Presentation and Treatment of a Patient With Jacobs Syndrome and Metopic Craniosynostosis.

Jacobs syndrome is a rare trisomy (47, XYY) found in ~1 in 1000 male children associated with infertility, autism spectrum disorders, macrocephaly, hypertelorism, tall stature, and macroorchidism. Diagnosis is often delayed due to relatively subtle phenotypic changes. Craniosynostosis, a fusion of the cranial sutures, has been described in ~1 in 2000 live births, of which 25% are related to a diagnosed syndrome with the most common being Apert and Crouzon. Craniosynostosis does not have a known association with Jacobs syndrome and no prior cases have been reported. This case report seeks to describe the presentation and treatment of a patient with Jacobs syndrome and metopic craniosynostosis.

Pierre Robin and breathing: What to do and when?

Robin sequence is characterized by mandibular retrognathia, airway obstruction, and glossoptosis; 80%-90% also have a cleft palate. Various treatment approaches exist, and although controlled studies are rare, objective assessment of treatment outcomes that address the leading clinical issues, namely obstructive sleep apnea and failure to thrive, are essential. Sleep-disordered breathing may be detected using cardiorespiratory polygraphy or polysomnography. Pulse oximetry alone may miss infants with frequent obstructive apneas, yet no intermittent hypoxia. Among conservative treatment options, the Tubingen Palatal Plate with a velar extension shifting the tongue base forward is the only approach that corrects the underlying anatomy and that has undergone appropriate evaluation. Of the surgical treatment options, which are not necessarily the first line of therapy, mandibular distraction osteogenesis (MDO) is effective and has been most extensively adopted. Notwithstanding, it is puzzling that MDO is frequently used in some countries, yet hardly ever in others, despite similar tracheostomy rates. Thus, prospective multicenter studies with side-by-side comparisons aimed at identifying an optimal treatment paradigm for this potentially life-threatening condition are urgently needed.

Consistency of Cranial Shape Measures Obtained From Laser Surface and Computed Tomography Imaging.

OBJECTIVE: Children with cranial shape abnormalities are often subjected to radiation from computed tomography (CT) for evaluation and clinical decision making. The STARscanner Laser Data Acquisition System (Orthomerica, Orlando, FL) may be a noninvasive alternate. The purpose of this study is to determine whether the STARscanner provides valid and accurate cranial measurements compared to CT. DESIGN: We performed an institutional review board-approved retrospective review of a prospectively maintained database of patients with metopic suture abnormalities from 2013 to 2016. SETTING: Plastic surgery clinic in an institutional tertiary care center. PATIENTS: Eight patients were included that presented with metopic suture abnormalities, age less than 1 year, and CT and STARscanner imaging within 30 days of one another. MAIN OUTCOME MEASURES: Cranial measurements were collected twice from 3 scan types: STARscanner, CT windowed for soft tissue, and CT windowed for bone. Measurements included: intracranial volume, height, base width, maximum antero-posterior length, maximum medio-lateral width, and oblique diameters. Nested analysis of variance were performed to determine the proportion of error attributable to: between-subject variance, scan type, and rater. RESULTS: Measurements from STARscanner and both CT scans windows were highly consistent, with less than 1% of total error attributable to scan type for all measures. CONCLUSIONS: Cranial shape measurements obtained from STARscanner images are highly consistent with those obtained from CT scans. The STARscanner has added benefits of speed of acquisition, minimal cost, and lack of radiation.

A case report: resolution of Chiari I malformation after helmet therapy for deformational brachycephaly.

Positional plagiocephaly is the most common cause of cranial asymmetry. Deformational brachycephaly denotes a head shape characterized by occipital flattening and increased bilateral width, which can also be caused by external deformation of the moldable infant cranium in positional bilateral posterior plagiocephaly. There are reports of craniosynostosis associated with Chiari I malformation (CIM), possibly caused by decreased posterior fossa volume and related to increased intracranial pressure. To the best of our knowledge, this is only the second case report demonstrating acquired CIM in a child with positional brachycephaly. Of note, the fact that the CIM resolved after helmet therapy could support the hypothesis that CIM is associated with decreased volume of the posterior fossa. However, these two conditions may be independent of one another. More research is needed to identify an association between the two conditions.

