement and control of life-threatening arrhythmias.
