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Ann Thorac Surg 2000;69:S308-S318
© 2000 The Society of Thoracic Surgeons

Congenital Heart Surgery Nomenclature and Database Project: vascular rings, tracheal stenosis, pectus excavatum

^a Department of Surgery, Northwestern University Medical School, Division of Cardiovascular-Thoracic Surgery, Children’s Memorial Hospital, Chicago, Illinois, USA

Address reprint requests to Dr Backer, Division of Cardiovascular-Thoracic Surgery, Children’s Memorial Hospital, 2300 Children’s Plaza, m/c 22, Chicago, IL 60614
e-mail: c-backer{at}nwu.edu

Presented at the International Nomenclature and Database Conferences for Pediatric Cardiac Surgery, 1998–1999.

Abstract

The extant nomenclature for vascular rings, tracheal stenosis, and pectus deformities is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Vascular rings are subclassified as double aortic arch, right arch/left ligamentum, pulmonary artery sling, and innominate compression. Tracheal stenosis is subclassified as congenital complete tracheal rings (localized or long-segment) or acquired postintubation types. Pectus deformities are subclassified as pectus excavatum and carinatum (mild, moderate, severe). A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

I. Background

Vascular rings are congenital anomalies of the aortic arch system where vascular structures encircle and compress the trachea and esophagus. In anatomic terms, there are two types of vascular rings; those that are complete and have vascular structures completely encircling the trachea and esophagus, and rings that are incomplete and only compress a portion of the esophagus or trachea without encircling it as a complete ring. These two types of tracheoesophageal compression anomalies present with the same symptoms, are evaluated in a similar fashion, and have similar surgical interventions, hence it is logical to group them together under the heading, vascular ring, even though some are not true complete anatomic rings. One type of vascular ring, the pulmonary artery (PA) sling, is very commonly (almost 50% of cases) associated with congenital tracheal stenosis. In addition, children with congenital tracheal stenosis can present with symptoms similar to children with anatomically complete vascular rings such as: stridor, retractions, dyspnea, apnea, and respiratory distress. Pectus excavatum, and less commonly, pectus carinatum, are congenital chest wall deformities found in children. Pectus excavatum is a depression of the sternum caused by concave development of the distal cartilages. Pectus carinatum is an abnormal elevation of the sternum caused by convex development of the costal cartilages. Both deformities can be associated with congenital heart defects.

II. Analysis: vascular rings

The phrase vascular ring was first used by Robert Gross in his report describing the first successful division of a double aortic arch in 1945 [1]. Since that time, the phrase vascular ring has been used to refer to a group of congenital vascular anomalies that encircle and compress the esophagus and trachea. In that original article by Gross, he described the two classic true anatomic vascular rings, double aortic arch and right aortic arch with a left ligamentum. The other two tracheoesophageal compression syndromes that have been included under the heading, vascular ring, are innominate artery compression syndrome (IACS) and PA sling. IACS occurs when there is an abnormally distal and posterior takeoff of the innominate artery and it compresses the trachea anteriorly as it courses from the left of the mediastinum to the right arm [2]. A PA sling occurs when the left pulmonary artery (LPA) originates from the right pulmonary artery (RPA) and encircles the distal trachea and right mainstem branches as it courses between the trachea and esophagus to the left lung [3]. Another type of vascular anomaly, which was earlier thought to cause symptoms in children, but usually does not, is the aberrant origin of the right subclavian artery as the most distal brachiocephalic branch from a normal left aortic arch. This anomaly is actually found in a significant number of normal people, 0.5% of the population [4].

The embryonic development of vascular rings was detailed by Edwards [5]. In the embryonic aortic arch system, the ventral and dorsal aorta are connected by six primitive aortic arches. Initially, the first, second, and fifth arches involute and a double aortic arch is formed. Some individuals remain at this stage and hence have a double aortic arch. In normals, the right fourth arch involutes and the left fourth arch persists as the aortic arch. If the left fourth arch involutes, a right arch is formed, and if the ligamentum goes to the descending thoracic aorta, a vascular ring results.

An early monograph from the Mayo Clinic divided vascular rings into 7 types [6]:

Type A. Double aortic arch with both arches patent and a left ductus or ligamentum.

Type A₁. Double aortic arch associated with coarc tation of the aorta.

Type B. Double aortic arch with atresia of the left arch distal to the origin of the left subclavian artery and left ligamentum.

Type C. Right aortic arch with aberrant left subcla vian artery and left ductus or ligamentum arteriosum.

Type D. Right aortic arch with mirror image branch ing and left ligamentum arteriosum.

