|Year : 2010 | Volume
| Issue : 4 | Page : 326-330
Pierre robin sequence: Report of two cases
RJ Hegde1, NR Mathrawala2
1 Professor and Head, Department of Pediatric and Preventive Dentistry, Bharati Vidyapeeth Deemed University Dental College and Hospital, Navi Mumbai, India
2 Senior Lecturer, Department of Pediatric and Preventive Dentistry, Bharati Vidyapeeth Deemed University Dental College and Hospital, Navi Mumbai, India
|Date of Web Publication||25-Jan-2011|
R J Hegde
Department of Pediatric and Preventive Dentistry, Bharati Vidyapeeth Deemed University Dental College and Hospital, Sector-7, C.B.D Belpada, Navi Mumbai - 400 614
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Pierre Robin sequence (PRS) or anomalad, a well-recognized presentation, is the association of the first brachial arch malformation. It presents with a classic triad of micrognathia, glossoptosis, and cleft palate. In a neonate with a complete cleft palate, problems with feeding are commonly encountered. Presented here are two cases with PRS in whom palatal obturators were constructed.
Keywords: Cleft palate, feeding plate, micrognathia, palatal feeding obturator, Pierre Robin sequence
|How to cite this article:|
Hegde R J, Mathrawala N R. Pierre robin sequence: Report of two cases. J Indian Soc Pedod Prev Dent 2010;28:326-30
| Introduction|| |
Pierre Robin Sequence is considered to be a nonspecific anomalad which may occur either as an isolated defect or as a broader group of malformations.  It presents with a classic triad of micrognathia, glossoptosis, and cleft palate. In 1923 French physician Pierre Robin introduced the term 'glossoptosis' in association with micrognathia. He later reported an association with cleft palate in 1934 and this constellation of findings was termed as syndrome. In 1976, Cohen renamed it as anomalad which he defined as 'a malformation together with its subsequent-derived structural changes'.  The term Pierre Robin sequence (PRS) is currently used. The primary defect lies in the arrested development of mandible leading to a characteristic bird face appearance. This in turn prevents the normal descent of the tongue between palatal shelves, resulting in cleft palate. The problem can be addressed via the fabrication of a feeding obturator, which is demonstrated to the parents.  Presented here is a report of two cases with PRS for whom a palatal obturator were constructed. This facilitated feeding required for proper growth and development and also to prevent other associated complications.
| Case Reports|| |
The parents of a three-months-old female child reported to the department of Pediatric Dentistry with the chief complaint of difficulty in feeding the child. On extra oral examination, the neonate showed receded chin and a characteristic bird face appearance [Figure 1] and [Figure 2]. Intraoral examination revealed a large palatal cleft with normal appearing gum pads. Medical and family history of the child was noncontributory. The child had been examined by the plastic surgeons and surgical correction of palate was planned at the age of 18 months. Based on these clinical findings, a diagnosis of PRS was made. For the purpose of ease of feeding and to prevent regurgitation or aspiration, it was decided to fabricate a palatal feeding obturator till the successful completion of surgery.
The impression of palate was taken using impression compound. The impression compound was softened and placed on the finger to allow the material to lose excess heat. Then the child was held upright and the finger carrying impression material was placed into the oral cavity. Once sufficient suction was achieved and impression compound hardened, the material was removed from the mouth. A plaster of paris model was obtained from the impression which was then used to prepare the wax pattern [Figure 3] and [Figure 4]. The wax pattern was invested. A heat cured acrylic feeding obturator was fabricated [Figure 5]. All the borders of the feeding obturator were rounded and a small hole was made in the labial portion of the feeding obturator in order to tie a dental floss which was used to obtain extraoral retention. The feeding obturator was checked in the infant's mouth [Figure 6]. Instructions regarding the insertion, removal and cleaning of feeding obturator were given to the parents. The child was recalled after 48 hours to observe any signs of ulceration or irritation. During the follow-up visit, it was observed that the child had accepted the feeding obturator well and the mother reported ease of feeding.
|Figure 5 :Heat cured acrylic palatal feeding obturator. Note ligature to facilitate removal|
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A five-year-old poorly built boy reported to the department of Pediatric dentistry with the chief complaint of decayed teeth. Extraoral examination revealed low set ears, receded chin, and convex profile resembling bird face appearance [Figure 7]. The child's past medical history revealed presence of cardiomegaly [Figure 8].
