Case Presentation

Vascular Ring

Dr. Ashis Kumar Das

Name: Moh’d Mujaheed Alshammary.

Age: 3 1/2 months.

Nationality: Saudi.

D.O.A: 24/3/2005 ( 14/2/1426).

HistoryHistory

Patient transfered from our Arar central hospital to higher center,Riyadh as a case of congenital laryngeal obstruction suspecting vascular ring, laryngeal web or laryngomalacia with severe respiratory distress on mechanical ventilation.

He is a full term delivered by C/S due to 2 previous C/S in Arar central hospital with birth weight 3.5 kg without any complication during pregnancy or after delivery.

Discharged on 4th day. Readmitted 5 days later because of stridor but discharged after 2 days as laryngomalasia after reassuring the parents.

There was no problems later on except the stridor, he was feeding well and gaining weight, no history of chocking or fever.

Patient was in normal state of health till the day before admission in Arar, when mother noticed mild cough, poor feeding and irritability seen initially at a clinic and immediately referred to the central hospital, intubated in ER due to severe respiratory distress and admitted to the PICU.There was no history of fever at that time. Patient required high ventilation setting and referred to higher center hospital because of difficulty in ventilation and failure of extubation for further diagnostic workup and management after 9 days.

Developmental HxNormal for age.

 

Nutritional HxSimilac formula and feedind well.

 

Immunization HxUpto date.

Family and social HxHe is the youngest of 5 siblings one sister (8yrs) has brain atrophy with developmental delay since birthNo consanguinityNo hx of atopyNo hx of other medical illnessFather and mother are school serventsLiving in Arar with poor income

Physical ExamintaionLooks well, conscious, alert, well hydrated, well nourished, pale, not jaundiced or cyanosed and not dysmorphic.

Pt is connected to the ventilator SIMV , ETT 4 mm.

V/S T: 37.2C HR: 120/min BP: 102/69 mmHg.sat.100%Growth parameters appropriate for age

wt: 5.4 kg = 25th percentile, length: 60.5 cm = 50th percentile, H/C: 41 cm = 50th percentile

ENT: High arched palate, normal lips, ears clear

Chest: No chest asymmetry, no scars, good expansion, equal adequate air entry with transmitted sounds

CVS: Regular pulse with good volume, all peripheral pulses felt, S1 + S2 + 0

Abd: Soft, lax no palpable organs, +ve B.S, normal male genitalia, patent anus.

CNS: Sedated with Medazolam and Fentanyl + Chloralhydrate PRN.

Other systems: NAD.

Summary

3 ½ month old Saudi boy admitted in PICU ACH and later on transferred to higher center as a case of congenital laryngeal obstruction with Laryngomalacia with suspicion of congenital anomaly of larynx eg Vascular ring. Child was sent to higher center on mechanical ventilation for further evaluation.

 

D.Dx

Congenital stridor Laryngomalacia , tracheomalacia Congenital subglottic stenosis laryngeal stenosis, Laryngael web, vascular ring

 

 

Initial Investigation

 

CBC

U/E Normal

LFT

 

Blood and Urine C/S was negative.

All peripheral C/S showed no growth.

Hospital course and investigations(Riyadh)

1st day

Pt was admitted to PICU and kept under close monitoring on mechanical ventilation. 1st CBG showed Ph 7.43, PCO2 33.6, PO2 67.7 and HCO3 22. CBG was done 2 hourly then OD.

Kept NPO.

IVF started.

Dexamethasone started 2mg IV Q6 hourly.

Ceftazidime IV.

Ampicillin IV.

2nd day

 

Feeding started by 10 cc and increased gradually.

Since admission pt required low ventilatory settings, then extubated after 3days but the pt did not tolerate the extubation and was having severe stridor. He was reintubated within 2 hours, after that the pt became stable and maintained normal blood gas.

There is no fever, hemodynamically stable and tolerating NGT feeding.

 

CXR: Hyperinflatted chest otherwise unremarkable.

CT chest (with contrast): shows ? vascular abnormality.

Upper GI study with Fluroscopy: Oesophageal filling defect.

Echo: possibility of vascular ring.

 Multi Detector CT (MDCT) or spiral CT of the chest: Double aortic arch.

Surgical management:The patient was operated by thoracoscopic surgical procedure. The right nondominant aortic arch was ligated and divided. Patient was stable in peri and post operative period. He was extubated on 4th postoperative day. He tolerated extubation well but was still having biphasic stridor. But he didn’t need oxygen and maintained good blood gases. He was tolerating feeds.

Vascular Rings & Vascular Rings & Sling Sling

DefinitionDefinition

Congenital abnormalities of the aortic

arch and its major branches result in the

formation of vascular rings around the

trachea and esophagus with varying

degrees of compression.

History and frequencyHistory and frequency

The 1st vascular ring described was a double AA noted by Hommel 1737.

Bayford reported retroesophageal RSCA in 1794 . In 1945 Gross used the term vascular ring in the

new england journal of medicine .

Its uncommon anomalies ~1% of all congenital cardiac defects , with equal frequency in both sexes , no geographical or racial predominance exists.

EmbryologyEmbryology– Early in the course of embryonic morphogenesis , 6 pairs

of pharyngeal arch arteries develop in conjunction with the branchial pouches .

Normaly :– 1- The left fourth arch becomes the aortic arch – 2- The right fourth arch contribute to the innominate

artery – 3- The distal left sixth arch becomes the ductus arteriosus– 4-The proximal sixth arches bilatraly contribute to the

proximal branch pulmonary arteries – 5- The left dorsal aorta becomes the descending thoracic

aorta– 6-The dorsal intersegmental arteries bilateraly become the

subclavian arteries .