Type E. Left aortic arch, right descending aorta, right ductus arteriosus.

Type F. Anomalous LPA (PA sling).

Type G. Left aortic arch with aberrant right subcla vian artery.

Although some authors have attempted to use this classification (ie, a patient had a Mayo type B vascular ring,), our preference has been to simply identify each vascular ring by describing its anatomic configuration rather than use a difficult to remember lettering or numerical system [7].

Over 95% of all vascular rings easily fit into 4 main categories:

Double aortic arch

Right arch/left ligamentum

Innominate artery compression

PA sling

Double aortic arch is conveniently grouped into 3 categories, dominant right arch, dominant left arch, and balanced arches. This has been our grouping of choice in over 300 patients with vascular rings [8], and was Langlois’ grouping of choice in nearly 500 patients with vascular rings [9]. Right aortic arch is grouped into 2 main categories: retroesophageal left subclavian (left subclavian artery is the last brachiocephalic branch), and mirror image branching (there is a left innominate artery which is the first brachiocephalic branch that gives rise to the left subclavian and left carotid arteries) [8–10]. Some authors have used the phrase, aberrant left subclavian artery to refer to the left subclavian artery when it is the last brachiocephalic branch in a patient with a right aortic arch. We have found that this is confusing with the diagnosis aberrant right subclavian and hence recommend the phrase, retroesophageal left subclavian. Aberrant origin of the right subclavian artery from a normal left arch rarely causes symptoms in children, and hence probably should not be considered a vascular ring [11].

The true vascular rings, double aortic arch and right arch/left ligamentum are usually surgically approached through a left thoracotomy [8]. There are only a few rare vascular rings that need to be approached through a right thoracotomy [12]. Innominate artery compression syndrome can be approached by right or left thoracotomy for innominate arteriopexy [13]. Innominate artery compression syndrome can also be treated by innominate artery reimplantation rightward of the original takeoff [14]. PA sling is now preferentially approached through a median sternotomy with the use of cardiopulmonary bypass (CPB) [15]. This has the advantage of optimizing the vascular anastomosis and simultaneously addressing the frequently associated tracheal stenosis. We have advocated the use of left pulmonary artery (LPA) reimplantation into the main pulmonary artery (MPA) anterior to the trachea [15], however others have reported good results with tracheal transection and LPA translocation without a vascular anastomosis [16].

Vascular rings: hierarchy level 1

Vascular ring

Vascular ring hierarchy level 1 definitions
Vascular ring
A congenital vascular anomaly causing compression of the trachea and/or esophagus. The compression may be from a complete anatomic ring or from a sling-like effect of an aberrant vessel.

Vascular ring hierarchy level 2

Vascular ring, NOS

Vascular ring, Double aortic arch

Vascular ring, Right aortic arch/left ligamentum

Vascular ring, Innominate artery compression

Vascular ring, PA sling

Vascular ring, Other

Vascular ring hierarchy level 2 definitions

Double aortic arch
There are two distinct aortic arches (right and left) encircling the trachea and esophagus. They may be patent or atretic.

Right arch/left ligamentum
The aortic arch is to the right of the trachea. The ligamentum extends from the MPA to the descending thoracic aorta completing the vascular ring.

Innominate compression
The innominate artery compresses the trachea anteriorly by more than 75% of the original tracheal lumen.

PA sling
The LPA originates from the RPA. The LPA courses posteriorly between the trachea and esophagus in its route to the left lung hilum.

Vascular ring hierarchy level 3

Vascular ring, NOS

Vascular ring, Double aortic arch, NOS

Vascular ring, Double aortic arch, Balanced arches

Vascular ring, Double aortic arch, Left arch dominant

Vascular ring, Double aortic arch, Right arch dominant

Vascular ring, Right aortic arch/left ligamentum, NOS

Vascular ring, Right aortic arch/left ligamentum, Mirror image branching

Vascular ring, Right aortic arch/left ligamentum, Retroesophageal left subclavian artery (aberrant left subclavian artery)

Vascular ring, Right aortic arch/left ligamentum, Circumflex aorta (left sided descending thoracic aorta)

Vascular ring, Innominate artery compression

Vascular ring, PA sling, NOS

Vascular ring, PA sling, With tracheal stenosis

Vascular ring, PA sling, With tracheomalacia

Vascular ring, PA sling, With tracheal stenosis and tracheomalacia

Vascular ring, PA sling, Without tracheal stenosis or tracheomalacia

Vascular ring, Other

Vascular ring hierarchy level 3 definitions

Left arch = apex of arch to left of trachea.

