The Science Journal of the American Association for Respiratory Care

1996 OPEN FORUM Abstracts

HIGH-FREQUENCY OSCILLATORY VENTILATION (HFOV) AS A TREATMENT MODALITY IN JARCHO-LEVIN SYNDROME.

Cynthia Bowman, RRT, Shoo Lee, MBBS, SM, FRCPC, FAAP. Dept. of Respiratory Care, Beth Israel Hospital, Boston, MA, & Centre for Evaluation Sciences, B.C. Research Institute for Child & Family Health, and Dept. of Pediatrics, B.C.'s Children's Hospital, University of British Columbia, Vancouver, B.C.

INTRODUCTION: Jarcho-Levin Syndrome (Spondylothoracic Dysostosis) is a congenital disorder characterized by extensive malformations and abnormal fusion of thoracic vertebrae and ribs, resulting in severely deformed thoracic cages, and a high neonatal mortality from respiratory insufficiency (only 7 survivors were reported in a series of 27 cases). We report a first case of Jarcho-Levin Syndrome successfully treated with HFOV.

CASE SUMMARY: A term, male infant was delivered to a 32 year old gravida 3, para 1 white woman by spontaneous vaginal delivery. Prenatal ultrasounds of the patient revealed multiple vertebral anomalies. Apgars were 6 at 1 minute, and 7 at 5 minutes. Shortly after delivery, the infant developed severe chest retractions, cyanosis, and decreased breath sounds and required endotracheal intubation and assisted ventilation. Despite extremely high peak inspiratory pressures of 50 cm H_{2}O and FiO_{2} of 1.0, the infant remained poorly ventilated, with minimal chest movements, diminished breath sounds, and transcutaneous oxygen saturations in the 70s. Chest radiograph confirmed extensive vertebral and rib anomalies, including hemivertebrae, butterfly vertebrae and absent vertebral bodies with pedicles. Both lungs appeared small and opacified. The infant was then placed on the Sensor Medics 3100A HFOV with a frequency of 10 Hz, mean airway pressure of 17 cm H_{2}O, delta p of 40 cm H_{2}O. IT ratio of 0.33, and FiO_{2} of 1.0. Initial venous blood gas was pH 7.22, PaCO_{2} 70, PaO_{2} 207, total CO_{2} 30 and base excess 0. Acidosis was corrected with intravenous sodium bicarbonate infusion, delta p was increased to 48 cm H_{2}O, and amplitude adjustments were based on visual assessment of chest vibrations. The infant developed persistent pulmonary hypertension of the newborn, confirmed by echocardiography and post-ductal desaturation, and required treatment with sodium bicarbonate and vasopressor infusions. Gradually, the infant's condition improved, and after three days, HFOV was changed to conventional ventilation with PIP 22, PEEP 5, rate 20/min, IT ratio 0.4, and FiO_{2} 1.0. On the fifth day of life, the infant was extubated to oxygen via nasal cannula at 100 cc/min and discharged home shortly after.

DISCUSSION: HFOV is an effective method of treatment for respiratory failure due to restrictive respiratory disease. It achieves oxygenation by maintaining or recruiting an "optimal lung volume" which maximizes ventilation-perfusion matching without compromising venous return and cardiac output. It is therefore an ideal method of treatment for Jarcho-Levin Syndrome, in which a small, deformed thoracic cage compromises ventilation. During the critical period following delivery, HFOV permits stabilization of the infant's cardio-pulmonary status while the lungs have an opportunity to become more complaint and adapt to extra-uterine life. HFOV can therefore be life-saving, and may improve the survival of patients with Jarcho-Levin Syndrome, who often lead full healthy lives if they survive the neonatal period.

Reference: OF-96-047

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