An unusual solitary endobronchial pulmonary arteriovenous malformation in a 10 year-old child

Pulmonary arteriovenous malformations (PAVMs) are relatively rare in children. They may occur both as single isolated anomalies and, much more commonly, as one of many widely distributed arteriovenous malformations (AVMs) in other organs and anatomic locations such as the liver, brain, nose and spine. The latter phenotypically defines the condition known as Hemorrhagic Hereditary Telengiectasia (HHT). We report a case of a 10 year-old female with an isolated endobronchial right lower lobe PAVM. The patient's chest radiograph and fine-cut contrast-enhanced CT (CECT) of the neck and thorax were both uncharacteristically normal and, hence, non-diagnostic. Surgical lobectomy was the only means by which to both diagnose and treat this life-threatening condition. This unique case points out the dilemma facing the physician in such a case with only two management options neither of which is ideal: to either obtain a diagnosis with biopsy which is minimally invasive yet life-threatening due to the potential for fatal hemorrhage, or to perform a pulmonary lobectomy which entails removal of an entire lobe of the lung without a pre-operatively confirmed indication to justify the procedure. To our knowledge, this is the first reported case of a child with an endobronchial PAVM who did not carry the diagnosis of HHT, had normal chest radiography, and had a normal fine-cut CECT of the neck and thorax where such a management dilemma arose. This case points out that a high index of suspicion for PAVMs must be maintained despite normal chest radiography and CECT of the chest. In such cases, empiric lobectomy becomes the sole therapeutic and definitive diagnostic intervention. We hope that such a case published in the literature serves as a guide to physicians confronting similar circumstances as the rarity of such a constellation of variables precludes higher levels of evidence reporting such as a retrospective case series or prospective randomized controlled clinical trials.