Predictors of speech outcomes in children with Pierre Robin sequence.

BACKGOUND: Pierre Robin sequence (PRS) has worse speech outcomes than isolated cleft palate. We aimed to search for possible associations of phonological outcomes with PRS status (isolated vs syndromic), clinical severity, soft palate muscles deficiency, or surgical procedure. METHODS: We designed a retrospective study of 130 children (male/female ratio: 0.4) with isolated (96) or syndromic (34) PRS with cleft palate. Grading systems were used to classify retrognathia, glossoptosis, and respiratory and feeding disorders. Electromyography was used to investigate levator veli palatini muscles. Hard cleft palate was measured using maxillary casts. Intravelar veloplasty was performed using the Sommerlad's technique. Phonological outcomes were assessed using the Borel-Maisonny classification. RESULTS: Cleft palate was repaired in one stage (65.5%) or hard palate closure was postponed (34.5%). Velopharyngeal insufficiency was more frequent in syndromic PRS (53%) vs. isolated PRS (30.5%) (p = 0.01), but was not statistically associated with clinical grade, hard cleft palate width, soft palate electromyography, and surgical procedure. CONCLUSIONS: In children with PRS, anatomic variables, initial clinical severity, and soft palate muscle deficiency are not predictors of speech prognosis.

Grommets and speech at three and six years in children born with total cleft or cleft palate.

OBJECTIVE: Grommets may be considered as the treatment of choice for otitis media with effusion (OME) in children born with a cleft. But the timing and precise indications to use them are not well established. The aim of the study is to compare the results of hearing and speech controls at three and six year-old in children born with total cleft or cleft palate in the presence or not of grommets. METHODS: This retrospective study concerns non syndromic children born between 1994 and 2006 and operated for a unilateral cleft lip palate (UCLP) or a cleft palate (CP) alone, by one surgeon with the same schedule of operations (Malek procedure). We compared the results of clinical observation, tympanometry, audiometry and nasometry at three and six year-old. The Borel-Maisonny classification was used to evaluate the velar insufficiency. None of the children had preventive grommets. The Fisher Exact Test was used for statistical analysis with p<0.05 considered as significant. RESULTS: Seventy-seven patients were analyzed in both groups. Abnormal hearing status was statistically more frequent in children with UCLP compared to children with CP, at three and six years (respectively, 80-64%, p<0.03 and 78-60%, p<0.02), with the use of grommets at six years in 43% of cases in both groups. Improvement of hearing status between three and six year-old was present in 5% of children with UCLP and 9% with CP, without the use of grommets. CONCLUSION: The use of grommets between three and six year-old was not associated to any improvement of hearing status or speech results children with UCLP or with CP, with a low risk of tympanosclerosis. These results favor the use of grommets before the age of three, taking into account the risk of long term tympanosclerosis.

Speech prognosis and need of pharyngeal flap for non syndromic vs syndromic Pierre Robin Sequence.

BACKGROUND: The aim of this retrospective study was to evaluate speech outcome and need of a pharyngeal flap in children born with nonsyndromic Pierre Robin Sequence (nsPRS) vs syndromic Pierre Robin Sequence (sPRS). METHODS: Pierre Robin Sequence was diagnosed when the triad microretrognathia, glossoptosis, and cleft palate were present. Children were classified at birth in 3 categories depending on respiratory and feeding problems. The Borel-Maisonny classification was used to score the velopharyngeal insufficiency. RESULTS: The study was based on 38 children followed from 1985 to 2006. For the 25 nsPRS, 9 (36%) pharyngeal flaps were performed with improvements of the phonatory score in the 3 categories. For the 13 sPRS, 3 (23%) pharyngeal flaps were performed with an improvement of the phonatory scores in the 3 children. There was no statistical difference between the nsPRS and sPRS groups (P = .3) even if we compared the children in the 3 categories (P = .2). CONCLUSIONS: Children born with nsPRS did not have a better prognosis of speech outcome than children born with sPRS. Respiratory and feeding problems at birth did not seem to be correlated with speech outcome. This is important when informing parents on the prognosis of long-term therapy.

Speech outcome after cranial-based pharyngeal flap in children born with total cleft, cleft palate, or primary velopharyngeal insufficiency.

PURPOSE: The aim of this study was to compare the effect of a cranial-based pharyngeal flap on the speech of children born with a unilateral cleft lip and palate (UCLP), bilateral cleft lip and palate (BCLP), cleft palate (CP), or primary velopharyngeal insufficiency (VPI) without cleft. PATIENTS AND METHODS: A total of 234 children born with clefts and 22 children born with primary VPI were evaluated. Children with associated abnormalities were excluded from this study. The Borel-Maisonny classification system was used to evaluate the velar insufficiency. The cranial-based pharyngeal flap was performed using the Sanvenero-Rosselli technique. RESULTS: Between 1984 and 2001, 74 children underwent pharyngeal flap for VPI. The mean follow-up period was 7 years. Borel-Maisonny scores after pharyngeal flap surgery were as follows: children with UCLP (n = 22), 59.1% type 1, 36.4% type 1/2, and 4.5% type 2; children with BCLP (n = 18), 44.4% type 1, 27.8% type 1/2, 16.7% type 2, and 11.1% type 2/3; children with CP (n = 17), 64.7% type 1, 23.5% type 1/2, and 11.8% type 2; children with primary VPI (n = 17), 29.4% type 1, 29.4% type 1/2, 29.4% type 2/3, and 11.8% type 3. There were significant differences in outcome among the 4 groups (P = .029; Fisher exact test). CONCLUSIONS: The positive effect on speech of a cranial-based pharyngeal flap is greater in children born with a UCLP or CP than in those born with a BCLP. In children born with primary VPI, this operation has only a slightly positive effect on speech that shows compensatory misarticulations; in such cases, alternative surgical choices or secondary procedures may be indicated. This information should be clearly conveyed to the parents in presurgical consultation so that they know what to expect from the procedure and postoperative adjuvant therapy.