Right arch = apex of arch to right of trachea.

Dominant arch = larger vessel of the two arches.

Balanced arches = arches of equal size.

Mirror image branching
The brachiocephalic vessel origins are mirror image to the normal left arch. The first vessel is a left innominate artery giving rise to the left carotid and left subclavian artery, the second vessel is the right carotid artery, the third vessel is the right subclavian artery.

Retroesophageal left subclavian artery
The left subclavian artery originates as the last brachiocephalic vessel from the descending thoracic aorta, usually immediately adjacent to the left ligamentum. This site is posterior (retro) to the esophagus. (Synonym: aberrant left subclavian artery).

Vascular ring hierarchy level 4

Vascular ring, NOS

Vascular ring, Double aortic arch, NOS

Vascular ring, Double aortic arch, Balanced arches

Vascular ring, Double aortic arch, Left arch dominant, NOS

Vascular ring, Double aortic arch, Left arch dominant, Right arch atretic

Vascular ring, Double aortic arch, Left arch dominant, Right arch patent

Vascular ring, Double aortic arch, Right arch dominant, NOS

Vascular ring, Double aortic arch, Right arch dominant, Left arch atretic

Vascular ring, Double aortic arch, Right arch dominant, Left arch patent

Vascular ring, Right aortic arch/left ligamentum, NOS

Vascular ring, Right aortic arch/left ligamentum, Mirror image branching

Vascular ring, Right aortic arch/left ligamentum, Retroesophageal left subclavian artery (aberrant left subclavian artery)

Vascular ring, Right aortic arch/left ligamentum, Circumflex aorta (left sided descending thoracic aorta)

Vascular ring, Innominate artery compression

Vascular ring, PA sling, NOS

Vascular ring, PA sling, With tracheal stenosis

Vascular ring, PA sling, With tracheomalacia

Vascular ring, PA sling, With tracheal stenosis and tracheomalacia

Vascular ring, PA sling, Without tracheal stenosis or tracheomalacia

Vascular ring, Other

Vascular ring modifiers

Vascular ring modifier, Double aortic arch, Coarctation left arch

Vascular ring modifier, Double aortic arch, Coarctation right arch

Vascular ring modifier, Double aortic arch, Left ligamentum

Vascular ring modifier, Double aortic arch, Right ligamentum

Vascular ring modifier, Double aortic arch, Left patent ductus arteriosus

Vascular ring modifier, Double aortic arch, Right patent ductus arteriosus

Vascular ring modifier, Right aortic arch/left ligamentum, Kommerell’s diverticulum

Vascular ring modifier, Right aortic arch/left ligamentum, Circumflex aorta (right aortic arch and left descending thoracic aorta)

Vascular ring modifier, Right aortic arch/left ligamentum, Ligamentum is patent

Additional vascular ring modifier comments

Oftentimes a double aortic arch will be associated with a ligamentum arteriosum, which in some rare cases can be a patent ductus arteriosus. The double aortic arch modifiers therefore include left ligamentum, right ligamentum, left patent ductus arteriosus, and right patent ductus arteriosus. Alternatively, the patent ductus arteriosus can be included in the database as a second diagnosis. In a similar fashion, the ligamentum in a patient with a right aortic arch and left ligamentum may in some very rare cases be patent.

Rare vascular rings

Vascular ring, Left aortic arch/aberrant right subclavian artery [11, 17]

Vascular ring, Left aortic arch/aberrant right subclavian artery-aneurysm base right subclavian artery

Vascular ring, Left cervical aortic arch [18]

Vascular ring, Right cervical aortic arch

Vascular ring, Left aortic arch/right descending aorta, right ligamentum [19]

Vascular ring, Left aortic arch/right descending aorta, right patent ductus arteriosus [20]

Vascular ring, Ductus arteriosus sling [21]

Vascular ring, Hemitruncal sling [22]

Vascular ring, Right aortic arch/right ligamentum/absent LPA [23]

Vascular ring, Right aortic arch/right patent ductus arteriosus/absent LPA

III. Nomenclature of vascular ring treatment options

Vascular ring treatment hierarchy level 1

Vascular ring repair

Vascular ring treatment hierarchy level 2

Vascular ring repair, NOS

Vascular ring repair, Double aortic arch

Vascular ring repair, Right aortic arch/left ligamentum

Vascular ring repair, Innominate artery compression

Vascular ring repair, PA sling

Vascular ring repair, Other

Vascular ring treatment hierarchy level 3

Vascular ring repair, NOS

Vascular ring repair, Double aortic arch, NOS

Vascular ring repair, Double aortic arch, Right arch ligation and division

Vascular ring repair, Double aortic arch, Right arch division and oversewing

Vascular ring repair, Double aortic arch, Right arch division using clips

Vascular ring repair, Double aortic arch, Left arch ligation and division

Vascular ring repair, Double aortic arch, Left arch division and oversewing

Vascular ring repair, Double aortic arch, Left arch division using clips

Vascular ring repair, Right aortic arch/left ligamentum, NOS

Vascular ring repair, Right aortic arch/left ligamentum, Ligation and division of ligamentum

