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. 2012 May;26(2):76-82.
doi: 10.1055/s-0032-1320065.

Pierre robin sequence

Noopur Gangopadhyay  1 , Derick A Mendonca, Albert S Woo
Affiliations

Pierre robin sequence

Noopur Gangopadhyay et al. Semin Plast Surg. 2012 May.

Abstract

Pierre Robin sequence (PRS) is classically described as a triad of micrognathia, glossoptosis, and airway obstruction. Infants frequently present at birth with a hypoplastic mandible and difficulty breathing. The smaller mandible displaces the tongue posteriorly, resulting in obstruction of the airway. Typically, a wide U-shaped cleft palate is also associated with this phenomenon. PRS is not a syndrome in itself, but rather a sequence of disorders, with one abnormality resulting in the next. However, it is related to several other craniofacial anomalies and may appear in conjunction with a syndromic diagnosis, such as velocardiofacial and Stickler syndromes. Infants with PRS should be evaluated by a multidisciplinary team to assess the anatomic findings, delineate the source of airway obstruction, and address airway and feeding issues. Positioning will resolve the airway obstruction in ~70% of cases. In the correct position, most children will also be able to feed normally. If the infant continues to show evidence of desaturation, then placement of a nasopharyngeal tube is indicated. Early feeding via a nasogastric tube may also reduce the amount of energy needed and allow for early weight gain. A proportion of PRS infants do not respond to conservative measures and will require further intervention. Prior to considering any surgical procedure, the clinician should first rule out any sources of obstruction below the base of the tongue that would necessitate a tracheostomy. The two most common procedures for treatment, tongue-lip adhesion and distraction osteogenesis of the mandible, are discussed.

Keywords: Pierre Robin sequence; airway obstruction; distraction osteogenesis; glossoptosis; micrognathia.

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Figures

Figure 1
Figure 1
Microretrognathia in an infant with Pierre Robin sequence, best seen on lateral view.
Figure 2
Figure 2
Nasopharyngeal airway used in treatment of Pierre Robin sequence.
Figure 3
Figure 3
A schematic of tongue–lip adhesion illustrating overlapping mucosal-based flaps and the posterior tongue/inferior chin buttons. (Reprinted with permission from Bartlett SP, Losee JE, Baker SB. Reconstruction: craniofacial syndromes. In: Mathes SJ, ed. Plastic Surgery. Vol. 4. Philadelphia, PA: Saunders Elsevier, 2006: 516.)
Figure 4
Figure 4
Postoperative view of patient after tongue–lip adhesion for airway obstruction. (Courtesy of Dr. Alex Kane.)
Figure 5
Figure 5
A 270-degree osteotomy for distraction osteogenesis. Note that the anterior cortex has been osteotomized. A straight osteotome is placed in the bone and canted to complete the fracture along the lingual cortex. The inferior alveolar nerve can be partially visualized in the midportion of the mandible.
Figure 6
Figure 6
Internal univector distractor placed following osteotomy. The distraction arm exits the skin via an incision inferior to the ear lobule.
Figure 7
Figure 7
External distraction multivector distractor.
See this image and copyright information in PMC

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