– Vascular rings are formed when this process of regression does not occur normally and the resulting vascular anatomy completely encircles the trachea and esophagus .

ClassificationClassification

Double Aortic Arch

Right Arch with left ligamentum

Left Arch with arch vessel anomalies

PA sling

Classification(cont.)Classification(cont.)

1. Complete Rings

* Double Aortic Arch

* Right arch/Left ligamentum

Incomplete Rings

* Innominate artery compression

* Pulmonary artery sling

* Left arch/aberrant R subclavian artery

Complete RingsComplete Rings

Double Aortic Arch

* Right dominant (70%)

* Left dominant (20%)

* balanced (10%)

Complete Rings(cont)Complete Rings(cont)

right arch dominant

(70%)

Complete Rings (cont)Complete Rings (cont)

•Left arch dominant (20%)

Morphology of Double Aortic ArchMorphology of Double Aortic Arch

1. The left arch is often narrower than the right.

2. Uncommonly right arch may be smaller in its distal part, but it is rarely atretic.

3. Associated cardiovascular anomalies are not common, but include TOF & TGA.

4. Vascular ring is present.

PathophysiologyPathophysiology

Large vessels apply local pressure

« Compress the trachea and bronchial tree « Compress esophagus« Compress BV

Double Aortic ArchDouble Aortic Arch

Associated congenital heart disease may be present in 5–50% of patients, depending on the vascular anomaly.

II- Right arch with left ligament

Persistant of right arch with involution of the LL between the LCCA & LSCA

Retroesophageal LSCA (65 % ) , or mirror image branching ( 35 % )

Types of Right Aortic ArchTypes of Right Aortic Arch

Rt. Aortic Arch with Lt. Ligamentum Arteriosum Rt. Aortic Arch with Lt. Ligamentum Arteriosum & Mirror Image Lt. Innominate Artery& Mirror Image Lt. Innominate Artery

Rt. Aortic Arch with Lt. Ligamentum Arteriosum Rt. Aortic Arch with Lt. Ligamentum Arteriosum & Retroesophageal LSCA& Retroesophageal LSCA

Classification(cont.)Classification(cont.)

1. Complete Rings

* Double Aortic Arch

* Right arch/Left ligamentum

Incomplete Rings

* Innominate artery compression

* Pulmonary artery sling

* Left arch/aberrant R subclavian artery

B- incomplete ring :

I- Lt arch with aberrant RSCA .

– secondary to regression of the R arch between the RCCA & RSCA

– aberrant RSCA deforms the esophagus post.

– most common arch anomaly ( 0.5 %)

Lt. Aortic Arch with Aberrant RSCA Arising Lt. Aortic Arch with Aberrant RSCA Arising from Descending Aortafrom Descending Aorta

II- Lt arch with aberrant innominate .

leftward and post. Displacement of the innominate on the arch .

ant. Compression of the trachea

III- PA sling .

Lt lung captures the arterial supply from the Rt 6th arch instead of the Lt 6th arch

origin of the LPA off the RPA courses between the trachea and esophagus

causing ant. Compression of the esophagus associated with tracheobronchial malacia

and complete tracheal ringsassociated with intracardiac defects

Pulmonary Artery SlingPulmonary Artery Sling

Clinical SymptomsClinical Symptoms Present in first 6 months Respiratory distress, Stridor, Cough, Dysphagia Early presentation in double arch Vomiting is frequent. Pneumonia is common.

Clinical Symptoms(cont)Clinical Symptoms(cont)

Dysphagia less common and presents as infant progresses from liquids to solids

– With innominate artery compression 1/2 have apneic spells

Clinical manifestation(cont..)Clinical manifestation(cont..)

Signs – Gargling sounds – Low pitched inspiratory and expiratory

– Inspiratory stridor – Expiratory wheezes– Sounds present since birth

Established over first month of life

Parent may not be aware of sounds

Clinical manifestation(cont..)Clinical manifestation(cont..)

Provocative maneuvers that increase dyspnea» Neck flexion

Palliative maneuvers that decrease dyspnea » Neck extension

Associated anomaliesAssociated anomalies

– TOF 20 % . – VSD – DiGeorge – VACTERL, CHARGE .– Esophageal atresia .

Diagnostic StudiesDiagnostic Studies

Chest x-ray – Note aortic arch location (right or left)

– Note tracheal caliber

Barium swallow – Note indentation

Diagnostic Studies(cont)Diagnostic Studies(cont)

BronchoscopyEspecially needed with PA sling To determine the extent of airway

narrowing. ECHO MRI/CTVery good at defining anatomy

TreatmentTreatment

A- General

I- All symptomatic pt. Surgical Correction

II-Asymptomatic

- complete ring –Elective repair

- incomplete ring – follow clinicaly

B- Double arch

I - left posteriolatral thoracotomy

II - divide the smaller of the two arches usually between the CCA and SCA

III - ligate the duct and fibrous tissue careful of the recurrent laryngeal nerve

C -Rt arch with left ligamentum

I - left posteriolatral thoracotomy

II - divide the ligament

D - Lt arch with aberrant RSCA

I - most are truly asymptomatic

II - symptoms

-ligate RSCA in small children

- reimplant in most others

E - Arch with aberrant innominate

I - suspend the arch to the post. Sternum

II - reimplant the innominate to the right and ant.on the arch

F - PA sling

I - median sternotomy

II - address tracheomalacia the primary repair or pericardial patch

III- reimplant LPA on to the main PA

ResultsResults

– Up to ½ have abnormal flow on formal pulmonary testing at one year despite been asymptomatic

– Noisy breathing may take awhile to resolve – successful 95% – Minimal morbidity except with PA sling

THANK YOUTHANK YOU