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A case report: Pulmonary venous malformation complicated with pulmonary hemorrhage
Journal of Pediatric Surgery (2012) 47, E35–E38
www.elsevier.com/locate/jpedsurg
A case report: Pulmonary venous malformation complicated with pulmonary hemorrhage Nucharin Supakul a,⁎, Rong Fan b , Boaz Karmazyn a a
Department of Pediatric Radiology, Riley Hospital for Children, Indianapolis Indiana 46202-5200 United States Department of Pathology and Laboratory, Riley Hospital for Children, Indianapolis Indiana 46202-5200 United States
b
Received 15 June 2012; revised 12 September 2012; accepted 12 September 2012
Key words: Pulmonary venous malformation; Pulmonary arteriovenous malformation; Vascular malformation; Venous malformation; Lung perfusion scan; CT angiography of the chest
Abstract Pulmonary venous malformation is extremely rare. We present imaging and clinical findings of a 17-year-old male with multifocal subcutaneous venous malformations and multiple cystic lesions in the liver and spleen, suggestive of slow flow vascular malformation. In the right lung, chest radiography followed by chest CT demonstrated large tortuous pulmonary veins and cystic emphysematous changes. Tc99m-MAA (pertechnetate-labeled macroaggregated albumin) lung perfusion scan demonstrated only 3% of normal perfusion to the right lung, with no evidence of arteriovenous shunting. The child had diffuse intraparenchymal hemorrhage throughout the right lower and middle lobes and underwent resection. Pathology confirmed the diagnosis of venous malformation complicated with bleeding. © 2012 Elsevier Inc. All rights reserved.
Vascular malformation is a congenital defect of vascular morphogenesis and is categorized according to the type of vessel involved (capillary, venous, arterial, and lymphatic) and its hemodynamic features (high flow versus low flow). Two-thirds of vascular malformations are venous [1–3]. Venous malformations are often asymptomatic, but may become painful in cases of thrombophlebitis or muscular or articular involvement [4]. Venous malformation most commonly affects head and neck regions and lower extremities [1]. There are only few reported cases of venous malformation involving the lungs as part of Proteus syndrome and one as an isolated finding [2,5]. We present a child with multifocal venous malformation with a right lung involvement diagnosed based on chest
⁎ Corresponding author. Tel.: +1 317 948 6303; fax: +1 317 944 2920. E-mail address: [email protected] (N. Supakul). 0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2012.09.037
computed tomography (CT) scan and Tc99m-MAA (pertechnetate-labeled macroaggregated albumin) lungs perfusion scan and confirmed by pathology.
1. Case report An asymptomatic 17-year-old Caucasian male presented with multifocal venous malformations involving the left neck, right shoulder, left forearm, and right upper thigh. The neck venous malformation was resected and diagnosis was confirmed by pathology. The initial chest radiographs performed to evaluate potential thoracic involvement demonstrated emphysematous right lung with marked dilatation and tortuosity of the right lung vessels. This was further evaluated with post contrast CT scan of the chest and abdomen, which confirmed that the tortuous dilated vessels drain to the right pulmonary vein (Fig. 1). It also demonstrated multiple
E36
N. Supakul et al. seven months later, the right lung appeared almost completely opacified (Fig. 4); CT did not demonstrate any changes in the vascular malformation. He denied any new or worsening symptoms. Based on his underlying pulmonary venous malformation, it was thought that the child had pulmonary hemorrhage. Resection of the right lower and middle lobe was performed (Fig. 5). The right upper lobe was spared in surgery as it appeared normal. He recovered well after surgery. The patient's hospital course was lengthened by prolonged chest tube drainage and a new diagnosis of Berker muscular dystrophy with a secondary cardiomyopathy requiring permanent pacemaker
Fig. 1 Contrast-enhanced chest CT scan demonstrates right lung venous malformation that drains to the right pulmonary vein. A: Coronal CT scan demonstrates dilated vessels, some with a thrombus (arrows). There are multiple hypodensity lesions in the spleen suggestive of vascular malformation. B: Coronal volume rendering CT scan demonstrates draining of the malformed veins to a dilated right pulmonary vein (arrow).