Odontogenic Cysts and Tumors.

Odontogenic cysts and tumors are mandibular and maxillary lesions that occur across all patient demographics across age, sex, race, and social economic status, as altered remnants of dental development. They may be incidental findings from routine imaging in any office or found through workup for craniofacial surgery or injury. Many of these patients present with asymptomatic lesions, whereas others may be symptomatic. In this article, we review the literature on the most common odontogenic tumors and cysts and discuss their presentation, their defining traits, and how to approach diagnosis and definitive management.

Relationship of a Metopic Ridge and Anterior Cranial Volume Measured by a Noninvasive Laser Shape Digitizer.

Cranial dysmorphology observed in patients with metopic craniosynostosis varies along a spectrum of severity including varying degrees of metopic ridging, bitemporal narrowing, and trigonocephaly. Management has been based upon the subjective clinical impression of presence and severity of trigonocephaly. Severity of cranial dysmorphology does not predict the occurrence or severity of associated abnormal neurodevelopment, as children with mild-to-moderate trigonocephaly may also experience developmental delays. The authors sought to determine the relationship between mild-to-moderate trigonocephaly and anterior cranial volume using a noninvasive laser shape digitizer (STARscanner) in patients with abnormal head shape.An IRB-approved retrospective review of a prospectively maintained database and medical records was performed. Two hundred three patients less than 1 year of age with abnormal head shape were categorized as having a metopic ridge with mild-to-moderate trigonocephaly, metopic ridge without trigonocephaly, or no ridge. Measurements of cranial volume, circumference, and symmetry were calculated by the STARscanner, which quantifies three-dimensional shape of the cranial surface. Measures were analyzed using a series of analyses of variance and post-hoc Tukey honest significant difference.The authors results showed ACV was significantly reduced in patients with mild-to-moderate trigonocephaly compared with those without metopic ridge (P = 0.009), and trended toward significance compared with those with a ridge but without trigonocephaly (P = 0.072). The ratio of anterior-to-posterior cranial volume was significantly reduced in those with mild-to-moderate trigonocephaly compared with those without metopic ridge (P = 0.036).In conclusion, patients with milder anterior cranial deformities demonstrated an association between a metopic ridge with mild-to-moderate trigonocephaly and reduced anterior cranial volume.

Traumatic Fracture of the Premaxilla in the Bilateral Cleft Patient.

Traumatic fracture of the premaxilla is a rare event, and there is minimal data regarding the presentation, management, and outcome of these patients. This article reports 2 patients with bilateral cleft lip and palate who each presented with traumatic fracture and displacement of the premaxilla. To authors' knowledge, the occurrence and management of a traumatic fracture and displacement of the premaxilla in a patient with cleft lip and palate has not been reported.

Incidence of Amblyopia and Its Risk Factors in Children With Isolated Metopic Craniosynostosis.

OBJECTIVE: Ophthalmic abnormalities in children with syndromic craniosynostosis have been reported previously, and referral of these children to a pediatric ophthalmologist is recommended. However, it is not as clear whether a child with nonsyndromic synostosis needs to be referred to a pediatric ophthalmologist. The aim of this study is to report the incidence of amblyopia and its risk factors in children with isolated metopic craniosynostosis. DESIGN: An institutional review board-approved, retrospective review was performed on 91 children diagnosed with isolated metopic craniosynostosis. Ophthalmologic records were reviewed for diagnoses of amblyopia, strabismus, and refractive error. RESULTS: Of the 91 children, 19 (20.9%) had astigmatism, eight (8.8%) had amblyopia, eight (8.8%) had strabismus, five had myopia (5.5%), five had hyperopia (5.5%), and five had anisometropia (5.5%). The incidence of amblyopia and its risk factors found in our study are higher than the rate found in the clinically normal pediatric population. CONCLUSIONS: In our patient population, children with isolated metopic craniosynostosis demonstrate an increased rate of amblyopia and its risk factors. Amblyopia is best treated early in life to achieve a successful outcome. A referral to a pediatric ophthalmologist for a formal eye exam and potential treatment is therefore recommended for children with isolated metopic craniosynostosis.

Use of the On-Q Pain Pump in Alveolar Bone Grafting: Effect on Hospital Length of Stay.

