Pierre Robin Sequence Flashcards
A 1-day-old newborn is noted to have difficulty breathing during feeding. On examination, there is retrognathia, a cleft palate, and significant airway obstruction due to posterior tongue displacement. Which of the following is the most appropriate next step in management?
A) Perform echocardiography
B) Genetic testing for CHD7 mutations
C) Prone positioning and feeding support
D) Immediate tracheostomy
E) Nasogastric tube placement
Answer: Prone positioning and feeding support
Explanation: In Pierre Robin sequence, prone positioning helps alleviate airway obstruction caused by the posteriorly displaced tongue. Feeding support is also essential to ensure adequate nutrition.
Incorrect Answers:
A) Echocardiography is not immediately necessary unless congenital heart disease is suspected.
B) CHD7 mutations are associated with CHARGE syndrome, not Pierre Robin sequence.
D) Tracheostomy is a last resort for severe airway obstruction unresponsive to conservative measures.
E) Nasogastric feeding may be used but does not address airway obstruction.
What is Pierre Robin Sequence?
A congenital condition characterized by a triad of micrognathia, glossoptosis, and cleft palate.
What is the prevalence of Pierre Robin Sequence in live births?
Approximately 1 in 8,500 to 14,000 live births.
True or False: Pierre Robin Sequence is always isolated and not associated with any other conditions.
False.
Which genetic syndromes are commonly associated with Pierre Robin Sequence?
Stickler syndrome and Treacher Collins syndrome.
Fill in the blank: The triad of Pierre Robin Sequence includes micrognathia, glossoptosis, and _______.
cleft palate.
What is the primary cause of airway obstruction in Pierre Robin Sequence?
Glossoptosis.
What is glossoptosis?
A condition where the tongue is positioned further back in the throat than normal.
This backward displacement of the tounge can cause airway obstruction.
Multiple choice: Which of the following is NOT a component of the Pierre Robin Sequence?
A) Cleft Palate
B) Macroglossia
C) Micrognathia
D) Glossoptosis
B) Macroglossia.
What is micrognathia?
A condition where the jaw is smaller than normal.
True or False: Pierre Robin Sequence can lead to feeding difficulties in infants.
True.
What is the typical management for airway obstruction in infants with Pierre Robin Sequence?
Positioning, use of a nasopharyngeal airway, or surgical intervention.
A 2-week-old newborn with retrognathia and a cleft palate is brought to the pediatrician due to feeding difficulties. The baby frequently gags and chokes during feeds. On examination, the tongue is displaced posteriorly, contributing to airway obstruction. What is the most appropriate intervention to address both feeding and airway issues?
A) Nasogastric tube placement and prone positioning
B) Tracheostomy and gastrostomy tube placement
C) Upright positioning with a bottle nipple adjustment
D) Immediate surgical correction of cleft palate
E) Introduction of solid foods to bypass aspiration risk
Answer: A) Nasogastric tube placement and prone positioning
Explanation:
In Pierre Robin sequence, prone positioning helps alleviate airway obstruction caused by the posteriorly displaced tongue. Nasogastric tube feeding ensures proper nutrition and avoids aspiration. Surgical correction of cleft palate is typically delayed until after 12 months.
Incorrect Answers:
B) Tracheostomy is only used for severe, refractory airway obstruction.
C) Upright positioning is insufficient to alleviate significant airway obstruction in this case.
D) Cleft palate repair is not the immediate priority and is performed later.
E) Solid foods are not appropriate for a 2-week-old and do not address airway obstruction.
What is the recommended position for infants with Pierre Robin Sequence to help alleviate airway obstruction?
Prone or lateral position.
Fill in the blank: Pierre Robin Sequence is diagnosed through clinical evaluation and _______.
physical examination.