Vascular ring repair, Right aortic arch/left ligamentum, Division and oversewing of ligamentum

Vascular ring repair, Right aortic arch/left ligamentum, Division of ligamentum between clips

Vascular ring repair, Right aortic arch/left ligamentum, Aortic uncrossing procedure

Aortic uncrossing procedure
An unusual group of patients with a right aortic arch and left ligamentum will have a left sided descending thoracic aorta, the so called circumflex aorta. For these patients the aortic uncrossing procedure may be required [24]. With CPB and circulatory arrest, the arch is mobilized, divided, and the descending aorta is anastomosed to the ascending aorta to the left and anterior to the trachea.

Vascular ring repair, Innominate artery compression, NOS

Vascular ring repair, Innominate artery compression, Pex innominate artery to sternum

Vascular ring repair, Innominate artery compression, Divide and reimplant innominate artery

Vascular ring repair, PA sling, NOS

Vascular ring repair, PA sling, Divide LPA and reimplant into MPA anterior to trachea

Vascular ring repair, PA sling, Transect trachea and translocate LPA anterior to trachea

Vascular ring repair, Other

Vascular ring repair modifiers

Vascular ring repair modifier, Location of arch division, Between ascending aorta/left arch

Vascular ring repair modifier, Location of arch division, Between ascending aorta/right arch

Vascular ring repair modifier, Location of arch division, Between left carotid/left subclavian

Vascular ring repair modifier, Location of arch division, Between left subclavian/descending aorta

Vascular ring repair modifier, Location of arch division, Between right carotid/right subclavian

Vascular ring repair modifier, Location of arch division, Between right subclavian/descending aorta

IV. Analysis tracheal stenosis

Tracheal stenosis in infants and children is most often secondary to a congenital anomaly commonly known as complete tracheal rings. When a child has a complete tracheal ring there is absence of the normal posterior membranous trachea and the cartilage ring is circumferential and complete. The number of complete rings in any one patient can range from 1 to over 20 (essentially, the entire trachea from cricoid to carina). The rings can also extend into the right or left main bronchus. In patients with complete tracheal rings, one third will have an associated PA sling, and one fourth will have a significant cardiac anomaly. These patients often present with severe respiratory distress, and attempts to intubate with a normal sized endotracheal tube (for age) are often unsuccessful. In an early review, Benjamin demonstrated that medical management is associated with a 40% to 50% mortality [25]. A classification of congenital tracheal stenosis was proposed by Cantrell and Guild in 1964 [26]. They classified congenital tracheal stenosis in these groups: I. segmental stenosis; II. funnel-like stenosis; and III. generalized hypoplasia.

In most cases, the area where the complete tracheal rings begins and ends narrows and widens over several rings in a "funnel" like nature. Another cause of tracheal stenosis in children is postintubation cicatricial scar formation. This is less common than in adults because typically pediatric endotracheal tubes are not cuffed.

The surgical treatment of congenital tracheal stenosis has evolved considerably over the past two decades. Originally, tracheal resection was the only technique described [27], however this is not applicable to most long-segment stenosis. Different types of tracheoplasty (tracheal patch augmentation) have been reported. These include, pericardial [28], cartilage [29], and slide tracheoplasty [30]. We have reported the use of a tracheal autograft [31]. Others have reported the use of tracheal homograft [32] and pulmonary or aortic homograft [33].

Tracheal stenosis hierarchy level 1

Tracheal stenosis

Tracheal stenosis
A reduction in the anatomic luminal diameter of the trachea by more than 50% of the remaining normal trachea.

