Isolated Endobronchial Pulmonary Arteriovenous Malformation presenting with Massive Hemoptysis

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ESPR455
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History (including chief complaint, history of present illness and relevant past and family medical history)

A 15-year-old previously healthy male was initially seen by an outpatient ENT for two days of a persistent cough with expectoration of frank blood and blood clots. There were no reports of recent fevers, congestion, vomiting or previous nose bleeds. No reports of any sick contacts or any recent travel. 2 months prior to his initial presentation the patient did have a few days of sinus headaches associated with a postnasal drip. A pertinent family history included a brother with occasional nose bleeds during allergy season and a maternal uncle with Wardenberg Syndrome. 

Once at the ENT office, the anterior vessel in his right nare was cauterized and the patient was discharged. Within 12 hours the epistaxis reoccurred, and the patient was taken to an ER where a nasopharyngeal swab testing for SARS CoV-2 was performed. This prompted massive epistaxis leading to hypovolemic shock and acute hypoxic respiratory failure requiring intubation.  The patient was then transferred to Westchester Medical Center

The patient was resuscitated with NS and 2 Units of PRBC's. Rhinoscopy was performed and unable to identify an origin of bleeding.  Sinus CT demonstrated right paranasal sinusitis and a chest radiograph(Figure 1) showed consolidation of the left lower lobe. The patient was started on empiric antibiotic treatment for a presumed pneumonia involving the left lower lobe. Within 5 days the bleed was controlled, and the patient was discharged home. 

The patient returned to the ED the following morning with hemoptysis, hematemesis and epistasis. 


Physical examination findings (including vital signs)

Temp-97.5F HR 170 BP 70/30 RR 34 02 Sats 86%  

Vent settings- SIMV VC TV 400 P10 R30 FiO2 1.0

General-Intubated, sedated

HEENT- Dried blood at the nares

Cardio- Tachycardia, decreased cap refill

Respiratory-Diminished breath sounds to the bases, bilateral fine crackles, no wheezing

Gi- Soft, non-distended 


Laboratory or Diagnostic imaging or Procedures

Rhinoscopy and an upper GI endoscopy were normal and did not show any signs of bleeding in the nasopharyngeal region or the upper gastrointestinal region. A chest CT with contrast was performed which was significant for patchy ground glass, nodular and consolidate opacities consistent with pulmonary hemorrhage. 

Emergent bronchoscopy was performed which demonstrated a highly vascular polyploid lesion in the proximal portion of the left lower lobe just distal to bifurcation of the mainstem bronchus. (Figure 1) An endobronchial blocker was placed to tamponade the bleeding. 

The patient was taken to the OR for emergent left lower lobectomy. Intra-operative bronchoscopy helped identify the lesion thereby ensure that the bronchus was divided proximal to the lesion. 

On post-operative day #2 the patient had repeated bronchoscopy with lavage and cryotherapy to remove residual intra-bronchial blood clots. His chest x-ray improved post bronchoscopy. His chest tube was removed on post-operative day 3 and he was discharged home on POD #12. 

Final pathology showed abnormal bronchial blood vessels with dilation, mural thickening, irregular muscularization and a loss of elastic laminae, consistent with a localized arteriovenous malformation which can be associated with hereditary hemorrhagic telangiectasia. 

Genetic testing for Hereditary Hemorrhagic Telangiectasia, Familial Aortopathy and Ehlers Danlos was conducted, and results were all negative. 


Final Diagnosis/Discussion

Endobronchial lesions are rare in pediatrics as are pulmonary arteriovenous malformations(AVM) presenting as endobronchial lesions. Here we have described a 15-year-hold boy who presented with massive hemoptysis and was found to have an Endobronchial AVM. Genetic testing for Hereditary Hemorrhagic Telangiectasia, Familial Aortopathy and Ehlers Danlos was conducted, and results were all negative. Such an isolated lesion has only been described in a limited number of studies. Our patient was successfully managed using a multi-disciplinary approach to a final diagnosis and treatment.



 

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Westchester Medical Center
Westchester Medical Center/Maria Fareri Children's Hospital
Westchester Medical Center
Westchester Medical Center
Westchester Medical Center
Westchester Medical Center

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