Imaging
Findings
Study shows aplastic right
lung with cardio-thoracic shift to the same side, and compensatory left
lung hyper-inflation.
Discussion
Agenesis
of lung is a rare anomaly. Bilateral agenesis has been described
in eight cases and is associated with multiple malformations that are incompatible
with life.
Classification
This
condition has been classified into three groups by Boyden (1955)
Group
1 – complete absence of one or both lungs (agenesis)
Group
2 – suppression of all but a rudimentary bronchus (aplasia)
Group
3 – abortive growth (Hypoplasia).
Initially
these patients are often misdiagnosed and treated as recurrent pneumonia
or collapse with consolidation. A high index of suspicion is required
during clinical examination so as to ask for appropriate radiological
investigations
Associated
Anomalies
Associated
anomalies are present in more than 50% patients. Multiple congenital anomalies
reported are cardiac defects such as ASD, VSD, PFO (patent foramen ovale),
A-V canal, TOF, PDA, single ventricle etc.
In
skeletal abnormalities the commonest is hemivertebrae and absent ribs,
causing scoliosis.
The
gastro-intestinal abnormalities include atresia, malrotation, tracheo-oesophageal
fistula, absent spleen and anomalies of diaphragm either on ipsilateral
or contralateral side.
The
genitourinary anomalies include renal agenesis, cystic kidneys and agenesis
of ovary or testis. Rarely brain and eye malformations
are also known. Ipsilateral facial abnormalities
have been also reported.
In
all such cases when patient does not improve after being diagnosed with
recurrent pneumonia, a differential diagnosis of pulmonary agenesis should
be considered especially if patient fails to improve with the routine
therapy.
Dr.
Ashok Raghavan, Manipal Hospital, Bangalore
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