Tracheal stenosis hierarchy level 2

Tracheal stenosis, NOS

Tracheal stenosis, Congenital-complete tracheal rings

Tracheal stenosis, Post intubation

Tracheal stenosis, Traumatic

Tracheal stenosis, Congenital web

Tracheal stenosis, Other

Tracheal stenosis hierarchy level 3

Tracheal stenosis, NOS

Tracheal stenosis, Congenital-complete tracheal rings, NOS

Tracheal stenosis, Congenital-complete tracheal rings, Localized (less than 50% tracheal length)

Tracheal stenosis, Congenital-complete tracheal rings, Long-segment (more than 50% of tracheal length)

Tracheal stenosis, Post intubation, NOS

Tracheal stenosis, Post intubation, Localized (less than 50% tracheal length)

Tracheal stenosis, Post intubation, Long-segment (more than 50% of tracheal length)

Tracheal stenosis, Traumatic, NOS

Tracheal stenosis, Traumatic, Localized (less than 50% tracheal length)

Tracheal stenosis, Traumatic, Long-segment (more than 50% of tracheal length)

Tracheal Stenosis, Congenital web

Tracheal Stenosis, Other

Tracheal stenosis modifiers

Tracheal right upper lobe (RUL)

PA sling

Intracardiac anomaly

Tracheomalacia

Bridging bronchus [34]

Number of complete rings

Subglottic stenosis

Bronchial stenosis

Bronchomalacia

V. Tracheal stenosis treatment options

Tracheal stenosis treatment hierarchy level 1

Tracheal procedure

Tracheal stenosis treatment hierarchy level 2

Tracheal procedure, NOS

Tracheal procedure, Resection with end-to-end anastomosis

Tracheal procedure, Pericardial tracheoplasty

Tracheal procedure, Rib cartilage tracheoplasty

Tracheal procedure, Slide tracheoplasty

Tracheal procedure, Tracheal autograft reconstruction

Tracheal procedure, Tracheal homograft reconstruction

Tracheal procedure, Homograft (aortic or PA) reconstruction

Tracheal procedure, Tracheoplasty (Other)

Tracheal procedure, Balloon dilatation

Tracheal procedure, Stent placement

Tracheal procedure, Stent dilation

Tracheal stenosis treatment hierarchy level 3

Tracheal procedure, NOS

Tracheal procedure, Resection with end-to-end anastomosis, NOS

Tracheal procedure, Resection with end-to-end anastomosis, Cervical

Tracheal procedure, Resection with end-to-end anastomosis, Intrathoracic

Tracheal procedure, Resection with end-to-end anastomosis, Intrathoracic with carinal reconstruction

Tracheal procedure, Pericardial tracheoplasty, NOS

Tracheal procedure, Pericardial tracheoplasty, Cervical

Tracheal procedure, Pericardial tracheoplasty, Intrathoracic

Tracheal procedure, Pericardial tracheoplasty, Intrathoracic with carinal reconstruction

Tracheal procedure, Rib cartilage tracheoplasty, NOS

Tracheal procedure, Rib cartilage tracheoplasty, Cervical

Tracheal procedure, Rib cartilage tracheoplasty, Intrathoracic

Tracheal procedure, Rib cartilage tracheoplasty, Intrathoracic with carinal reconstruction

Tracheal procedure, Slide tracheoplasty, NOS

Tracheal procedure, Slide tracheoplasty, Cervical

Tracheal procedure, Slide tracheoplasty, Intrathoracic

Tracheal procedure, Slide tracheoplasty, Intrathoracic with carinal reconstruction

Tracheal procedure, Tracheal autograft reconstruction, NOS

Tracheal procedure, Tracheal autograft reconstruction, Cervical

Tracheal procedure, Tracheal autograft reconstruction, Intrathoracic

Tracheal procedure, Tracheal autograft reconstruction, Intrathoracic with carinal reconstruction

Tracheal procedure, Tracheal homograft reconstruction, NOS

Tracheal procedure, Tracheal homograft reconstruction, Cervical

Tracheal procedure, Tracheal homograft reconstruction, Intrathoracic

Tracheal procedure, Tracheal homograft reconstruction, Intrathoracic with carinal reconstruction

Tracheal procedure, Homograft (aortic or PA) reconstruction, NOS

Tracheal procedure, Homograft (aortic or PA) reconstruction, Cervical

Tracheal procedure, Homograft (aortic or PA) reconstruction, Intrathoracic

Tracheal procedure, Homograft (aortic or PA) reconstruction, Intrathoracic with carinal reconstruction

Tracheal procedure, Tracheoplasty (Other), NOS

Tracheal procedure, Tracheoplasty (Other), Cervical

Tracheal procedure, Tracheoplasty (Other), Intrathoracic

Tracheal procedure, Tracheoplasty (Other), Intrathoracic with carinal reconstruction