OBJECTIVE: Alveolar bone grafting (ABG) with iliac crest bone graft can be associated with significant pain at the donor site. The On-Q pain pump has been shown to be efficacious in treating postsurgical pain. The aim of this study was to compare the length of postoperative hospital stay in patients undergoing ABG who received the On-Q pain pump at the iliac crest donor site (On-Q+) with that of patients who did not receive the On-Q pain pump (On-Q-). DESIGN: A retrospective, cohort study, approved by institutional review board, was performed. Thirty-one consecutive patients in the On-Q- group were compared with 38 consecutive patients in the On-Q+ group. The two cohorts were assessed for length of stay. Statistical analysis was performed using the Fisher exact probability test. SETTING: Tertiary care academic medical center. PATIENTS: Sixty-nine patients with cleft lip and/or cleft palate (CL/P) undergoing secondary ABG with iliac crest bone graft were operated on between May 1993 and January 2014. MAIN OUTCOME MEASURE: Length of postoperative hospital stay. RESULTS: Mean length of stay in the On-Q- patients was 0.52 days versus 0.37 days for the On-Q+ patients. This difference between the two cohorts was not statistically significant (P = .234). CONCLUSION: Although there is a trend toward a shorter length of stay in our patients who received the On-Q pump, this finding was not statistically significant. Given the expense and additional burden of care associated with the device, we have become more selective in its utilization.

Evaluating Children With Metopic Craniosynostosis: The Cephalic Width-Intercoronal Distance Ratio.

OBJECTIVE: To identify an additional objective measure to aid in the evaluation of children with isolated metopic craniosynostosis. DESIGN: This is a retrospective study comparing specific computed tomography scan measurements between surgical and nonsurgical cohorts of children with isolated metopic craniosynostosis. Children were included if they were diagnosed with isolated metopic craniosynostosis and ultimately underwent computed tomography scan imaging as part of their evaluation. The subjects were placed in the surgical or nonsurgical cohorts on the basis of the final treatment recommendation after they completed a full multidisciplinary, multimodality evaluation. Comparisons were made with a control group of unaffected patients from our institutional trauma registry. SETTING: Tertiary academic institution. PATIENTS, PARTICIPANTS: The subjects are patients who had been previously evaluated in our clinic for isolated metopic craniosynostosis and received a computed tomography scan as part of their workup. RESULTS: The average intercoronal distances were significantly different among all three groups (P < .002). The average cephalic width-intercoronal distance ratio for children who received a recommendation for surgery differed significantly from that of both the observation cohort and the control group (P < .001). However, the cephalic width-intercoronal distance ratio did not differ between the observation cohort and the control group (P = .927). CONCLUSIONS: The cephalic width-intercoronal distance ratio may be an additional objective measurement to aid in the clinical evaluation of children with metopic craniosynostosis.

Intraoperative tissue expansion in the surgical correction of craniosynostosis.

BACKGROUND: Wound healing complications can occur after calvarial vault expansion due to tension on the scalp flaps. OBJECTIVES: To compare wound healing outcomes in patients with craniosynostosis undergoing calvarial vault expansion with and without intraoperative tissue expansion of the scalp. METHODS: The present analysis was an institutional review board-approved, retrospective cohort study involving 40 consecutive patients at the University of Missouri Hospitals and Clinics (Columbia, Missouri, USA) who underwent calvarial vault expansion for nonsyndromic craniosynostosis between June 1, 2009 and June 30, 2012. Patients were divided into two sequential cohorts: the first 20 underwent calvarial vault expansion without intraoperative tissue expansion; the second 20 underwent calvarial vault expansion with intraoperative tissue expansion. The main outcome measures included presence or absence of wound healing complications (persistent scabbing or slow-healing wounds, hardware exposure, need for operative wound revision or healed but widened scars), with documented postoperative follow-up of at least three months. The primary end point was the presence of a well-healed scar; the secondary end point was the need for an operative revision. RESULTS: Patients in the intraoperative tissue expansion group had a higher percentage of well-healed scars (73.6%) than those in the nonexpansion group (42.1%) (P=0.0487). This difference was primarily due to scar widening in the nonexpansion group. CONCLUSIONS: The present study demonstrated that the use of intraoperative tissue expansion in patients with nonsyndromic craniosynostosis who underwent calvarial vault expansion resulted in a greater likelihood of a well-healed incision with a lower rate of poor scarring.

