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Our Experience With Regional Anesthesia in a Case of Pierre Robin Syndrome.

Onal O, Zora ME, Saltali A, Bozdogan S, Bengi Celik J - Anesth Pain Med (2016)

View Article: PubMed Central - PubMed

Affiliation: Department of Anesthesiology and Reanimation, Selcuk University Medical Faculty, Konya, Turkey.

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Pierre-Robin Syndrome (PRS) is characterized by micrognathia, glossoptosis, and a “u-shaped” cleft palate... Neonates with PRS present with respiratory obstruction varying from mild to severe directly after birth or during the first week of life... A sufficiently severe obstruction can result in obstructive apnea, hypoxia, failure to thrive, and death... We report a case of a patient with PRS who could not be intubated previously and who had a history of tracheostomy; this patient was scheduled to undergo an operation for hip dysplasia... An 8-year-old boy weighing 18 kg and diagnosed with PRS underwent preoperative evaluation that revealed restricted mouth opening, micrognathia, short extremities, mild airway obstruction, and respiratory distress (Figure 1)... His Mallampati score was 3, his head-neck extension was comfortable, and his thyromental distance was within the normal range... Preoperative laboratory and imaging tests yielded normal results... Airway obstruction is caused by the presence of glossoptosis, in addition to cleft palate and micrognathia with retrognati... No problems were encountered with mask use in our case, so an I-gel was placed under propofol anesthesia without using any muscle relaxants, and a spinal block was carried out for postoperative analgesia... The intubation and extubation of PRS cases requires special care, and preoperative monitoring is warranted... For operations under the umbilicus, regional anesthesia can be a good alternative in children who are at high risk from general anesthesia... In fact, previous studies have demonstrated that regional anesthesia is a safe alternative in neonates.

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Related in: MedlinePlus

Image of the Patient With Pierre Robin Syndrome
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fig27451: Image of the Patient With Pierre Robin Syndrome

Mentions: Pierre-Robin Syndrome (PRS) is characterized by micrognathia, glossoptosis, and a “u-shaped” cleft palate (1). The incidence of PRS varies from 1: 5000 to 1: 85000 (2). Neonates with PRS present with respiratory obstruction varying from mild to severe directly after birth or during the first week of life. A sufficiently severe obstruction can result in obstructive apnea, hypoxia, failure to thrive, and death (3). We report a case of a patient with PRS who could not be intubated previously and who had a history of tracheostomy; this patient was scheduled to undergo an operation for hip dysplasia. We present our anesthesia management and approach. An 8-year-old boy weighing 18 kg and diagnosed with PRS underwent preoperative evaluation that revealed restricted mouth opening, micrognathia, short extremities, mild airway obstruction, and respiratory distress (Figure 1).


Our Experience With Regional Anesthesia in a Case of Pierre Robin Syndrome.

Onal O, Zora ME, Saltali A, Bozdogan S, Bengi Celik J - Anesth Pain Med (2016)

Image of the Patient With Pierre Robin Syndrome
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4834743&req=5

fig27451: Image of the Patient With Pierre Robin Syndrome
Mentions: Pierre-Robin Syndrome (PRS) is characterized by micrognathia, glossoptosis, and a “u-shaped” cleft palate (1). The incidence of PRS varies from 1: 5000 to 1: 85000 (2). Neonates with PRS present with respiratory obstruction varying from mild to severe directly after birth or during the first week of life. A sufficiently severe obstruction can result in obstructive apnea, hypoxia, failure to thrive, and death (3). We report a case of a patient with PRS who could not be intubated previously and who had a history of tracheostomy; this patient was scheduled to undergo an operation for hip dysplasia. We present our anesthesia management and approach. An 8-year-old boy weighing 18 kg and diagnosed with PRS underwent preoperative evaluation that revealed restricted mouth opening, micrognathia, short extremities, mild airway obstruction, and respiratory distress (Figure 1).

View Article: PubMed Central - PubMed

Affiliation: Department of Anesthesiology and Reanimation, Selcuk University Medical Faculty, Konya, Turkey.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Pierre-Robin Syndrome (PRS) is characterized by micrognathia, glossoptosis, and a “u-shaped” cleft palate... Neonates with PRS present with respiratory obstruction varying from mild to severe directly after birth or during the first week of life... A sufficiently severe obstruction can result in obstructive apnea, hypoxia, failure to thrive, and death... We report a case of a patient with PRS who could not be intubated previously and who had a history of tracheostomy; this patient was scheduled to undergo an operation for hip dysplasia... An 8-year-old boy weighing 18 kg and diagnosed with PRS underwent preoperative evaluation that revealed restricted mouth opening, micrognathia, short extremities, mild airway obstruction, and respiratory distress (Figure 1)... His Mallampati score was 3, his head-neck extension was comfortable, and his thyromental distance was within the normal range... Preoperative laboratory and imaging tests yielded normal results... Airway obstruction is caused by the presence of glossoptosis, in addition to cleft palate and micrognathia with retrognati... No problems were encountered with mask use in our case, so an I-gel was placed under propofol anesthesia without using any muscle relaxants, and a spinal block was carried out for postoperative analgesia... The intubation and extubation of PRS cases requires special care, and preoperative monitoring is warranted... For operations under the umbilicus, regional anesthesia can be a good alternative in children who are at high risk from general anesthesia... In fact, previous studies have demonstrated that regional anesthesia is a safe alternative in neonates.

No MeSH data available.


Related in: MedlinePlus