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pierre robin

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by

Jenny Smith

on 12 September 2013

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Transcript of pierre robin

Pierre Robin Sequence
What is it?
- Micrognathia
- Glossoptosis
- Cleft palate
Other features
Facial dysmorphism
Short neck
Hyperextensible finger joints
Hypoplastic fingernails and toenails
Ocular anomalies (Stickler syndrome)
Cardiac defects (Velocardiofacial syndrome)

Epidemiology
Classic triad...
Incidence 1:8500 live births
Male : female ratio > 1:1
No ethnic differences reported
Etiology
"
Sequence
" = pattern of multiple anomalies due to prior developmental anomalies or mechanical processes. Not specific to any particular disease/diagnosis

Deformational
: ~40%
Intrauterine constraint
Chromosomal
:
Deletion (22q) syndrome ~8%
Velocardiofacial syndrome
4q, 6q, 11q chromosomal abnormality
Monogenetic
: ~15-30%
Stickler syndrome
Teratogenic
:
Fetal alcohol syndrome
Neuromuscular disorders
Complications
Respiratory
Can present at birth with obstruction
Sleep apnea common problem

GI
Feeding difficulties (commonest)
Reflux
Failure to thrive

Neuro

Cerebral impairment (chronic hypoxia)

ENT
Ear infections
Hearing loss
Management
Conservative
:
Positioning - prone or side-lying
Feeding - Haberman nipple; positioning for feeds
Multidisciplinary management

Medical
:
Reflux management - ranitidine, omeprazole, domperidone
Airway management - NPT, ETT

Surgical
:
Repair of cleft palate at 9-18months
FTT / feeding difficulties: G-tube insertion
Prognosis
Study of 125 PRS patients, 1994
Group 1
:
Adequate respiration in prone position
Bottle-feeding
>>Mortality 1.8%
Group 2
:
Adequate respiration in prone position
Requiring tube feeds
>>Mortality 10%
Group 3
:
Resp distress + intubation
Tube feeds
>>Mortality 41%
Full transcript