HISTORIQUE: Après une expansion de la calotte crânienne, la cicatrisation peut être compliquée par la tension exercée sur les lambeaux du cuir chevelu. OBJECTIFS: Comparer les résultats de la cicatrisation chez des patients ayant une craniosténose soignée par expansion de la calotte crânienne accompagnée ou non d'une expansion peropératoire des tissus du cuir chevelu. MÉTHODOLOGIE: La présente analyse, une étude de cohorte rétrospective approuvée par le conseil d'examen de l'établissement, a été réalisée auprès de 40 patients consécutifs des hôpitaux et des cliniques de l'université du Missouri (Columbia, Missouri, États-Unis) qui ont été soignés pour une craniosténose non syndromique au moyen d'une expansion de la calotte crânienne entre le 1er juin 2009 et le 30 juin 2012. Les chercheurs ont divisé les patients en deux cohortes séquentielles. Les 20 premiers ont subi une expansion de la calotte crânienne sans expansion peropératoire des tissus et les 20 suivants, une expansion de la calotte crânienne accompagnée d'une expansion peropératoire des tissus. Les principales mesures de résultats incluaient la présence ou l'absence de complications de la cicatrisation (gales persistantes ou guérison ralentie, exposition à un corps étranger, nécessité d'une chirurgie corrective ou guérison, mais élargissement des cicatrices), étayée par un suivi postopératoire d'au moins trois mois. Le paramètre primaire était la présence d'une cicatrice bien guérie et le paramètre secondaire, la nécessité de procéder à une chirurgie corrective. RÉSULTATS: Les patients faisant partie du groupe soigné par expansion peropératoire des tissus présentaient un pourcentage plus élevé de cicatrices bien guéries (73,6 %) que ceux du groupe n'ayant pas subi cette expansion (42,1 %) (P=0,0487). Cette différence était surtout attribuable à l'élargissement des cicatrices dans le groupe n'ayant pas subi cette expansion. CONCLUSIONS: La présente étude démontre que l'expansion peropératoire des tissus chez les patients ayant une craniosténose non syndromique soignés par expansion de la calotte crânienne était plus susceptible de présenter une incision qui guérissait bien et qui cicatrisait mieux.

Analysis of individualized education programs to quantify long-term educational needs following surgical intervention for single-suture craniosynostosis.

BACKGROUND: Single-suture craniosynostosis (SSC) is a common craniofacial condition with potential neurocognitive sequelae. OBJECTIVE: To quantify any long-term functional academic and behavioural difficulties of children with SSC as indicated by the need for individualized education programs (IEPs), despite having undergone surgical treatment. METHODS: Records of all school-age patients from 1992 to 2011 who underwent operative intervention for SSC were identified. Fifty-nine patients' guardians were contacted by telephone to provide informed consent for completion of a mailed standardized questionnaire querying demographic information as well as information regarding the patient's health, family and educational history; specifically whether the patient had ever been provided educational support as delineated in an IEP. The primary outcome measure was the history of the patient being assigned educational support as delineated in an IEP. RESULTS: Thirty-seven consenting guardians completed and returned the standardized questionnaire (response rate 62.7%). Twenty-one patients were male and 16 were female, with an age range of five to 14 years (mean age 10.2 years). Eleven (29.7%) patients had a previous history of or currently were receiving educational support delineated in an IEP. CONCLUSIONS: A higher proportion of school-age patients with a history of SSC (status postsurgical intervention) in the present study received educational support delineated in an IEP than the proportion of IEPs in the general student population of the United States (11.3%).