Tracheal procedure, Balloon dilatation, NOS

Tracheal procedure, Balloon dilatation, Trachea

Tracheal procedure, Balloon dilatation, Bronchus - left

Tracheal procedure, Balloon dilatation, Bronchus - right

Tracheal procedure, Stent placement, NOS

Tracheal procedure, Stent placement, Trachea

Tracheal procedure, Stent placement, Bronchus - left

Tracheal procedure, Stent placement, Bronchus - right

Tracheal procedure, Stent dilation, NOS

Tracheal procedure, Stent dilation, Trachea

Tracheal procedure, Stent dilation, Bronchus - left

Tracheal procedure, Stent dilation, Bronchus - right

Tracheal stenosis treatment hierarchy level 4

Tracheal procedure, NOS

Tracheal procedure, Resection with end-to-end anastomosis, NOS

Tracheal procedure, Resection with end-to-end anastomosis, Cervical

Tracheal procedure, Resection with end-to-end anastomosis, Intrathoracic

Tracheal procedure, Resection with end-to-end anastomosis, Intrathoracic with carinal reconstruction

Tracheal procedure, Pericardial tracheoplasty, NOS

Tracheal procedure, Pericardial tracheoplasty, Cervical

Tracheal procedure, Pericardial tracheoplasty, Intrathoracic

Tracheal procedure, Pericardial tracheoplasty, Intrathoracic with carinal reconstruction

Tracheal procedure, Rib cartilage tracheoplasty, NOS

Tracheal procedure, Rib cartilage tracheoplasty, Cervical

Tracheal procedure, Rib cartilage tracheoplasty, Intrathoracic

Tracheal procedure, Rib cartilage tracheoplasty, Intrathoracic with carinal reconstruction

Tracheal procedure, Slide tracheoplasty, NOS

Tracheal procedure, Slide tracheoplasty, Cervical

Tracheal procedure, Slide tracheoplasty, Intrathoracic

Tracheal procedure, Slide tracheoplasty, Intrathoracic with carinal reconstruction

Tracheal procedure, Tracheal autograft reconstruction, NOS

Tracheal procedure, Tracheal autograft reconstruction, Cervical

Tracheal procedure, Tracheal autograft reconstruction, Intrathoracic

Tracheal procedure, Tracheal autograft reconstruction, Intrathoracic with carinal reconstruction

Tracheal procedure, Tracheal homograft reconstruction, NOS

Tracheal procedure, Tracheal homograft reconstruction, Cervical

Tracheal procedure, Tracheal homograft reconstruction, Intrathoracic

Tracheal procedure, Tracheal homograft reconstruction, Intrathoracic with carinal reconstruction

Tracheal procedure, Homograft (aortic or PA) reconstruction, NOS

Tracheal procedure, Homograft (aortic or PA) reconstruction, Cervical

Tracheal procedure, Homograft (aortic or PA) reconstruction, Intrathoracic

Tracheal procedure, Homograft (aortic or PA) reconstruction, Intrathoracic with carinal reconstruction

Tracheal procedure, Tracheoplasty (Other), NOS

Tracheal procedure, Tracheoplasty (Other), Cervical

Tracheal procedure, Tracheoplasty (Other), Intrathoracic

Tracheal procedure, Tracheoplasty (Other), Intrathoracic with carinal reconstruction

Tracheal procedure, Balloon dilatation, NOS

Tracheal procedure, Balloon dilatation, Trachea

Tracheal procedure, Balloon dilatation, Bronchus - left

Tracheal procedure, Balloon dilatation, Bronchus - right

Tracheal procedure, Stent placement, NOS

Tracheal procedure, Stent placement, Trachea, NOS

Tracheal procedure, Stent placement, Trachea, Expandable metal stent

Tracheal procedure, Stent placement, Trachea, Expandable metal stent-Palmaz

Tracheal procedure, Stent placement, Trachea, Expandable metal stent-Palmaz-Endoscopically

Tracheal procedure, Stent placement, Trachea, Expandable metal stent-Palmaz-Under fluoroscopy

Tracheal procedure, Stent placement, Trachea, Silicone

Tracheal procedure, Stent placement,Trachea, Other

Tracheal procedure, Stent placement, Bronchus-left, NOS

Tracheal procedure, Stent placement, Bronchus-left, Expandable metal stent

Tracheal procedure, Stent placement, Bronchus-left, Expandable metal stent-Palmaz

Tracheal procedure, Stent placement, Bronchus-left, Expandable metal stent-Palmaz-Endoscopically

Tracheal procedure, Stent placement, Bronchus-left, Expandable metal stent-Palmaz-Under fluoroscopy

