Right-sided bronchial isomerism diagnosed in adulthood

Abstract

This case report describes a diagnosis of right-sided isomerism and specifically right-sided bronchial isomerism in a patient who was being investigated for deteriorating bronchiectasis. Right-sided bronchial isomerism is a variation of the normal bronchial anatomy (situs solitus) consisting of a left lung that is identically configured at the bronchial and lobar level to the right. It is sometimes referred to as bilateral right lung and is usually associated with congenital asplenia and therefore impaired immunity with susceptibility to pneumococcal sepsis and cardiac abnormalities which may be severe and result in a high mortality in infancy. Ivemark syndrome (also known as right atrial isomerism) combines these associations with malrotation of the gut and a midline liver.2 Interestingly, left-sided isomerism is associated with polysplenia as well as midline liver, malrotation of the gut, partially anomalous pulmonary venous drainage and cardiac septal defects.3 To the best of our knowledge cases of right-sided isomerism are sufficiently rare in adulthood that there are only two other reports in the literature and only one of the patients had bronchial isomerism.4,5.

Bronchial isomerism in a Kabuki syndrome patient with a novel mutation in MLL2gene

“…Several patients with bronchial isomerism present with severe respiratory injuries, such as: recurrent episodes of coughing, wheezing, asthma and bronchiectasies. In particular three patients diagnosed with severe asthma, classified as resistant to high-dose corticosteroid therapy, were found to have left bronchial isomerism [13,16,19]. With the detection of left-bronchial isomerism in our patient, in order to prevent his recurrent pulmonary infections, aggressive antibiotic combination together with a regular chest physiotherapy was started, improving his respiratory performances.…”

Common Congenital Anomalies of the Central Airways in Adults

“…In a bronchoscopy series by Beder, 475 of 1,114 patients werefound to have at least one tracheo-bronchial variation; trifurcation of the LMB was accounted in 0.1%. [92] This type of anatomical variation is usually associated with congenital asplenia and impaired immunity. [91] Theoretically, it is possible that the trifurcation of the LMB is a type of left tracheal bronchus.…”

“…[89] Bronchial atresia is most commonly found in the apico-posterior segmental bronchus of the left upper lobe, followed by segmental bronchus of the right upper, middle, and lower lobes, respectively. [92] There is no gender predilection. [85,91] When a bronchial atresia is found in the lower lobe, especially the postero-basal segment of the LLL, it is often associated with intralobar bronchopulmonary sequestration.…”

“…[92] There is no gender predilection. [86,88,92] The treatment is customized according to the symptoms. [85] In adults, bronchial atresia is usually diagnosed in the second to fourth decade of life but it can be found in elderly as well.…”