Diffuse Lung Disease: Student/Resident Case Report Poster - Diffuse Lung Disease |

Deadly Complications of the Williams-Campbell Syndrome With Initial Presentation as Septic Shock in an Adult FREE TO VIEW

Kasra Sedarati, MD; Igor Aksenov, MD
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Memorial Health University Medical Center, Savannah, GA

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):533A. doi:10.1016/j.chest.2016.08.547
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SESSION TITLE: Student/Resident Case Report Poster - Diffuse Lung Disease

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Williams-Campbell Syndrome, named after Howard Williams and Peter Campbell, is a congenital abnormality resulting in the lack of cartilage in the distal bronchial tree. The lack of cartilage leads to a cystic bronchiectasis, which usually presents as recurrent pulmonary infections early in life.

CASE PRESENTATION: A 35 year old African American male presented in respiratory distress requiring intubation after 2 weeks of progressive fever and productive cough. He was born premature at 28 weeks and had a distant history of pneumonia at age 12 but was otherwise healthy. CXR showed bilateral lower lobe infiltrates and irregular lucencies. Laboratory data demonstrated Leukocytosis with bandemia, Lactic Acidosis, and a compensated respiratory acidosis. CT imaging revealed consolidation and ground glass attenuation within the bilateral lung bases and diffuse cystic disease. The patient required vasopressor support and broad-spectrum antibiotics, but was fully weaned off of vasopressor support on day 3 of hospitalization. Repeat bronchoscopies were unable to isolate a pathogen, growing only normal respiratory flora. Urine/Serologies for Alpha-1 antitrypsin, viral pathogens, mycobacterium, Legionella, Streptococcus, along with remainder of infectious workup was negative. Due to refractory hypoxemia despite high peep ventilator strategies, patient was started on extracorporeal membrane oxygenation (ECMO). He was later transferred to an ECMO specialty center where the remainder of care was provided. A video-assisted thoracoscopic surgery procedure offered a lung biopsy, which showed lack of cartilage in the distal bronchial tree and confirmed the diagnosis of Williams-Campbell syndrome.

DISCUSSION: Our case demonstrates an initial presentation of severe and deadly complication of the disease. There have been case reports indicating a possible genetic component to the disease, but there has yet to be an association with premature birth as in our patient.

CONCLUSIONS: Williams-Campbell syndrome, first noted in 1959, is a structural lung disease, which predisposes patients to the complications of cystic bronchiectasis. Generally, it is diagnosed early in life due to recurrent pulmonary infections and abnormal imaging. Further evaluation into the association between the disease and premature birth should be undertaken.

Reference #1: WILLIAMS H, CAMPBELL P. Generalized bronchiectasis associated with deficiency of cartilage in the bronchial tree. Arch Dis Child. 1960 Apr;35:182-91.

Reference #2: Palmer SM Jr, Layish DT, Kussin PS, Oury T, Davis RD, Tapson VF. Lung transplantation for Williams-Campbell syndrome. Chest. 1998 Feb;113(2):534-7.

Reference #3: Noriega Aldave AP, William Saliski D. The Clinical Manifestations, Diagnosis and Management of Williams-Campbell Syndrome. North American Journal of Medical Sciences. 2014;6(9):429-432. doi:10.4103/1947-2714.141620.

DISCLOSURE: The following authors have nothing to disclose: Kasra Sedarati, Igor Aksenov

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