Tracheal procedure, Stent placement, Bronchus-left, Silicone

Tracheal procedure, Stent placement, Bronchus-left, Other

Tracheal procedure, Stent placement, Bronchus-right, NOS

Tracheal procedure, Stent placement, Bronchus-right, Expandable metal stent

Tracheal procedure, Stent placement, Bronchus-right, Expandable metal stent-Palmaz

Tracheal procedure, Stent placement, Bronchus-right, Expandable metal stent-Palmaz-Endoscopically

Tracheal procedure, Stent placement, Bronchus-right, Expandable metal stent-Palmaz-Under fluoroscopy

Tracheal procedure, Stent placement, Bronchus-right, Silicone

Tracheal procedure, Stent placement, Bronchus-right, Other

Tracheal procedure, Stent dilation, NOS

Tracheal procedure, Stent dilation, Trachea

Tracheal procedure, Stent dilation, Bronchus - left

Tracheal procedure, Stent dilation, Bronchus - right

Tracheal procedure modifiers

With tracheostomy

With cardiopulmonary bypass

Without cardiopulmonary bypass

VI. Potential diagnostic related risk factors for tracheal stenosis

Granulation tissue

Residual/recurrent tracheal stenosis

Tracheo/bronchomalacia

Air leak pneumomediastinum

Mediastinitis

Pulmonary hypertension

Respiratory failure requiring prolonged ventilation

VII. Analysis pectus excavatum

Pectus excavatum is a relatively commonly occurring chestwall deformity in children. It is a depression of the sternum that commonly starts at the angle of Louis, and is deepest at the xiphisternal junction. Pectus carinatum is a protrusion of the sternum, and is approximately one-tenth as common as pectus excavatum. A severe pectus excavatum deformity can cause cardiopulmonary insufficiency from the compression of the right atrium and right ventricle and diminished vital capacity of the lungs. This analysis is based on our surgical experience over 26 years with children undergoing repair of pectus deformities [35]. The classic surgical repair of pectus excavatum is the Ravitch repair [36]. This entails subperichondrial resection of the deformed cartilages and a sternal osteotomy. Retrosternal support with a metal bar is often used with this approach [37, 38]. Similar techniques are used to repair pectus carinatum [39]. Recently a noninvasive approach, using a convex steel bar placed beneath the pectus deformity and turned to correct the defect, has been reported by Nuss [40].

Pectus hierarchy level 1

Pectus excavatum

Pectus carinatum

Pectus hierarchy level 2

Pectus excavatum, NOS

Pectus excavatum, Mild (< 2 cm in depth)

Pectus excavatum, Moderate (2–3 cm in depth)

Pectus excavatum, Severe (> 3 cm in depth)

Pectus carinatum, NOS

Pectus carinatum, Mild

Pectus carinatum, Moderate

Pectus carinatum, Severe

Pectus modifiers

Associated cardiac anomaly

Associated scoliosis

Marfan’s syndrome

Right side dominant

Left side dominant

Unwillingness to expose chest (psychological factor)

Easy fatigability

Decreased stamina

VIII. Pectus treatment options

Pectus treatment hierarchy level 1

Pectus excavatum repair

Pectus carinatum repair

Pectus treatment hierarchy level 2

Pectus excavatum repair, NOS

Pectus excavatum repair, Ravitch repair, with metal strut

Pectus excavatum repair, Ravitch repair, without metal strut

Pectus excavatum repair, Sternal turnover

Pectus excavatum repair, Costoplasty (unilateral)

Pectus excavatum repair, Nuss minimally invasive technique

Pectus excavatum repair, Silicone implant reconstruction

Pectus carinatum repair, NOS

Pectus carinatum repair, Ravitch repair, with metal strut

Pectus carinatum repair, Ravitch repair, without metal strut

Pectus repair modifiers

Vertical incision

Horizontal incision

Simultaneous intracardiac procedure

Delayed intracardiac procedure

IX. Potential diagnostic related risk factors for pectus

Mediastinitis

Bar migration

Cardiac injury

Pleural effusion

Subcutaneous fluid accumulation

Pectus recurrence

Unstable sternum

Pectus reoperation

X. Diagnosis and procedure short list

Diagnosis Short List

Vascular ring

PA sling

Tracheal stenosis

Tracheal disease, Other

Pectus

Procedure Short List

Vascular ring repair

PA sling repair

Tracheal procedure

Pectus repair

XI. Outcome reports

Vascular rings

Incidence of the types of vascular rings by year, by age at presentation and symptoms.

Types of operations that are performed for each subset of vascular ring by year which include double aortic arch, right arch/left ligamentum, innominate compression, PA sling, and other categories.

