Pulmonary artery aneurysm (PAA) is an uncommon entity and is usually congenital in origin or sec-ondary to pulmonary arterial hypertension. Infections causing PAA are few, tuberculosis and bacterialinfections being the common causative organisms. There have been few cases reported previously, inwhich the organism causing PAA was found to be a rare fungus called mucor. Pulmonary mucormycosiscausing PAA is an infrequent and almost fatal complication as most of the diagnosis was made postmortem.
Pulmonary artery aneurysm (PAA) is an uncommon entity in amedical ICU as compared to the more prevalent aortic and intra-cranial aneurysms [1,2]. Most of them are found incidentally duringradiological investigations in adults and are commonly congenitalin origin. Mycotic or infectious pulmonary artery aneurysms arerare cause of acquired PAA. These are most commonly caused byTuberculosis, syphilis, bacteria like streptococcus and staphylo-coccus and rarely fungus like actinomycosis and candida [3,4].There are few scattered published cases describing pulmonarymucormycosis as causing PAA, with only very few survivals as antemortem diagnosis is difficult [5,6]. Pulmonary mucormycosis is arare opportunistic infection in patients with severe defects in anyone of the arms of their immune system like diabetes, chronic renalfailure, haematological malignancies, etc. We present a case reportof a successful management of a ruptured PAA caused by mucor-mycosis in a diabetic patient.
2. Case description
A 58 yr old male, diabetic since 7yrs was presented to theEmergency Room (ER) with fever, breathlessness, cough and hae-moptysis for the last two months which had increased in severitysince two days.
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One month back the patient had been evaluated for the abovecomplaints and a diagnosis of right sided tubercular empyema(Fig. 1) was made, for which a chest tube drain was inserted andwas prescribed anti tubercular treatment. The inter-costal drainwas removed subsequently a week later and patient was dis-charged. But, his symptoms recurred and he was brought to ourhospital with worsening of his condition.
In ER, on receiving, the patient was found to be pale, drowsy andhad obvious respiratory distress. He also had features of circulatoryshock with a blood pressure of 60/40 mm of Hg and increased heartrate of around 150 beats per minute. Immediate resuscitation wasstarted with intravenous fluids, inotropes and oxygen supple-mentation by face mask. On examination, he was found to have aright-sided tension pneumothorax and an ICD was immediatelyinserted to decompress it. His general condition graduallyimproved. The patient was stabilised and shifted to MedicalIntensive Care Unit (MICU).
A Computed Tomography (CT) of the chest done subsequentlyshowed a large area of consolidation in the apical segment of rightlower lobe (Fig. 2). He was put on broad spectrum intravenousantibiotics and his ATT (Anti Tubercular Treatment) was continued.His condition however did not improve. He continued to remaintachypneic and had recurrent episodes of massive haemoptysis(4e5 cupful). Subsequently Computed Tomography PulmonaryAngiography (CTPA) was done which revealed a saccular aneurysminvolving the distal part of right lower lobar pulmonary arterymeasuring 3.1 � 2.3 cmwith a neck diameter of 4.2 mm suggestiveof Pulmonary Artery Aneurysm (PAA) (Fig. 3).
nder the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Fig. 1. Chest X-ray showing right hydro pneumothorax.
Fig. 2. CT showing collapse consolidation in right lower lobe with cavitation in theapical segment.
Fig. 3. A saccular aneurysm in the right lower lobar pulmonary artery.
L. Ramachandran et al. / Respiratory Medicine Case Reports 16 (2015) 71e7372
Few hours later, the patient suddenly went into respiratorydistress and shock. His trachea was immediately intubated andmechanical ventilation was initiated. CT chest was done whichshowed right sided complete air space opacification probablyhaemorrhage. Suspecting it to be a ruptured PAA, patient wasimmediately shifted to the OT, where laparotomy and right lowerlobe lobectomy with PAA clipping was done. The lobectomy spec-imen was sent for Histo Pathological Examination. (HPE).