Intraoral examination revealed a large intraoral cleft involving the soft and hard palate. The child was a case of severe early childhood caries [Figure 9],[Figure 10],[Figure 11],.[Figure 12]. Based on these clinical findings, a diagnosis of PRS was confirmed.
The present case was treated with radical approach. 51, 52, 53, 54, 61, 62, 63, 71, 72, 73, 73, 74, 81, 82, 83, 84 were clinically nonrestorable and planned for extraction. The primary second molars were treated by pulp therapy followed by stainless steel crowns. The child was referred to a plastic surgeon for management of untreated cleft but parents did not show interest in surgical correction. A removable functional space maintainer was fabricated in the maxillary and mandibular arch. The maxillary functional space maintainer also aided in preventing regurgitation while eating. Instructions for maintaining oral hygiene and use of the functional space maintainer were given. Regular follow up was carried out in every three months.
| Discussion|| |
Pierre Robin Sequence is a congenital abnormality characterized by the presence of a combination of mandibular hypoplasia, glossoptosis and often labio palatine clefting.  As with all clinically recognizable syndromes, cases of syndromic cleft lip and palate can be subdivided into those that occur as a part of a characterized Mendelian disorder, those due to structural abnormalities of chromosomes and those occurring as part of syndromes associated with teratogens or those whose causation remains obscure and therefore are currently uncharacterized.  A relatively small number of patients with clefts of palate are not of the multifactorial inheritance.  These patients usually make up approximately 3% of child population and present with one or more additional structural abnormalities. 
Three pathophysiological theories exist to explain the occurrence of micrognathia: 
The mechanical theory, which is the most accepted postulate that the in utero constraint of mandibular growth results in failure of descent of tongue which further prevents fusion of palatal shelves resulting in a cleft palate.
The neurological maturation theory that suggests by the inability of the developing fetus to engage in mandibular exercise prevents the tongue from descending.
The dysregulation theory or the motor and regulatory organization of the rhomboncephalus is related to major problem of ontogenesis.
In general, death from PRS is thought to be the result of poorly controlled combined effects of obstructive apnea and failure to thrive. The palatal cleft interferes with nursing and causes regurgitation of food through nose. Infection of the nasopharynx is frequent. Otitis media may result in 30- 40% of the afflicted, leading to hearing impairment or permanent deafness.  Bronchitis and pneumonia can complicate the local infections. Hence, the construction of the palatal obturator or a feeding plate is very important until the surgical correction of the defect is carried out. Other treatment modalities include the use of specially designed nipples with enlarged openings, and use of orogastric and naso gastric tubes. , Feeding obturator is a prosthetic aid that is designed to obturate the cleft and restore separation between the oral and nasal cavities.  It creates a rigid platform against which the infant can press the nipple and extract milk.  It facilitates feeding, reduces nasal regurgitation, reduces choking and shortens feeding time. It also helps in correct tongue positioning, speech development and reduces the incidence of nasopharyngeal infections.
Use of thermoplastic materials poses a danger of inflicting thermal damage upon delicate soft tissues of the infant. Hence, use of light polymerizing acrylic resin material has been suggested.  The comprehensive management of children with cleft lip and palate demands a multidisciplinary team approach. Pedodontists and orthodontists with medical and allied health specialities must formulate rationale for timing, method and extent of surgical and dental intervention. Immediate fabrication of feeding obturator eliminates problems of failure to thrive and respiratory infections.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12]
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|[Pubmed] | [DOI]|