The Science Journal of the American Association for Respiratory Care

2002 OPEN FORUM Abstracts

Case Report: Proactive Invasive Pulmonary Function Testing for Surgical Intervention in a Barnes Syndrome Patient:

Timothy Cox, RRT, Thomas Blackson, RRT., Thomas Shaffer, PhD, David Paul., MD, Kerry Weis, M.D.; A. I. duPont Hospital for Children, Wilmington, De. Christiana Care Health System, Newark, De.; St. Peters Hosp., New Brunswick , N.J.

Introduction: Barnes Syndrome and Jeune asphyxiating thoracic dystrophy are rare, complicated, syndromes, that when symptomatic, can result in significant ventilatory impairment in the affected newborn. We report a case in which pulmonary mechanics measurements, utilizing esophageal manometry, were utilized to determine the relative contribution of the chest wall and lung to the decrease in total thoracic compliance experienced by a newborn with Barnes Syndrome. The results of the pulmonary evaluation were utilized to assist in establishing acceptable ventilator settings as well as to serve as a pre-surgical screening tool prior to consideration of lateral thoracic expansion.

Case Report: The infant was a full term, day 36, female weighing 3.2 kg. She was born with obvious thoracic dystrophy, and was breathing through a 3.5mm ID tracheostomy tube with mechanical ventilatory assistance at the time of the evaluation. The ventilator was set to deliver PCV ventilation, IMV RR: 40/ min., PIP: 26 cm H2O, PEEP: 7 cm H2O, TI: 0.4 sec., FIO2: 0.4. The patient was instrumented with a 6fr. esophageal pressure monitoring catheter and a differential pressure transducer at the airway, (PEDS system, MAS inc., Hatfield, PA), for data collection.


Parameter Predicted Normal Test Results Percent Predicted
Compliance Total 0.8- 1.3 ml/ cm H2O/kg 0.224 ml/cm H2O/kg 23%
Compliance, Lung 1.0-1.5 ml/ cm H2O/kg 0.24 ml/cm H2O/kg 19%
Compliance, Chest Wall 4.2 ml/cm H2O/kg 2.7 ml/cm H2O/kg 64%
Resistance, Total 29 – 40 cm H2O/L/sec 138.8 cm H2O/L/sec 397%
Resistance, Insp. 29 – 40 cm H2O/L/sec 105.4 cm H2O/L/sec 303%
Resistance, exp. 29 – 40 cm H2O/L/sec 129.9 cm H2O/L/sec 371%
FRC 24 – 30 ml/kg 10.1 ml/kg 37%
C20/Ctotal >1.0 2.5 250%

Discussion: These findings indicate that the chest wall and lungs were contributing to the decrease in total compliance in this infant. In addition, the elevation in pulmonary resistance may appear secondary to the low FRC. Based on this data, the ventilatory management plan was directed towards reducing PIP ventilatory support, increasing PEEP for FRC management, and emphasizing chest wall stabilization to allow growth and maturation.

Clinical Implications/Prognosis: Surgical intervention through the use of lateral thoracic expansion is a safe and effective procedure for infants with asphyxiating thoracic dystrophy. It is most commonly indicated after the first year of life. Initial management of patients includes a lung protective strategy aimed at acceptable ventilation while attempting to avoid further respiratory damage from mechanical ventilation. This case demonstrates the use of advanced pro-active pulmonary function assessment in the early stabilization and management of a patient destined to require long term respiratory care. Finally, serial pulmonary function studies may be warranted following surgery.


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