Post operatively, he continued to have fever with rising TLC(Total Leukocyte Count) counts. He also had frequent recurrentright middle lobe and upper lobe collapse for which frequentbronchoscopic suctioning for mucus plug removal was done.Meanwhile, the HPE report of the lobectomised lung tissue wasreported as invasive mucormycosis (Fig. 4) which was sensitive toamphotericin. Liposomal Amphotericin B was added at a dose of200 mg per day (4 mg/kg/day). He responded well to the antifungaland became afebrile. His TLC counts normalised, oxygenation
improved and there was significant radiological Clearance. He wasrapidly weaned off the ventilator and extubated. Post extubation,he was unable to clear secretions adequately because of his respi-ratory muscle weakness and poor cough reflex. So, on third day ofextubation, he underwent minitracheostomy for adequate clearingof the secretions. Gradually, with aggressive chest physiotherapyand regular suctioning, the patient improved andwas subsequently‘discharged on request’ in a stable condition (with mini-tracheostomy in situ) to a primary health care centre. Unfortu-nately, Patient was lost in follow up.
3. Discussion
Pulmonary mucormycosis which was previously found only inimmunocompromised individuals like solid organ transplant re-cipients, patients on immunosuppressant, haematological malig-nancies etc., has now emerged as a life-threatening infection inpatients with diabetes in medical ICU [7e9]. Most of the cases arediagnosed post-mortem, hence early suspicion and detection cancurb the high mortality rate.
Mucormycosis belongs to the genusmucor, order mucorales andclass zygomycetes which survive on decaying organic matter.Humans are mainly infected through inhalation of the spores [10].
Pulmonary mucormycosis is the second most common form ofhuman mucormycosis after the rhino-orbital-cerebral form andpresents with fever, chest pain, cough and rapidly progressingbreathlessness and occasionally as life-threatening massive hae-moptysis [8]. Rarely, it can also present with vocal cord palsy [9].Radiological examination may show a wedge-shaped lobarconsolidation, cavitation or an air crescent sign which indicates alife threatening vascular invasion by mucor and requires a promptintervention [11]. Its uniqueness lies in its ability to invade thevessel wall causing thrombophlebitis and thrombosis leading tolarge parenchymal infarctions and occasionally, aneurysm andpseudo aneurysm formations in the pulmonary vascular system [4].
Pulmonary artery aneurysm is the focal dilatation of pulmonaryvessels beyond 29 mm of diameter on CT scan [2,12]. A true
Fig. 4. H & E stained section showing sparsely septate irregular obtuse fungal hyphae(broken arrows) in a necrotic background (solid arrows). X10X.
L. Ramachandran et al. / Respiratory Medicine Case Reports 16 (2015) 71e73 73
aneurysm involves all three layers of the vessel wall whereas, apseudo aneurysm is just a leaking haematoma layered by sur-rounding soft tissues. PAA are mostly congenital. Acquired formsare less common and are usually secondary to pulmonary hyper-tension associated with chronic pulmonary emboli or obstructiveairway disease, mitral stenosis and other veno-occlusive diseases[13]. Mycotic aneurysms due to infections are very rare and aremainly associated with tuberculosis (Rossenmuesen aneurysm)which shows a characteristic peripheral distribution of aneurysms[14,15]. We believe, in the present case, mucor was directlyresponsible for the development of PAA. Mucor had encroachedupon the vessel wall from the surrounding parenchyma causinginflammation and weakening of the vessel wall leading to theaneurysm formation.
Pulmonary CT angiography is the gold standard to diagnose PAA[12]. Combination of antifungal with surgical resection of theinvolved tissue is the best approach advocated [16]. Surgicalresection is necessary as medical treatment alone has highermortality because of the angioinvasive nature of the fungus. Sur-gical intervention is preferred earlier if the patient presents withhaemoptysis. Surgical modalities now include aneurysectomy, lo-bectomy or pneumectomy [17]. If the aneurysm does not involvethe main pulmonary trunk, nonsurgical techniques like embolo-therapy or endovascular coiling is preferred [18].
Liposomal Amphotericin B is the recommended antifungalagent. The dose and duration of therapy is individualised depend-ing on the clinical response, clearing in the radiological findings
and negativity of the cultures. Posaconazole is the alternative drugagainst mucorales species for patients who are refractory or intol-erant to amphotericin therapy [15].
In a nutshell, PAA is a deadly complication of pulmonarymucormycosis. For early diagnosis, keep a high index of suspicion inhigh risk patients like diabetes, chronic renal failure, haemato-logical malignancies, etc. who are not responding to conservativemanagement. Lung biopsy and pulmonary angiography to confirminfection and aneurysm respectively are necessary, when routineinvestigations are inconclusive. Multimodality approach combiningmedical therapy and surgical or endovascular intervention is thebest approach observed although data available is limited. In ourview, a good post-operative rehabilitation care also contributes toimproved results.
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