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Name: Moh¶d Mujaheed Alshammary.
Age: 3 1/2 months.
Nationality: Saudi.
D.O.A: 24/3/2005 ( 14/2/1426).
 v

(matient 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.
(matient 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 m CU.
(There was no history of fever at that time. matient
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.
  
(Normal for age.

!  
(Similac formula and feedind well.

‰  

(Upto date.
 ü   
 
(He is the youngest of 5 siblings one sister (8yrs) has
brain atrophy with developmental delay since birth
(No consanguinity
(No hx of atopy
(No hx of other medical illness
(Father and mother are school servents
(Living in Arar with poor income

    
(Looks well, conscious, alert, well hydrated, well
nourished, pale, not jaundiced or cyanosed and not
dysmorphic.

(mt is connected to the ventilator S MV , ETT 4 mm.

(
T: 37.2C HR: 120/min Bm: 102/69

mmHg.sat.100%
(rowth parameters appropriate for age
wt: 5.4 kg = 25th percentile,
length: 60.5 cm = 50th percentile,
H/C: 41 cm = 50th percentile
(r
 High arched palate, normal lips, ears clear

(|
 No chest asymmetry, no scars, good expansion, equal adequate

air entry with transmitted sounds

(|
 Regular pulse with good volume, all peripheral pulses felt,
S1 + S2 + 0

(á
 Soft, lax no palpable organs, +ve B.S, normal male genitalia,

patent anus.

(|
 Sedated with Medazolam and Fentanyl + Chloralhydrate mRN.

(Other systems: NAD.

  
3 ½ month old Saudi boy admitted in m CU 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.
 

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

CBC
U/E Normal
LFT

Blood and Urine C/S was negative.

All peripheral C/S showed no growth.
 v 






 

=
  
mt was admitted to m CU and kept under close monitoring on
mechanical ventilation. 1st CB showed mh 7.43, mCO2 33.6,
mO2 67.7 and HCO3 22. CB was done 2 hourly then OD.
Kept NmO.
VF started.
Dexamethasone started 2mg V Q6 hourly.
Ceftazidime V.
Ampicillin V.
ï  

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 NT
feeding.
CXR: Hyperinflatted chest otherwise
unremarkable.
CT chest (with contrast): shows ? vascular
abnormality.
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Upper  study with Fluroscopy:
Oesophageal filling defect.
( 

 

 
!  |"#!|"$ 
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 Surgical management:
The patient was operated by thoracoscopic surgical
procedure. The right nondominant aortic arch was ligated
and divided. matient 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.

 




 `
  

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.
 v
 
  


( The 1st vascular ring described was a double AA

noted by Hommel 1737.
( Bayford reported retroesophageal RSCA in 1794 .
( n 1945 ross used the term vascular ring in the
new england journal of medicine .

( ts uncommon anomalies ~1% of all congenital

cardiac defects , with equal frequency in both
sexes , no geographical or racial predominance
exists.
 r


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 .
 |


( Double Aortic Arch

( Right Arch with left ligamentum

( Left Arch with arch vessel anomalies

( mA sling
 |

 

= |  
‰  


A Double Aortic Arch A nnominate artery

compression
A Right arch/Left A mulmonary artery sling
ligamentum A Left arch/aberrant R
subclavian artery
 | 
( Double Aortic Arch
A Right dominant (70%)
A Left dominant (20%)
A balanced (10%)
 |  

( right arch dominant

(70%)
 |  

â Left arch dominant

(20%)
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 m 

Large vessels apply local pressure

« Compress the trachea and bronchial tree

« Compress esophagus
« Compress BV
 `
 



Associated congenital
heart disease may be
present in 5
50% of
patients, depending on the
vascular anomaly.
( - Right arch with left ligament

( mersistant of right arch with involution

of the LL between the LCCA & LSCA
( Retroesophageal LSCA (65 % ) , or
mirror image branching ( 35 % )
D

 




      
 
j      


      
 
 
  
 |

 

= |  
‰  


A Double Aortic Arch A nnominate artery

compression
A Right arch/Left A mulmonary artery sling
ligamentum A Left arch/aberrant R
subclavian artery
( B- incomplete ring :

- 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 arch with aberrant innominate .

( leftward and post. Displacement of the

innominate on the arch .
( ant. Compression of the trachea
( - mA sling .

( Lt lung captures the arterial supply from the

Rt 6th arch instead of the Lt 6th arch
( origin of the LmA off the RmA

( courses between the trachea and esophagus

causing ant. Compression of the esophagus
( associated with tracheobronchial malacia and
complete tracheal rings
( associated with intracardiac defects
m   
  
 

( mresent in first 6 months
( Respiratory distress,
( Stridor,
( Cough,
( Dysphagia
( Early presentation in double arch
( Vomiting is frequent.
( mneumonia is common.
  
 


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

ith innominate artery compression 1/2
have apneic spells
  
 

 




argling sounds

Low pitched inspiratory and expiratory

nspiratory stridor

Expiratory wheezes

Sounds present since birth
Established over first month of life
marent may not be aware of sounds
  
 

 

( mrovocative maneuvers that increase dyspnea
» Neck flexion

( malliative maneuvers that decrease dyspnea

» Neck extension
 


  

TOF 20 % .

VSD

Dieorge

VACTERL, CHARE .

Esophageal atresia .
 ` 



|
- 

Note aortic arch location (right or left)

Note tracheal caliber

o 
% %

Note indentation
 ` 




o 
 
( Especially needed with mA sling
( To determine the extent of airway
narrowing.
(|)
!‰ |"
( Very good at defining anatomy
 D  

( A- eneral

- All symptomatic pt. Surgical Correction

-Asymptomatic
- complete ring
Elective repair
- incomplete ring
follow clinicaly
( B- Double arch

- left posteriolatral thoracotomy

- divide the smaller of the two arches usually
between the CCA and SCA
- ligate the duct and fibrous tissue careful of
the recurrent laryngeal nerve
(C -Rt arch with left ligamentum

- left posteriolatral thoracotomy

- divide the ligament
(D- Lt arch with aberrant RSCA

- most are truly asymptomatic

- symptoms
-ligate RSCA in small children
- reimplant in most others
(E- Arch with aberrant innominate

- suspend the arch to the post. Sternum

- reimplant the innominate to the right and
ant.on the arch
(F - mA sling

- median sternotomy
- address tracheomalacia the primary repair
or pericardial patch
- reimplant LmA on to the main mA
 


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 mA sling

D