hypodensity lesions in the liver and spleen suggestive of a slow flow vascular malformation (Fig. 2). Pulmonary function testing showed severe restrictive lung disease with no response to bronchodilator. Tc99m-MAA (pertechnetate-labeled macroaggregated albumin) lung perfusion scan demonstrated only 3% of normal perfusion to the right lung with no evidence of arteriovenous shunting (Fig. 3). This patient was asymptomatic and decided for conservative treatment. On routine follow up chest radiography
Fig. 2 A and B: CT scan of the abdomen in venous (A) and delayed (B) phases demonstrates hypodensity lesions in the liver and spleen with some filling in delayed images to suggest venous malformation.
Complicated pulmonary venous malformation
Fig. 3 Perfusion Tc-99 m MAA scan demonstrates about 3% of perfusion to the right lung, with no evidence of right-to-left shunting.
placement. His postoperative course was otherwise uneventful and he was discharged in good condition.
2. Discussion Venous malformations are found in about two-thirds of congenital vascular malformations, can occur anywhere in the body, but are most commonly observed in the head and neck (40%) and extremities (40%), and less often in the trunk (20%) [2,4]. Most venous malformations are solitary, but can be multiple [2]. In multiple venous malformations, they may be associated with chromosome abnormalities, or syndromes such as Proteus, blue rubber bleb nevus, or Maffucci [1,3–5]. Hereditary hemorrhagic telangiectasia (HHT, Osler– Weber–Rendu) is the most common vascular abnormality in the lungs and should be differentiated from venous malformation. The diagnostic criteria of HHT include spontaneous recurrent epistaxis, multiple telangiectasias in typical locations (skin, oral cavity or GI tract), visceral organ arteriovenous malformations (AVMs) (lung, liver, brain, and spine) or a firstdegree family member with HHT [1–3]. In this case, none of the clinical and imaging characteristics of HHT were met. Pulmonary venous malformation is extremely rare. It was reported in Proteus syndrome and was associated with cystic emphysematous changes [5] and also as a sporadic occurrence in a four year- old child with recurrent pneumonia and airway obstruction who was treated with surgical resection [2]. Our patient had multifocal venous malformations, including the neck, thigh, liver, spleen, and right lung, and was complicated with lung hemorrhage.
E37 Cross-sectional imaging may not differentiate between pulmonary arteriovenous malformation (PAVM) due to a lack of definite connection between arterial and venous sites in the lung. Radionuclide perfusion lung scanning may be needed to confirm right-to-left shunt in PAVM. In subjects without intrapulmonary shunt, peripheral intravenous injection of Tc-99 m MAA (particle diameter of 10 to 60 mm) or albumin microspheres (particle diameter of 7 to 25 mm) result in filtering of these particles by the capillaries of the lung. However, anatomic shunts with dilated pulmonary vascular channels will allow passage of these particles through the lungs, and subsequent filtering by capillary beds in other organs such as brain and kidneys [6]. In this patient, imaging findings were not characteristic of PAVM. CT scan did not demonstrate the characteristic findings of an enlarged feeding artery, aneurysmal malformation, and a draining vein. Albumin microspheres labeled with technetium99 m perfusion test did not show left to right shunting. Hemorrhage is a known complication of venous malformation [2]. Other complications include severe local pain (particularly when exercising), skin ulceration, or high cardiac output failure [2]. Treatment of vascular malformation is dependent on clinical symptoms, location, size, and whether infiltrative or well-localized. Treatment includes conservative treatment, surgery, sclerotherapy, or laser [1,4,7]. Management in asymptomatic patients may be conservative follow-up and surgery if complications occur. There is no report of management of pulmonary venous malformation by an interventional radiology procedure. If the pulmonary venous malformation drains to the azygous vein, an option is coiling the azygous vein from the internal jugular approach. However, this was not a feasible option in our case as it drained to the right pulmonary veins. Initially as the patient did not have any respiratory symptoms, the decision was not to operate but rather to
Fig. 4 Follow-up PA chest radiograph demonstrates complete opacity of the right middle and lower lobes.
