Pierre Robin Syndrome Case in Saudi Arabia: Case Report
Saja Alhaidar1*, Jawaher Alharbi2, Albandari Aldaws3, Najd Alsubaie4 and Rana Alasmari5
1,2Consultant Pediatric Dentist, King Saud Medical City
3,4,5General Dentist Riyadh, Saudi Arabia
*Corresponding author: Saja Alhaidar, Consultant Pediatric Dentist, King Saud Medical City
Citation: Alhaidar S , Aldaws A , Alsubaie N, Alasmari R. Pierre Robin Syndrome Case in Saudi Arabia: Case Report. J Oral Med and Dent Res. 6(1):1-9.
Received:December 23, 2024 | Published: January 24, 2025
Copyright© 2025 genesis pub by Alhaidar S, et al. CC BY-NC-ND 4.0 DEED. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives 4.0 International License. This allows others distribute, remix, tweak, and build upon the work, even commercially, as long as they credit the authors for the original creation.
DOI: https://doi.org/10.52793/JOMDR.2025.6(1)-90
Abstract
Pierre Robin Syndrome (PRS) is a congenital condition characterized by micrognathia, glossoptosis, airway obstruction, and often a U-shaped cleft palate. This case report details the management of a 5-year-old Saudi boy diagnosed with PRS, presenting with multiple dental issues, malocclusion, impacted teeth, and feeding difficulties. There was no known family history of syndromes. Over the years, the patient underwent multiple surgical and orthodontic interventions, including mandibular distraction, cleft palate repair, and dental rehabilitation. The report highlights the complexity of treating PRS, with challenges such as airway management, delayed dental development, and significant malocclusion, requiring close collaboration between dental, orthodontic, and surgical teams. Long-term multidisciplinary follow-up, orthodontic treatment, and planned orthognathic surgery are essential for improving both functional and aesthetic outcomes. Early intervention, coupled with continuous dental supervision, plays a crucial role in managing the dental and craniofacial challenges associated with PRS.
Keywords
Congenital defects; Feeding problems; Micrognathia; Pediatrics; Pierre Robin syndrome; Pneumonia; Softpalate
Introduction
Pierre Robin Syndrome (PRS) is a congenital condition characterized by micrognathia or retrognathia, feeding difficulties, respiratory issues, cleft palate, and glossoptosis [1,2]. Although the exact prevalence of PRS is unknown, it is estimated to affect between 1 in 8,500 and 1 in 20,000 newborns [2]. There are theories suggesting that PRS may result from abnormal embryonic development in the caudal hindbrain [1]. PRS is a clinically well-defined subgroup of individuals with cleft lip and palate, though its precise cause remains unclear. It is often associated with other Mendelian syndromes, such as Marshall’s syndrome, velocardiofacial syndrome, and Stickler’s syndrome [3].
Recent genetic studies have indicated that PRS may be linked to the dysregulation of the genes SOX9 and KCNJ2, as evidenced by familial translocations with a breakpoint in the intergenic region between SOX9 and KCNJ2, as well as decreased expression of these genes in non-translocated PRS patients (4). Historically, airway obstruction in PRS has been reported to occur shortly after birth [5]. However, Ogborn and Pemberton refuted this, stating that airway obstruction in 5 of their 16 cases (30%) became apparent between the ages of 2 and 21 days [6]. Bull et al. noted that upper airway obstruction could manifest within the first month of life, though they provided no precise clinical details [7]. Later-occurring upper airway obstruction can have severe consequences, particularly after the initial hospital discharge. This case study explores such complications.
To date, no published studies from Saudi Arabia have examined the incidence or etiology of craniofacial anomalies. Among various facial syndromes, 72 cases (59.0%) were identified as having Pierre Robin sequence, 17 cases (13.9%) had Goldenhar syndrome, and another 17 cases (13.9%) had Van der Woude syndrome [8]. Given the rarity of published studies from Saudi Arabia, this case report presents the case of a 5-year-old Saudi boy diagnosed with PRS, who presented with multiple dental issues, malocclusion, impacted teeth, and feeding difficulties. There was no known family history of syndromes.
Case Report
A 5-year-old Saudi boy diagnosed with Pierre Robin Sequence presented with a chief complaint of restricted tongue movement, affecting speech, and significant dental decay. The patient’s history included palatoplasty, ear surgery, and the use of Continuous Positive Airway Pressure (CPAP) at a sleep clinic for respiratory support. There was no known family history of syndromes, allergies, or ongoing medication use. Since the age of 2, he had undergone multiple procedures, including cleft palate repair, tongue-tie release, and mandibular distraction, with the distraction device removed in January 2015. His mother noted a habit of finger-sucking during sleep.
Initial dental evaluation and management
During his first dental evaluation, restricted tongue movement and multiple carious teeth were observed, including stainless steel crowns (SSCs) from previous dental treatments. Further assessment by the Pediatric Department at King Saud Medical City revealed an open bite, dental crowding, caries in teeth #16 and #26, and poor oral hygiene. Radiographs, including periapical (PA) and orthopantomogram (OPG) views, were taken to assess the dental condition (Figure 1).
Figure 1: Periapical radiograph of tooth #36 showing its condition prior to treatment.
Preventive resin restorations (PRR) were applied to teeth #16 and #26, and the patient was referred to the orthodontics and oral and maxillofacial surgery (OMFS) departments for further management.
Figure 1.1: Periapical radiograph of teeth #46, and #85 prior treatment.
Figure 1.2: Orthopantomogram (OPG) of the patient at age 5, illustrating dental development and alignment at this stage.
Dental symptoms and evaluation at age 9
At the age of 9, the patient presented with intense pain in tooth #36. The extraoral examination revealed a retrognathic mandible, while the intraoral examination showed signs of plaque-induced gingivitis, occlusal imbalance, significant decay in tooth #36, dental crowding, and the absence of teeth #35, #37, and #38. An orthopantomogram (OPG) was ordered for a more detailed assessment (Figure 2). Tooth #36 was found to be non-restorable and was recommended for extraction due to pain upon percussion. The extraction attempt under local anesthesia (LA) was unsuccessful due to the patient's limited cooperation, prompting a referral for behavioral management and sedation with nitrous oxide.
Figure 2: Orthopantomogram (OPG) of the patient at age 9, illustrating dental development and alignment at this stage.
Orthodontic Observations
During follow-up appointments, orthodontic assessments revealed a convex facial profile, Class II malocclusion with a 7 mm overjet, a 5 mm open bite, a right-sided crossbite, and an impacted tooth #13. The patient also exhibited gingival overgrowth around the lower primary molars and the absence of teeth #35, #37, #38, #47, and #48. The proposed treatment plan included palatal expansion, with subsequent orthodontic treatment to be monitored after the eruption of the premolars.
Evaluation and treatment at age 10
At age 10, the follow-up evaluation showed healed surgical sites, a retruded mandible, fair oral hygiene, and improved sleep-related breathing patterns. A lateral cephalometric X-ray was taken (Figure 3). The patient underwent full-mouth dental rehabilitation under general anesthesia, which included fissure sealants on teeth #26, #34, and #45, preventive resin restorations (PRR) on tooth #16, and extractions of teeth #36 and #46 performed by the oral and maxillofacial surgery (OMFS) team.