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Smith-Magenis Syndrome—A Developmental Disorder with Circadian Dysfunction
Published in Merlin G. Butler, F. John Meaney, Genetics of Developmental Disabilities, 2019
Ann C.M. Smith, Wallace C. Duncan
There are only two known cases of SMS with published CNS neuropathological findings. The first (2) died 6hr postoperatively of cardiogenic shock following repair of a ventricular septal defect. Notable CNS findings included microcephaly and foreshortened frontal lobes with depletion of neurons frontally and a small chorid plexus hemangioma in the lateral ventricle. The second case, a male born with cleft palate, died 2 days postoperatively following palatoplasty at 11 months of age (66). Clinically, he suffered respiratory arrest and hypotension, and died of apparent acute adrenal insufficiency that was confirmed at postmortem exam. On gross examination, the brain structures were normally formed (cerebrum, basal ganglia, midbrain, pons, and cerebellum) with widened gyri and narrowed sulci. Histological exam showed defined loss of the granular layer of cerebellum, but no evidence of dysplasia or lissencephaly.
Clefts and craniofacial
Published in Tor Wo Chiu, Stone’s Plastic Surgery Facts, 2018
Some have a blue line/translucency aka ‘zona pellucida’ indicating a separation of the palatal musculature. Most patients have normal speech, though about 15% have VPI, which can be the presenting complaint. Others can present with feeding or hearing problems, or else simply found on routine paediatric examination. The severity of the VPI is not directly correlated to the physical signs. If speech and ENT assessment are acceptable, then palatal or pharyngeal surgery can be avoided; otherwise, the traditional surgical option is pharyngoplasty. Other options include palatoplasty (Furlows, intravelar veloplasty).
Cleft Lip and Palate
Published in John C Watkinson, Raymond W Clarke, Christopher P Aldren, Doris-Eva Bamiou, Raymond W Clarke, Richard M Irving, Haytham Kubba, Shakeel R Saeed, Paediatrics, The Ear, Skull Base, 2018
David M. Wynne, Louisa Ferguson
Management decisions come down to underlying symptoms and the size and location of the velopharyngeal gap. Palatoplasty may be indicated to treat VPI, either as a re-repair where there is evidence of anterior insertion of the levator muscles, or in the case of a submucous cleft. It is mainly used where the velopharyngeal gap is small73,74 and has been shown to have a lower morbidity than a pharyngoplasty.75 Pharyngoplasty involves altering the shape of the velopharyngeal port in order to allow closure on speech. This can be done either by using flaps from the midline of the pharyngeal wall or by employing medial transposition of flaps from the lateral pharyngeal wall. Both types of pharyngoplasty have been shown to improve speech outcomes, with the possibility of achieving normal resonance in up to 85% of cases. The main downside to pharyngoplasty is the associated increased risk of sleep apnoea, and this must be discussed with the patient and their family pre-operatively.
Long-term outcomes in children with and without cleft palate treated with tympanostomy for otitis media with effusion before the age of 2 years
Published in Acta Oto-Laryngologica, 2020
Maki Inoue, Mariko Hirama, Shinji Kobayashi, Noboru Ogahara, Masahiro Takahashi, Nobuhiko Oridate
In the present study, the otological and audiological outcomes of children with cleft palate who were treated with early tympanostomy for OME were similar to those of children without cleft palate. None of the children developed cholesteatoma, and, overall, the audiological prognosis of most of the children was favorable. Therefore, from an audiological point of view, the findings of this study suggest that children treated with early tympanostomy can have satisfactory hearing levels in the long term (up to 7 years), regardless of the presence or absence of cleft palate. Thus, we suggest that children with cleft palate may be treated for OME in the same way as children without cleft palate. In addition, we speculate that early palatoplasty in children with cleft palate may contribute to favorable outcomes, although further research is needed in this respect. Some children with and without cleft palate underwent their first retympanostomy after more than 5 years from the first tympanostomy. Moreover, some children in both groups had ears with TM perforation. Therefore, long-term follow-up is important for children treated with early tympanostomy, regardless of the presence or absence of cleft palate.
Comparison of postoperative pain scores and dysphagia between anterior palatoplasty and uvulopalatal flap surgeries
Published in Acta Oto-Laryngologica, 2018
Elvan Yüksel, Murad Mutlu, Ömer Bayır, Melike Yüceege, İstemihan Akın, Güleser Saylam, Ali Özdek, Hikmet Fırat, Mehmet Hakan Korkmaz
Surgery is one of the options in OSAS treatment. Obstruction may be located in any part of the upper airway from nose to the epiglottis. It is a well-known fact that retropalatal region is the most common place of obstruction in patients with OSAS [3]. In order to create scar tissue in soft palate, various surgical techniques were implemented, which led to fibrosis and stiffening of the palate. Numerous surgical procedures directed to the soft palate may be performed alone or together with some other surgical interventions. Anterior palatoplasty (AP) is one of the recent surgical procedures performed with this purpose. In our center, AP is performed as defined by Pang and Terris [4]. Uvulopalatal flap (UPF) is another surgical procedure directed to the soft palate and this procedure is performed as defined by Powell et al. [5] in our clinic. Oropharyngeal and nasopharyngeal airway openings are enlarged with those operations.
Speech outcomes at 5 and 10 years of age after one-stage palatal repair with muscle reconstruction in children born with isolated cleft palate
Published in Journal of Plastic Surgery and Hand Surgery, 2018
Jill Nyberg, Erik Neovius, Anette Lohmander
The minimal incision surgical technique for palatal repair including muscle reconstruction (MITmr) has been used since 1997 by the Stockholm Craniofacial Team. The technique was developed by Sommerlad [21]. The muscle reconstruction is radical with an intravelar veloplasty. Andrades et al. [22] showed significantly better speech outcomes and the prevalence of fewer secondary palatal surgeries after performing a two-flap palatoplasty with radical muscle reconstruction, as compared to surgery that did not involve an extensive dissection and repositioning of the levator muscle. This is similar to the results presented by Nyberg et al. [18], where a group treated with MITmr showed significantly fewer velopharyngeal flaps when compared to a group with minimal incision technique without muscle reconstruction (MIT). However, the MITmr group was small (only 26 children compared with the MIT group with 60 children), which could have affected the results, as well as the surgical skill and experience.