E38
A
N. Supakul et al.
B
C
D
Fig. 5 A and B: Gross images of the right mid and lower lobes showed tan to red, heterogeneous, boggy parenchyma with focal distended air space. C: Histology demonstrates emphysematous changes and extensive venous malformation in the lung. There are multiple large venous lakes (arrows), one of the thrombose (T, arrow) and a vein with arterialized thickened wall (arrowhead). D: Lymphatic stain with D2-40: The lining of vascular spaces characterizing venous malformation is negative for D2-40. Some small lymphatic channels are highlighted and are useful internal controls.
follow up conservatively. During his routine follow up, he had worsening chest radiograph and was suspected of having pulmonary hemorrhage. The treatment option is only surgical resection. In summary, we presented an unusual case of a child with multifocal venous malformations with involvement of the right lung.
References [1] Enjolras O., Wassef M. and Chapot R. Color atlas of vascular tumors and vascular malformations. Cambridge University Press.
[2] Lee JY, Kim JH, Yoo SY, et al. Pulmonary venous malformation in a 4year-old boy: a case report. Korean J Radiol 2009;10(4):420-3. [3] Abernethy LJ. Classification and imaging of vascular malformations in children. Eur Radiol 2003;13:2483-97. [4] Puig S, Casati B, Staudenherzc A, et al. Vascular low-flow malformations in children: current concepts for classification, diagnosis and therapy. Eur J Radiol 2005;53:35-45. [5] Lim GY, Kim OH, Kim HW, et al. Pulmonary manifestations in Proteus syndrome: pulmonary varicosities and bullous lung disease. Am J Med Genet A 2011;155(4):865-9. [6] Gossage JR, Kanj G. Pulmonary arteriovenous malformations a state of the art review. Am J Respir Crit Care Med 1998;158(2):643-61. [7] Legehn GM, Heran MK. Venous malformation: classification, development, diagnosis and interventional radiologic management. Radiol Clin North Am 2008;46:545-97.
www.elsevier.com/locate/jpedsurg
A case report: Pulmonary venous malformation complicated with pulmonary hemorrhage Nucharin Supakul a,⁎, Rong Fan b , Boaz Karmazyn a a
Department of Pediatric Radiology, Riley Hospital for Children, Indianapolis Indiana 46202-5200 United States Department of Pathology and Laboratory, Riley Hospital for Children, Indianapolis Indiana 46202-5200 United States
b
Received 15 June 2012; revised 12 September 2012; accepted 12 September 2012
Key words: Pulmonary venous malformation; Pulmonary arteriovenous malformation; Vascular malformation; Venous malformation; Lung perfusion scan; CT angiography of the chest
Abstract Pulmonary venous malformation is extremely rare. We present imaging and clinical findings of a 17-year-old male with multifocal subcutaneous venous malformations and multiple cystic lesions in the liver and spleen, suggestive of slow flow vascular malformation. In the right lung, chest radiography followed by chest CT demonstrated large tortuous pulmonary veins and cystic emphysematous changes. Tc99m-MAA (pertechnetate-labeled macroaggregated albumin) lung perfusion scan demonstrated only 3% of normal perfusion to the right lung, with no evidence of arteriovenous shunting. The child had diffuse intraparenchymal hemorrhage throughout the right lower and middle lobes and underwent resection. Pathology confirmed the diagnosis of venous malformation complicated with bleeding. © 2012 Elsevier Inc. All rights reserved.
Vascular malformation is a congenital defect of vascular morphogenesis and is categorized according to the type of vessel involved (capillary, venous, arterial, and lymphatic) and its hemodynamic features (high flow versus low flow). Two-thirds of vascular malformations are venous [1–3]. Venous malformations are often asymptomatic, but may become painful in cases of thrombophlebitis or muscular or articular involvement [4]. Venous malformation most commonly affects head and neck regions and lower extremities [1]. There are only few reported cases of venous malformation involving the lungs as part of Proteus syndrome and one as an isolated finding [2,5]. We present a child with multifocal venous malformation with a right lung involvement diagnosed based on chest
⁎ Corresponding author. Tel.: +1 317 948 6303; fax: +1 317 944 2920. E-mail address: [email protected] (N. Supakul). 0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2012.09.037
computed tomography (CT) scan and Tc99m-MAA (pertechnetate-labeled macroaggregated albumin) lungs perfusion scan and confirmed by pathology.
