The maxillofacial growth in 2 patients with Pierre Robin Sequence who received mandibular lengthening in infancy: 5-year follow-up

Objective: Children with Pierre Robin sequence (PRS) and cleft palate have a high rate of velopharyngeal insufficiency (VPI) following primary palatoplasty. Our purpose was to determine the long-term incidence of speech-correcting surgeries (SCSs) and fistula rates in PRS after primary palatoplasty and the influence of possible causal factors. Design: A retrospective single-center, observational chart review study. Participants: After exclusion, the study cohort comprised 78 nonsyndromic PRS children (48 females) born between 1990 and 2009 and treated at the Cleft Palate and Craniofacial Center of Helsinki University Hospital, Finland. Causal factors included gender, surgeon, age at primary palatoplasty, surgical technique, airway obstruction in infancy, and cleft severity. We analyzed the outcome at age 8 years and at data retrieval, with a median follow-up of 14 years (range: 8-27 years). Results: Thirty-four (43.6%) children received SCS by age 8 years, and of the 19 (24.4%) postoperative fistulas, 6 (7.7%) underwent closure. At data retrieval, 37 (47.4%) children had undergone SCS and 8 (10.3%) had a fistula closure. Median age at SCS was 6 years. The results showed no significant association for gender, surgeon, age at primary palatoplasty, surgical technique, cleft severity, or airway obstruction in infancy regarding incidence of SCS, fistulas, or repaired fistulas. Conclusion: Pierre Robin sequence in children is associated with a high incidence of SCS and fistula formation, which necessitates accurate clinical follow-up and observation of speech development. The development of VPI in PRS is complex and most likely involving multiple factors.

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Pierre Robin Sequence: Diagnostic Difficulties Faced while Differentiating Isolated and Syndromic Forms

Acta Medica (Hradec Kralove Czech Republic) ◽

10.14712/18059694.2020.23 ◽

2020 ◽

Vol 63 (2) ◽

pp. 86-90

Author(s):

Girish Gulab Meshram ◽

Neeraj Kaur ◽

Kanwaljeet Singh Hura

Keyword(s):

Positive Family History ◽

Management Plan ◽

Pierre Robin Sequence ◽

Robin Sequence ◽

Long Term Follow Up ◽

Diagnostic Difficulties ◽

History Of ◽

Pierre Robin

Pierre Robin sequence (PRS) is characterized by the triad of retrognathia, glossoptosis, and airway obstruction. PRS may occur in isolation or in conjunction with other syndromes. Distinguishing isolated and syndromic forms of PRS helps clinicians decide the management plan. We describe two cases of PRS of Indian ethnicity and describe some of the difficulties that we faced while distinguishing isolated PRS from syndromic PRS. Both cases had a similar clinical presentation. However, one of the cases had a positive family history of congenital deafness and cleft palate, whereas the other case had apparent upper limb anomalies. These facts heightened the suspicion of an associated syndrome. However, based on the available facts and after thorough investigations, a tentative diagnosis of isolated PRS was made for both the patients. Both the cases were managed conservatively and were advised a long-term follow-up. When the associated anomalies are few, minor or concealed at birth, longitudinal follow-up of all PRS cases combined with thorough diagnostics including chromosomal analysis could help differentiate syndromic PRS from isolated PRS. Regardless, all cases of PRS require a multidisciplinary approach.

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Gastroesophageal Reflux in Pierre Robin Sequence—Early Surgical Treatment

The Cleft Palate-Craniofacial Journal ◽

10.1597/1545-1569_2000_037_0205_griprs_2.3.co_2 ◽

2000 ◽

Vol 37 (2) ◽

pp. 205-208 ◽

Cited By ~ 8

Author(s):

Zofia Dudkiewicz ◽

Ewa Sekuła ◽

Anna Nielepiec-Jałosińska

Keyword(s):

Gastroesophageal Reflux ◽

Pierre Robin Sequence ◽

Upper Respiratory Tract ◽

Floor Of The Mouth ◽

Robin Sequence ◽

Early Closure ◽

Hospital Stays ◽

Tract Infections ◽

Pierre Robin

Objective To assess the prevalence of gastroesophageal reflux (GER) in patients with Pierre Robin sequence and to assess whether early palatoplasty with subperiosteal release of the floor of the mouth eliminates upper respiratory tract infections and gastroesophageal reflux. Methods In the period from 1993 to 1997, 24 children with Pierre Robin sequence were treated. Because GER is known to occur in patients with this syndrome, we screened for GER in each child for whom an early palatoplasty was to be performed at 3 months of age. In 21 cases, the presence of GER was confirmed. Within the past 2 years, palatoplasty has been combined with subperiosteal release of the floor of the mouth (SRFM). Prompt clinical improvement and shortened hospital stays have resulted. Results and Conclusions Follow-up studies performed several months after the surgery showed no evidence of GER. The authors conclude that an early closure of the impaired palatopharyngeal ring and the elimination of the mechanism that causes the posterior rotation of the tongue can reduce the need for such surgical procedures as glossopexy, gastrostomy, tracheostomy, and Nissen's fundoplication.

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