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Sarcoidosis with Arteriovenous Malformation in a 15-Year-Old Girl - The Rarest of the Rare.

Qaiser I, Nayani K, Ahmed S, Ali R, Atiq M - Front Pediatr (2015)

Bottom Line: She responded to oral corticosteroids, her ACE and ALT levels improved.On a 3-year follow-up, her condition improved and she is clinically well.Regular follow-up visits should be scheduled to rule out new complications and to monitor the past ones.

View Article: PubMed Central - PubMed

Affiliation: Medical College, Aga Khan University , Karachi , Pakistan.

ABSTRACT

Introduction: Sarcoidosis is an uncommon multi-system disorder with many possible complications. Arteriovenous malformations (AVMs) are a rare vascular complication of sarcoidosis.

Case description: A 15-year-old girl presented to the Pediatric Clinic at AKUH with pulmonary, hepatic, joint, and skin manifestations. Physical examination and investigations pointed toward sarcoidosis, including raised erythrocyte sedimentation rate, angiotensin converting enzyme (ACE), and alanine transaminase (ALT). An incidental finding of pulmonary arteriovenous malformation (PAVM) was noticed on echocardiography. She responded to oral corticosteroids, her ACE and ALT levels improved. There was lack of indication for pulmonary angio-embolization for her PAVM. On a 3-year follow-up, her condition improved and she is clinically well.

Discussion: Pulmonary arteriovenous malformation is an extremely rare complication of sarcoidosis, especially among the pediatric population. Hence, this is the first reported case of its kind. The relation between sarcoidosis and PAVM is difficult to establish; however, there are some theories. This condition may be treated depending on the symptoms. Since our patient did not have any significant symptoms of PAVM, she was treated for the underlying disease, i.e., sarcoidosis.

Conclusion: While dealing with patients having multi-system disorders like sarcoidosis, one must be very vigilant so as not to miss out on any complication. Regular follow-up visits should be scheduled to rule out new complications and to monitor the past ones.

No MeSH data available.


Related in: MedlinePlus

Coronal CT images showing AVM on right and left side (arrow).
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Figure 2: Coronal CT images showing AVM on right and left side (arrow).

Mentions: Echocardiography was done to investigate cyanosis and breathlessness. Conventional echocardiography ruled out any structural lesions of the heart but bubble echocardiography showed contrast bubbles in left atrium after three cardiac cycles, suggestive of pulmonary AV malformation (Figure 1). CT angiogram was thus done and revealed three soft tissue density nodules. These were in the right upper lobe, right lower lobe, and a smaller one in the left lower lobe. Vessels were seen to be arising from the nodules. No signs of infection of interstitial lung disease were seen (Figure 2). All these findings were complementing pulmonary arteriovenous malformation (PAVM).


Sarcoidosis with Arteriovenous Malformation in a 15-Year-Old Girl - The Rarest of the Rare.

Qaiser I, Nayani K, Ahmed S, Ali R, Atiq M - Front Pediatr (2015)

Coronal CT images showing AVM on right and left side (arrow).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4585201&req=5

Figure 2: Coronal CT images showing AVM on right and left side (arrow).
Mentions: Echocardiography was done to investigate cyanosis and breathlessness. Conventional echocardiography ruled out any structural lesions of the heart but bubble echocardiography showed contrast bubbles in left atrium after three cardiac cycles, suggestive of pulmonary AV malformation (Figure 1). CT angiogram was thus done and revealed three soft tissue density nodules. These were in the right upper lobe, right lower lobe, and a smaller one in the left lower lobe. Vessels were seen to be arising from the nodules. No signs of infection of interstitial lung disease were seen (Figure 2). All these findings were complementing pulmonary arteriovenous malformation (PAVM).

Bottom Line: She responded to oral corticosteroids, her ACE and ALT levels improved.On a 3-year follow-up, her condition improved and she is clinically well.Regular follow-up visits should be scheduled to rule out new complications and to monitor the past ones.

View Article: PubMed Central - PubMed

Affiliation: Medical College, Aga Khan University , Karachi , Pakistan.

ABSTRACT

Introduction: Sarcoidosis is an uncommon multi-system disorder with many possible complications. Arteriovenous malformations (AVMs) are a rare vascular complication of sarcoidosis.

Case description: A 15-year-old girl presented to the Pediatric Clinic at AKUH with pulmonary, hepatic, joint, and skin manifestations. Physical examination and investigations pointed toward sarcoidosis, including raised erythrocyte sedimentation rate, angiotensin converting enzyme (ACE), and alanine transaminase (ALT). An incidental finding of pulmonary arteriovenous malformation (PAVM) was noticed on echocardiography. She responded to oral corticosteroids, her ACE and ALT levels improved. There was lack of indication for pulmonary angio-embolization for her PAVM. On a 3-year follow-up, her condition improved and she is clinically well.

Discussion: Pulmonary arteriovenous malformation is an extremely rare complication of sarcoidosis, especially among the pediatric population. Hence, this is the first reported case of its kind. The relation between sarcoidosis and PAVM is difficult to establish; however, there are some theories. This condition may be treated depending on the symptoms. Since our patient did not have any significant symptoms of PAVM, she was treated for the underlying disease, i.e., sarcoidosis.

Conclusion: While dealing with patients having multi-system disorders like sarcoidosis, one must be very vigilant so as not to miss out on any complication. Regular follow-up visits should be scheduled to rule out new complications and to monitor the past ones.

No MeSH data available.


Related in: MedlinePlus