HISTORIQUE: La craniosténose à suture simple (CSS) est un problème craniofacial courant qui s'associe à un potentiel de séquelles neurocognitives. OBJECTIF: Quantifier les problèmes scolaires et comportementaux à long terme chez les enfants ayant une CSS, tels qu'ils sont indiqués par la nécessité de recourir à des programmes d'enseignement individualisé (PEI) malgré un traitement chirurgical. MÉTHODOLOGIE: Les chercheurs ont extrait les dossiers de tous les patients d'âge scolaire qui ont subi une intervention chirurgicale en raison d'une CSS entre 1992 et 2011. Ils ont téléphoné aux tuteurs ou aux parents de 59 patients afin d'obtenir leur consentement éclairé et de leur poster un questionnaire standardisé sur la démographie et la santé des patients, leur famille et leurs antécédents éducatifs. Ils ont demandé si le patient a déjà reçu un soutien éducatif expliqué dans un PEI, ce qui constituait la mesure d'issue primaire. RÉSULTATS: Trente-sept parents et tuteurs consentants ont rempli et envoyé le questionnaire standardisé (taux de réponse de 62,7 %). Ainsi, 21 patients étaient des garçons et 16 des filles, et tous avaient de cinq à 14 ans (âge moyen de 10,2 ans). Onze patients (29,7 %) avaient des antécédents de soutien éducatif expliqué dans un PEI ou recevaient encore un tel soutien. CONCLUSIONS: Dans la présente étude, une plus forte proportion de patients d'âge scolaire ayant une histoire de CSS (intervention sur l'état postchirurgical) a reçu un soutien éducatif exposé dans un PEI que la proportion de PEI dans la population générale d'élèves des États-Unis (11,3 %).

Does the use of particulate bone graft increase the incidence of postoperative infection in surgery for craniosynostosis?

OBJECTIVE: The use of particulate bone graft (PBG) has become an accepted technique for filling cranial defects created during cranial vault expansion for craniosynostosis. However, the use of PBG may be a risk factor for postoperative infection. The aim of this study was to compare the rate of postoperative infection in patients who received particulate bone graft (PBG+) with that in patients who did not (PBG-). DESIGN: An Institutional Review Board-approved, retrospective, cohort study of consecutive patients was performed. Twenty-seven consecutive patients in the PBG- group were compared with 21 consecutive patients in the PBG+ group. The two cohorts were assessed for incidence of surgical-site infection. RESULTS: Statistical analysis was performed using the Fisher exact probability test. Surgical site infection occurred in none of the PBG- patients (0%) versus one of the PBG+ patients (4.76%). This difference in infection rates between the two cohorts was not statistically significant (P = .4375). CONCLUSIONS: Although there may be concern that PBG could serve as a facilitative medium for bacterial growth, this study demonstrates no statistically significant increase in infection rates with its use. Particulate bone grafting of cranial defects resulting from cranial vault expansion in craniosynostosis remains a useful and valuable technique.

Incidence of Amblyopia and Its Risk Factors in Children With Isolated Metopic Craniosynostosis.

OBJECTIVE: Ophthalmic abnormalities in children with syndromic craniosynostosis have been reported previously, and referral of these children to a pediatric ophthalmologist is recommended. However, it is not as clear whether a child with nonsyndromic synostosis needs to be referred to a pediatric ophthalmologist. The aim of this study is to report the incidence of amblyopia and its risk factors in children with isolated metopic craniosynostosis. DESIGN: An institutional review board-approved, retrospective review was performed on 91 children diagnosed with isolated metopic craniosynostosis. Ophthalmologic records were reviewed for diagnoses of amblyopia, strabismus, and refractive error. RESULTS: Of the 91 children, 19 (20.9%) had astigmatism, eight (8.8 %) had amblyopia, eight (8.8%) had strabismus, five had myopia (5.5%), five had hyperopia (5.5%), and five had anisometropia (5.5%). The incidence of amblyopia and its risk factors found in our study are higher than the rate found in the clinically normal pediatric population. CONCLUSIONS: In our patient population, children with isolated metopic craniosynostosis demonstrate an increased rate of amblyopia and its risk factors. Amblyopia is best treated early in life to achieve a successful outcome. A referral to a pediatric ophthalmologist for a formal eye exam and potential treatment is therefore recommended for children with isolated metopic craniosynostosis.

Influence of preoperative velar closing ratio and lateral wall movement on outcomes of Furlow palatoplasty for velopharyngeal incompetence.