1. Case report An asymptomatic 17-year-old Caucasian male presented with multifocal venous malformations involving the left neck, right shoulder, left forearm, and right upper thigh. The neck venous malformation was resected and diagnosis was confirmed by pathology. The initial chest radiographs performed to evaluate potential thoracic involvement demonstrated emphysematous right lung with marked dilatation and tortuosity of the right lung vessels. This was further evaluated with post contrast CT scan of the chest and abdomen, which confirmed that the tortuous dilated vessels drain to the right pulmonary vein (Fig. 1). It also demonstrated multiple
E36
N. Supakul et al. seven months later, the right lung appeared almost completely opacified (Fig. 4); CT did not demonstrate any changes in the vascular malformation. He denied any new or worsening symptoms. Based on his underlying pulmonary venous malformation, it was thought that the child had pulmonary hemorrhage. Resection of the right lower and middle lobe was performed (Fig. 5). The right upper lobe was spared in surgery as it appeared normal. He recovered well after surgery. The patient's hospital course was lengthened by prolonged chest tube drainage and a new diagnosis of Berker muscular dystrophy with a secondary cardiomyopathy requiring permanent pacemaker
Fig. 1 Contrast-enhanced chest CT scan demonstrates right lung venous malformation that drains to the right pulmonary vein. A: Coronal CT scan demonstrates dilated vessels, some with a thrombus (arrows). There are multiple hypodensity lesions in the spleen suggestive of vascular malformation. B: Coronal volume rendering CT scan demonstrates draining of the malformed veins to a dilated right pulmonary vein (arrow).
hypodensity lesions in the liver and spleen suggestive of a slow flow vascular malformation (Fig. 2). Pulmonary function testing showed severe restrictive lung disease with no response to bronchodilator. Tc99m-MAA (pertechnetate-labeled macroaggregated albumin) lung perfusion scan demonstrated only 3% of normal perfusion to the right lung with no evidence of arteriovenous shunting (Fig. 3). This patient was asymptomatic and decided for conservative treatment. On routine follow up chest radiography
Fig. 2 A and B: CT scan of the abdomen in venous (A) and delayed (B) phases demonstrates hypodensity lesions in the liver and spleen with some filling in delayed images to suggest venous malformation.
Complicated pulmonary venous malformation
Fig. 3 Perfusion Tc-99 m MAA scan demonstrates about 3% of perfusion to the right lung, with no evidence of right-to-left shunting.
placement. His postoperative course was otherwise uneventful and he was discharged in good condition.
2. Discussion Venous malformations are found in about two-thirds of congenital vascular malformations, can occur anywhere in the body, but are most commonly observed in the head and neck (40%) and extremities (40%), and less often in the trunk (20%) [2,4]. Most venous malformations are solitary, but can be multiple [2]. In multiple venous malformations, they may be associated with chromosome abnormalities, or syndromes such as Proteus, blue rubber bleb nevus, or Maffucci [1,3–5]. Hereditary hemorrhagic telangiectasia (HHT, Osler– Weber–Rendu) is the most common vascular abnormality in the lungs and should be differentiated from venous malformation. The diagnostic criteria of HHT include spontaneous recurrent epistaxis, multiple telangiectasias in typical locations (skin, oral cavity or GI tract), visceral organ arteriovenous malformations (AVMs) (lung, liver, brain, and spine) or a firstdegree family member with HHT [1–3]. In this case, none of the clinical and imaging characteristics of HHT were met. Pulmonary venous malformation is extremely rare. It was reported in Proteus syndrome and was associated with cystic emphysematous changes [5] and also as a sporadic occurrence in a four year- old child with recurrent pneumonia and airway obstruction who was treated with surgical resection [2]. Our patient had multifocal venous malformations, including the neck, thigh, liver, spleen, and right lung, and was complicated with lung hemorrhage.