Types of double aortic arch by year. Operative approach and type of operation by year. Mortality and complication rate by year. Length of hospital stay by year. Long-term outcome defined as the length of time that the patient requires to become asymptomatic.

Operative approach and type of operation by year for right aortic arch/left ligamentum. Mortality and complication rate by year. Length of hospital stay by year. Long-term outcome defined as the length of time that the patient requires to become asymptomatic.

Operative approach and type of operation by year for innominate compression syndrome. Mortality and complication rate by year. Length of hospital stay by year. Long-term outcome defined as the length of time that the patient requires to become asymptomatic.

Operative approach and type of operation by year for PA sling. Mortality and complication rate by year. Length of hospital stay by year. Long-term outcome defined as the length of time that the patient requires to become asymptomatic. Long-term patency rate of the implanted PA by angiography and by differential nuclear medicine perfusion techniques.

Incidence of rare vascular rings by year, by types of operations, by complication rate, by mortality rate, and by outcome based on time required to become asymptomatic.

Incidence of associated tracheal rings with PA sling by year.

Incidence of associated PA sling with tracheal rings by year.

Tracheal stenosis

Incidence of tracheal stenosis by year and by categories of complete tracheal rings, post intubation, and other forms.

Types of operations for complete tracheal rings, post intubation, and other by year.

Incidence of tracheal stenosis and complete tracheal rings categorized by localized tracheal stenosis and long segment tracheal stenosis.

Incidence of tracheal stenosis and post intubation tracheal stenosis categorized by localized tracheal stenosis and long segment tracheal stenosis.

Tracheal procedures which include resection with end-to-end anastomosis, pericardial tracheoplasty, cartilage tracheoplasty, slide tracheoplasty, tracheal autograft, tracheal homograft, homograft (aortic or PA), other tracheoplasty, balloon dilatation, Palmaz stent placement, silicone stent placement, or other stent placement by year.

Tracheal procedures which include resection with end-to-end anastomosis, pericardial tracheoplasty, cartilage tracheoplasty, slide tracheoplasty, tracheal autograft, tracheal homograft, homograft (aortic or PA), other tracheoplasty, balloon dilatation, Palmaz stent placement, silicone stent placement, or other stent placement by complication rate and mortality.

Tracheal procedures which include resection with end-to-end anastomosis, pericardial tracheoplasty, cartilage tracheoplasty, slide tracheoplasty, tracheal autograft, tracheal homograft, homograft (aortic or PA), other tracheoplasty, balloon dilatation, Palmaz stent placement, silicone stent placement, or other stent placement by length of stay and by number of postoperative bronchoscopies.

Tracheal procedures which include resection with end-to-end anastomosis, pericardial tracheoplasty, cartilage tracheoplasty, slide tracheoplasty, tracheal autograft, tracheal homograft, homograft (aortic or PA), other tracheoplasty, balloon dilatation, Palmaz stent placement, silicone stent placement, or other stent placement by number of reoperations.

Incidence of cardiac anomalies associated with congenital complete tracheal rings which includes PA sling, tetralogy of Fallot, atrioventricular canal, double outlet right ventricle, ventricular septal defect, atrial septal defect by year.

Incidence of cardiac anomalies associated with congenital complete tracheal rings which includes PA sling, tetralogy of Fallot, atrioventricular canal, double outlet right ventricle, ventricular septal defect, atrial septal defect by mortality, by complication rate, and by length of stay compared to patients with complete tracheal rings without associated cardiac anomalies.

Pectus excavatum

Incidence of pectus excavatum and pectus carinatum by year.

Age at presentation and types of symptoms by type of pectus.

Incidence of pectus excavatum by mild forms, moderate forms, and severe forms by year.

Incidence of pectus carinatum by mild forms, moderate forms, and severe forms by year.

Incidence of associated anomalies with pectus excavatum which include associated cardiac anomalies, associated scoliosis and Marfan’s syndrome.

Incidence of associated anomalies with pectus carinatum which include associated cardiac anomalies, associated scoliosis and Marfan’s syndrome.

Types of repair for pectus excavatum which include Ravitch repair with metal strut, Ravitch repair without metal strut, sternal turnover, unilateral costoplasty, (Nuss) minimally invasive technique, silicone implant reconstruction by year.

Types of repair for pectus excavatum which include Ravitch repair with metal strut, Ravitch repair without metal strut, sternal turnover, unilateral costoplasty, (Nuss) minimally invasive technique, silicone implant reconstruction relating to length of stay, complication rate, mortality, objective reports of patient satisfaction, long-term outcome based on objective measurements.

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