Currently, there is no consensus regarding how to determine the optimal surgical procedure for a patient with velopharyngeal incompetence (VPI) post-primary palate repair. The purpose of the present study was to assess the effect of preoperative velar closing ratio (VCR) and lateral wall movement (LWM) on nasal emission and hypernasality after Furlow double-opposing Z-plasty. A retrospective analysis involving patients with VPI post-primary palatoplasty whose VPI was treated with double-opposing Z-plasty by a single surgeon was performed. Ten consecutive patients with VPI postpalatoplasty were reviewed. Videonasendoscopy, videofluoroscopy and perceptual speech examinations were performed preoperatively and postoperatively. VCR improved from an mean of 0.5 preoperatively (range 0.1 to 0.95) to 0.9 postoperatively (range 0.55 to 1.0). Postoperative mean LWM was 0.5 (range 0.3 to 0.9), unchanged from preoperative ratings. A trend toward an inverse relationship between preoperative VCR and improvement in hypernasality and resolution of nasal emission was observed. No relationship was noted between the degree of preoperative LWM and mean improvement in hypernasality. However, patients with worse preoperative LWM experienced better resolution of nasal emission postoperatively.

Il n'y a pas de consensus pour déterminer l'intervention chirurgicale optimale chez un patient présentant une insuffisance vélopharyngée (IVP) après une réparation palatine primaire. La présente étude visait à évaluer l'effet du ratio de fermeture vélaire (RFV) et de mouvement des parois latérales (MPL) sur l'émission nasale et l'hypernasalité après une plastie en Z en double opposition selon Furlow. Les chercheurs ont procédé à une analyse rétrospective de patients qui avaient subi une palatoplastie primaire et qui présentaient une IVP traitée par un chirurgien par une plastie en Z en double opposition. Ils ont examiné dix patients consécutifs ayant une IVP après leur palatoplastie. Ils ont effectué une vidéo-endoscopie nasale, une vidéo-fluoroscopie et des examens orthophoniques perceptuels avant et après l'opération. Le RFV s'est amélioré d'une moyenne de 0,5 avant l'opération (plage de 0,1 à 0,95) à une moyenne de 0,9 après l'opération (plage de 0,55 à 1,0). Le MPL moyen après l'opération était de 0,5 (plage de 0,3 à 0,9), identique aux mesures préopératoires. Les chercheurs ont observé une tendance inversement proportionnelle entre le RFV préopératoire, l'amélioration de la nasalité et la résolution de l'émission nasale. Ils n'ont pas remarqué de relation entre le degré préopératoire de MPL et l'amélioration moyenne de l'hypernasalité. Cependant, les patients dont le MPL était plus prononcé avant l'opération présentaient une meilleure résolution de l'émission nasale après l'opération.

Normocephalic pancraniosynostosis.

Normocephalic pancraniosynostosis is a rare variant of craniosynostosis associated with delayed presentation and elevated intracranial pressure. We present 2 cases of normocephalic pancraniosynostosis highlighting the common clinical course, radiographic findings, and intraoperative findings seen in children with normocephalic pancraniosynostosis.

A practical correction of great toe claw deformity.

Ischemic contracture of the leg causing clawing of the toes is a known complication of compartment syndrome of the leg. Although a substantial amount of published data are available on the prevention and acute management of compartment syndrome, a relative paucity of data has been published on the optimal management of the resultant claw toe deformity. In the present case report, the operative management of a patient with left great toe clawing secondary to ischemia is described. Surgical management included lengthening of the extensor hallucis longus tendon and transfer of the extensor hallucis brevis tendon to the extensor hallucis capsularis, with percutaneous pinning of the great toe.

Contractility of sphincter pharyngoplasty: Relevance to speech outcomes.