E37 Cross-sectional imaging may not differentiate between pulmonary arteriovenous malformation (PAVM) due to a lack of definite connection between arterial and venous sites in the lung. Radionuclide perfusion lung scanning may be needed to confirm right-to-left shunt in PAVM. In subjects without intrapulmonary shunt, peripheral intravenous injection of Tc-99 m MAA (particle diameter of 10 to 60 mm) or albumin microspheres (particle diameter of 7 to 25 mm) result in filtering of these particles by the capillaries of the lung. However, anatomic shunts with dilated pulmonary vascular channels will allow passage of these particles through the lungs, and subsequent filtering by capillary beds in other organs such as brain and kidneys [6]. In this patient, imaging findings were not characteristic of PAVM. CT scan did not demonstrate the characteristic findings of an enlarged feeding artery, aneurysmal malformation, and a draining vein. Albumin microspheres labeled with technetium99 m perfusion test did not show left to right shunting. Hemorrhage is a known complication of venous malformation [2]. Other complications include severe local pain (particularly when exercising), skin ulceration, or high cardiac output failure [2]. Treatment of vascular malformation is dependent on clinical symptoms, location, size, and whether infiltrative or well-localized. Treatment includes conservative treatment, surgery, sclerotherapy, or laser [1,4,7]. Management in asymptomatic patients may be conservative follow-up and surgery if complications occur. There is no report of management of pulmonary venous malformation by an interventional radiology procedure. If the pulmonary venous malformation drains to the azygous vein, an option is coiling the azygous vein from the internal jugular approach. However, this was not a feasible option in our case as it drained to the right pulmonary veins. Initially as the patient did not have any respiratory symptoms, the decision was not to operate but rather to
Fig. 4 Follow-up PA chest radiograph demonstrates complete opacity of the right middle and lower lobes.
E38
A
N. Supakul et al.
B
C
D
Fig. 5 A and B: Gross images of the right mid and lower lobes showed tan to red, heterogeneous, boggy parenchyma with focal distended air space. C: Histology demonstrates emphysematous changes and extensive venous malformation in the lung. There are multiple large venous lakes (arrows), one of the thrombose (T, arrow) and a vein with arterialized thickened wall (arrowhead). D: Lymphatic stain with D2-40: The lining of vascular spaces characterizing venous malformation is negative for D2-40. Some small lymphatic channels are highlighted and are useful internal controls.
follow up conservatively. During his routine follow up, he had worsening chest radiograph and was suspected of having pulmonary hemorrhage. The treatment option is only surgical resection. In summary, we presented an unusual case of a child with multifocal venous malformations with involvement of the right lung.
References [1] Enjolras O., Wassef M. and Chapot R. Color atlas of vascular tumors and vascular malformations. Cambridge University Press.
[2] Lee JY, Kim JH, Yoo SY, et al. Pulmonary venous malformation in a 4year-old boy: a case report. Korean J Radiol 2009;10(4):420-3. [3] Abernethy LJ. Classification and imaging of vascular malformations in children. Eur Radiol 2003;13:2483-97. [4] Puig S, Casati B, Staudenherzc A, et al. Vascular low-flow malformations in children: current concepts for classification, diagnosis and therapy. Eur J Radiol 2005;53:35-45. [5] Lim GY, Kim OH, Kim HW, et al. Pulmonary manifestations in Proteus syndrome: pulmonary varicosities and bullous lung disease. Am J Med Genet A 2011;155(4):865-9. [6] Gossage JR, Kanj G. Pulmonary arteriovenous malformations a state of the art review. Am J Respir Crit Care Med 1998;158(2):643-61. [7] Legehn GM, Heran MK. Venous malformation: classification, development, diagnosis and interventional radiologic management. Radiol Clin North Am 2008;46:545-97.