Revisit on congenital bronchopulmonary vascular malformations: A haphazard branching theory of malinosculations and its clinical classification and implication

Abstract

We propose a haphazard branching theory to support the concept of bronchopulmonary malinosculations, and we apply this theory to classify congenital bronchopulmonary vascular malformation (BPVM) based on the anatomical results we have found. Between January 1990 and December 1997, a total of 22 pediatric patients (10 male and 12 female), aged 2 days to 14 years (median, 19.6 months), with congenital BPVM were enrolled in this retrospective study. Study modalities include the clinical features and plain chest films (n = 22) plus at least two of the following: echocardiography (n = 13), barium esophagraphy (n = 2), bronchoscopy (n = 4), contrast bronchography (n = 8), high-resolution direct coronal CT (n = 1) and electron beam or ultrafast CT (n = 1) of the chest, MRI (n = 10), MRA (n = 1), contrast cineangiocardiography (n = 9), surgery (n = 11), or autopsy (n = 2). The salient clinical features were recurrent lung infections in 14 patients, acute respiratory distress in 13, associated cardiovascular malformations in 8, dextroversion in 7, congestive heart failure in 7, dextrocardia in 4, and complex congenital heart diseases in 4. There were abnormal openings (malinosculations) of the pulmonary airway in 20 patients: to an artery in 12, to a vein in 8, and to the lung parenchyma in 9. These 22 patients with congenital BPVM can be classified into bronchial malinosculation (10 cases), arterial malinosculation (2 cases), and bronchoarterial malinosculation (10 cases). Congenital BPVM can be classified in terms of bronchopulmonary malinosculation based upon a haphazard branching theory, in which abnormal communications between two independent systems (primitive foregut system and aortic-pulmonary arch system) occurred coincidentally rather than causally.

Isolated and Complex Scimitar Vein Anomalies and Their Differentiation from the Meandering Right Pulmonary Vein

“…Midline shifting of the heart in dextroposition (rather than dextrocardia) is a secondary effect due to hypoplasia of the right lung and should not be included as a criterion for defining scimitar syndrome. In scimitar syndrome, the systemic artery could supply either a segment or a lobe of a normal lung,4 an abnormal lung without bronchopulmonary sequestration,3 or an abnormal lung with bronchopulmonary sequestration 5-7. Neither the dextroposed heart, nor bronchopulmonary sequestration is a sine qua non criterion for defining scimitar syndrome.…”

A Systematic Classification of the Congenital Bronchopulmonary Vascular Malformations: Dysmorphogeneses of the Primitive Foregut System and the Primitive Aortic Arch System

“…We propose a "haphazard theory" to substantiate this concept of PM 3. However, there is no category in which to classify patients with isolated scimitar vein anomalies and meandering right pulmonary veins 3. Nevertheless, isolated scimitar vein anomalies have been observed in our clinical practice15 and in that of others' 16.…”

“…Clements et al proposed a "wheel theory" to explain the serial formation of congenital BPVM, and coined "pulmonary malinosculation" (PM) to classify these malformations. We propose a "haphazard theory" to substantiate this concept of PM 3. However, there is no category in which to classify patients with isolated scimitar vein anomalies and meandering right pulmonary veins 3.…”

“…Congenital bronchopulmonary vascular malformations (BPVM) encompass a broad spectrum of diseases involving abnormal communication or anastomosis of one or more components of the lung 1-3. Despite early definition of the sequestration spectrum,4-7 and scimitar syndrome,8,9 reports of incomplete-, atypical-, and pseudo-sequestration/scimitar syndrome continued to prevail in the literature, which implies that a universal understanding of the sequestration spectrum and scimitar syndrome is still lacking.…”

Transcatheter occlusion of the isolated scimitar vein anomaly camouflaged under dual pulmonary venous drainage of the right lung by the Amplatzer Ductal Occluder

“…First of all, scimitar vein may as well exist as an isolated anomaly [1], without the other two characteristics of the scimitar syndrome, i.e., hypoplasia of the ipsilateral pulmonary artery and bronchial airway, and a systemic arterial supply to the involved lung [2], in that the patterning anomalies involving pulmonary airway, pulmonary arterial supply, and pulmonary venous drainage may occur independently within the lung parenchyma [3]. Many authors have contributed to the understanding of early development of the pulmonary venous circulation [4][5][6][7].…”