BACKGROUND: Sphincter pharyngoplasty has demonstrated time-tested results as a surgical treatment for velopharyngeal incompetence (VPI). However, controversy surrounding the contractility of the transposed muscles persists. Completely unaddressed in the literature is whether the dynamism of the sphincter affects speech outcomes. OBJECTIVE: To determine whether active sphincter contraction following sphincter pharyngoplasty influences velopharyngeal closure, nasal emission and hypernasality. METHODS: A prospective analysis of patients with VPI after cleft palate repair undergoing sphincter pharyngoplasty by a single surgeon was performed. Video nasendoscopy and videofluoroscopy were performed preoperatively and postoperatively at three and 12 months. Eighteen consecutive patients with cleft palate with or without cleft lip and VPI were reviewed. The average age of the patients at initial evaluation was 7.3 years, with a range of three to 19 years. Dynamicity of sphincter pharyngoplasty, velar closing ratio (VCR), and lateral wall movement (LWM) were assessed by nasendoscopy and videofluoroscopy. Nasal emission and hypernasality were assessed by perceptual speech examination. RESULTS: FOR LONGITUDINAL COMPARISON, THREE GROUPS WERE CREATED: dynamic at three and 12 months (n=12); adynamic at three months and dynamic at 12 months (n=4); and adynamic at three and 12 months (n=2). Perceived hypernasality scores significantly improved at three months (P=0.0001) and showed continued improvement at 12 months (P=0.03), despite no change in VCR and LWM from three to 12 months. There were no significant differences among the three groups at any time point. DISCUSSION: Sphincter pharyngoplasty effectively treats VPI in appropriately selected patients. Although the VCR and LWM remained stable between three months and one year, four of six adynamic sphincters became dynamic. Considering all patients, hypernasality showed continued improvement from three months to one year. CONCLUSIONS: There were no differences between dynamic and adynamic sphincters in terms of speech outcomes or the mechanical properties of velopharyngeal closure.

HISTORIQUE: La pharyngoplastie du sphincter est un traitement chirurgical de l'incompétence vélopharyngienne (IVP) qui a fait ses preuves. Cependant, une controverse persiste à l'égard de la contractilité des muscles transposés. Les publications n'abordent pas du tout l'influence du dynamisme du sphincter sur les issues du discours. OBJECTIF: Déterminer si la contraction active du sphincter après une pharyngoplastie du sphincter influe sur la fermeture vélopharyngienne, l'émission nasale et l'hypernasalité. MÉTHODOLOGIE: Les chercheurs ont procédé à une analyse prospective des patients présentant une IVP après la réparation d'une fente palatine qui ont subi une pharyngoplastie du sphincter exécutée par un seul chirurgien. Ils ont procédé à une nasendoscopie vidéo et à une vidéofluoroscopie avant l'opération, puis trois et 12 mois après l'opération. Ils ont examiné 18 patients consécutifs ayant une fente palatine, accompagnée ou non d'une fente labiale, et une IVP. Les patients de trois à 19 ans avaient un âge moyen de 7,3 ans au moment de la première évaluation. Les chercheurs ont évalué le dynamisme de la pharyngoplastie du sphincter, le ratio de fermeture vélaire (RFV) et le mouvement des parois latérales (MPL) par nasendoscopie et vidéofluoroscopie. Ils ont également évalué l'émission nasale et l'hypernasalité au moyen de l'examen du discours perceptuel. RÉSULTATS: Pour des besoins de comparaison longitudinale, les chercheurs ont créé trois groupes, soit un groupe dynamique à trois et 12 mois (n=12), un groupe adynamique à trois mois et dynamique à 12 mois (n=4) et un groupe adynamique à trois et 12 mois (n=2). Leurs indices d'hypernasalité perçus s'étaient considérablement améliorés à trois mois (P=0,0001) et avaient continué de s'améliorer à 12 mois (P=0,03), malgré l'absence de changement de l'IPV et du MPL entre trois et 12 mois. On ne constatait pas de différence significative à un moment ou à un autre entre les trois groupes. EXPOSÉ: La pharyngoplastie du sphincter traite l'IPV de manière efficace chez des patients bien sélectionnés. Même si le RFV et le MPL étaient demeurés stables entre trois mois et un an, quatre des six sphincters adynamiques sont devenus dynamiques. Chez l'ensemble des patients, l'hypernasalité s'est atténuée de manière continue entre trois mois et un an. CONCLUSIONS: On n'a constaté aucune différence entre les sphincters dynamiques et adynamiques sur les issues du discours ou les propriétés mécaniques de la fermeture vélopharyngienne.

Prenatal counseling's effect on rates of neonatal intensive care admission for feeding problems cleft lip/palate infants.

Prenatal counseling and feeding instruction is standard at our institution for parents of cleft lip and palate patients. We studied this intervention's effect on Neonatal Intensive Care Unit (NICU) admission solely for feeding. Ten percent (2/20) of patients whose parents received counseling were admitted to the NICU for feeding issues alone compared to 21% (5/24) of the non-counseling group. Prenatal counseling and feeding instruction appears to decrease NICU admission, duration and health care costs.