Download PDF
CC BY-NC-ND 4.0 · Journal of Health and Allied Sciences NU 2012; 02(02): 63-67
DOI: 10.1055/s-0040-1703576
Case Report

PULMONARY AGENESIS-GROUP I WITH UPAA DIAGNOSED IN ELDERLY GERIATRIC AGE

Raghav Sharma
1   Professor, Department of Medicine, K.S. Hegde Medical Academy, Deralakatte, Mangalore - 575 018
,
Vidya Upadhyaya
1   Assistant Professor, Department of Radio Diagnosis, K.S. Hegde Medical Academy, Deralakatte, Mangalore - 575 018
,
Nazir Attar
1   Professor & Unit Head, Department of Medicine, K.S.Hegde Medical Academy, Deralakatte, Mangalore - 575 018
› Author Affiliations
› Further Information
  PDF Download Permissions and Reprints

Abstract

Pulmonary agenesis and UPAA (unilateral pulmonary artery agenesis) is a rare developmental lung defect in which there is complete absence or hypoplasia of one or both lungs. It can be either isolated or associated with other congenital anomalies. The percentage of diagnosis of this condition during life has risen considerably, however it is true that more than 50% of the patients with this condition die in the first year of life. The symptoms and severity of this condition is often variable. Here with we present a male patient with left upper lobe pulmonary agenesis with left unilateral pulmonary artery agenesis (UPAA) which was detected and diagnosed for the first time at the ripe age of 70 years. Here we also review the relevant literature regarding the condition and the radiology including CT scan is described.

Keywords

Pulmonary agenesis - Agenesis of left upper lobe - Unilateral pulmonary artery agenesis-UPAA - Congenital lung malformations - CT chest


Publication History

Article published online:
02 March 2020

© .

Thieme Medical and Scientific Publishers Private Ltd.
A-12, Second Floor, Sector -2, NOIDA -201301, India

 

  • References

  • 1 Borja AR, Ransdell HT, Villa S. Congenital developmental arrest of the lung, Ann thorax Surg 1970; 10:317-26
  • 2 Rare diseases.info.nih.gov/GARD-genetic and rare diseases information centre.
  • 3 Fokstunen S, Schinzel A. Unilateral lobar pulmonary agenesis in sibs, J Med Genet 2000;37:557-9
  • 4 Kadir IS, Thekudan J, congenital unilateral pulmonary artery agenesis and aspergilloma Ann. Thorac Surg 2002; 74(6):2169-2171
  • 5 Schaffer AJ, Pulmonary agenesis and hypoplasia. In Schaffer AJ, Avery ME eds. Disease of the new born. Philadelphia: WB Saunders, 1960:124-209.
  • 6 Wexels P: Agensis of lung. Thorax, 6:171,1951
  • 7 JP Booth, CL Berry, Unilateral pulmonary agenesis. Arch, Dis Childh, 1967, 42,361.
  • 8 Mendelson DS, Hahn P, Cohen BA, Hypoplasia of the lung-CT appearance, Mount Sinai J Med 1986; 53:297-298.
  • 9 Schaffer AJ, Rider AV, A note on the prognosis of pulmonary agenesis and hypoplasia according to the side affected. J Thorac Surg 1957; 33:379-82.