Intact Palate Research Articles

Impact of upper airway obstruction management in Robin Sequence on need for myringotomy tubes

ObjectiveInvestigate an association between upper airway obstruction (UAO) management in Robin Sequence (RS) and need for bilateral myringotomy and tubes (BMT). MethodsRetrospective chart review of RS patients treated at a tertiary free-standing pediatric hospital from 1995 to 2020 was performed. Patients were grouped based on airway management: conservative, tracheostomy, tongue-lip adhesion (TLA), and mandibular distraction osteogenesis (MDO). Demographic data, cleft palate (CP) association, numbers of BMT and ear infections, and audiogram data including tympanograms were collected. One-way ANOVA and Chi-square/Fisher's exact tests were used to compare continuous and categorical data, respectively. Multivariable regression analysis was used to compare BMT rates between treatment groups. ResultsOne hundred forty-eight patients were included, 70.3 % of which had CP. Most patients (67.6 %) had at least one BMT; 29.1 % required two or more BMT. The rate of BMT was higher in patients with CP compared to those with intact palates (p = 0.003; 95 % CI 1.30–3.57) and those treated with tracheostomy (p = 0.043; 95 % CI 1.01, 2.27). Surgically managed patients were more likely to have hearing loss (67.5 % vs. 35.3 %, p = 0.017) and ear infections (42.1 % vs. 20.0 %, p = 0.014) pre-compared to post-procedure for airway management. ConclusionMost RS patients require at least 1 set of BMT. Those with CP and/or treated with tracheostomy had a higher likelihood of needing BMT. Rate of hearing loss and ear infection was higher in surgically managed RS patients. Patients with RS and overt CP require a statistically higher number of BMTs compared to those with either submucous cleft palate or intact palate.

The Hard Palate Sweep: a multiplanar 2-dimensional sonographic method for the prenatal detection of cleft palate

Prenatal diagnosis of cleft palate is challenging. Numerous 2-dimensional and 3-dimensional methods have been proposed to assess the integrity of the fetal palate, yet detection rates remain relatively low. We propose the "Hard Palate Sweep," a novel 2-dimensional method that enables clear demonstration of the entire fetal palate throughout pregnancy, in a single sweep, avoiding acoustic shadows cast by surrounding bones. This study aimed to assess the feasibility and performance of the Hard Palate Sweep, performed throughout pregnancy. This was a prospective cross-sectional study performed between 2018 and 2022 in pregnant patients referred for a routine or targeted anomaly scan between 13 and 40 weeks of gestation. The presence or absence of a cleft palate was determined using the "Hard Palate Sweep." This was compared with the postnatal palate integrity assessment. Test feasibility and performance indices, including sensitivity, specificity, and positive and negative predictive values were calculated. Offline clips were reviewed by 2 investigators for the assessment of inter- and intraoperator agreement, using Cohen's kappa formula. The study protocol was approved by the institutional ethics committee. All participating patients were informed and provided consent. A total of 676 fetuses were included in the study. The Hard Palate Sweep was successfully performed in all cases, and 19 cases were determined to have a cleft palate. Of these, 13 cases were excluded because postmortem confirmation was not performed, leaving 663 cases available for analysis. Six cases determined to have a cleft palate were confirmed postnatally. In 655 of 657 cases prenatally determined to have an intact palate, this was confirmed postnatally. In the 2 remaining cases, rare forms of cleft palate were diagnosed postnatally, rendering 75% sensitivity, 100% specificity, 100% positive predictive value, and 99.7% negative predictive value for the Hard Palate Sweep (P<.001). There was complete intra- and interoperator agreement (kappa=1; P<.0001). The Hard Palate Sweep is a feasible and accurate method for prenatally detecting a cleft palate. It was successfully performed in all attempted cases between 13 and 40 weeks of gestation. This method is reproducible, offering high sensitivity and specificity. Implemented routinely, the Hard Palate Sweep is expected to increase the prenatal detection of cleft palate.

The Effects of Gingivoperiosteoplasty and Cleft Palate Repair on Facial Growth.

Gingivoperiosteoplasty (GPP) can avoid secondary alveolar bone graft in up to 60% of patients. The effects of GPP on maxillary growth are a concern. However, palatoplasty can also negatively impact facial growth. This study quantifies the isolated effects of GPP and cleft palate repair on maxillary growth at the age of mixed dentition. A single-institution, retrospective study of all patients undergoing primary reconstruction for unilateral cleft lip and alveolus (CLA) or cleft lip and palate (CLP) was performed. Study patients had lateral cephalograms at the age of mixed dentition. Patients were stratified into four groups: CLA with GPP (CLA + GPP), CLA without GPP (CLA - GPP), CLP with GPP (CLP + GPP), and CLP without GPP (CLP - GPP). Cephalometric measurements included the following: sella-nasion-point A (SNA), sella-nasion-point B (SNB), and A point-nasion-B point. Landmarks were compared between patient groups and to Eurocleft center D data. One hundred ten patients met inclusion criteria: seven CLA - GPP, 16 CLA + GPP, 24 CLP - GPP, and 63 CLP + GPP patients. There were no significant differences in SNA, SNB, and A point-nasion-B point between CLA + GPP and CLA - GPP, or between CLP + GPP and CLP - GPP groups. In patients who did not receive GPP, SNA was significantly lower in patients with a cleft palate compared with patients with an intact palate ( P < 0.05). There were no significant differences in SNA or SNB of CLP - GPP or CLP + GPP groups when compared with Eurocleft data. When controlling for the effects of cleft palate repair, GPP does not appear to negatively affect midface growth at the age of mixed dentition. Therapeutic, III.

Cleft palate morphology, genetic etiology, and risk of mortality in infants with Robin sequence.

Robin sequence (RS) has many genetic and nongenetic causes, including isolated Robin sequence (iRS), Stickler syndrome (SS), and other syndromes (SyndRS). The purpose of this study was to determine if the presence and type of cleft palate varies between etiologic groups. A secondary endpoint was to determine the relationship of etiologic group, cleft type, and mortality. Retrospective chart review of patients with RS at two high-volume craniofacial centers. 295 patients with RS identified. CP was identified in 97% with iRS, 95% with SS, and 70% of those with SyndRS (p < .0001). U-shaped CP was seen in 86% of iRS, 82% with SS, but only 27% with SyndRS (p < .0001). At one institution, 12 children (6%) with RS died, all from the SyndRS group (p < .0001). All died due to medical comorbidities related to their syndrome. Only 25% of children who died had a U-shaped CP. The most common palatal morphology among those who died was an intact palate. U-shaped CP was most strongly associated with iRS and SS, and with a lower risk of mortality. RS with submucous CP, cleft lip and palate or intact palate was strongly suggestive of an underlying genetic syndrome and higher risk of mortality.

Palatal Lift Appliance in a Case of Palatal Incompetence Secondary to Motor Neurone Disease

Velopharyngeal deficiencies are challenging conditions to manage, often requiring input from a number of different specialties including restorative dentistry. Palatal incompetence, that is, the inability of a structurally intact palate to elevate and close the nasopharynx from the oropharynx can result in hypernasality and air escape, compromising speech sounds as well as causing swallowing difficulty. The palatal lift appliance is a prosthesis designed to elevate the palate to aid velopharyngeal closure, and has been used to manage patients with neurological disorders affecting the palate. This report presents such management in a patient with motor neurone disease. CPD/Clinical Relevance: Dentists should be integral in the management of patients with motor neurone disease to help maintain oral health and prevention of dental disease, but also to provide speech prostheses and liaise with speech and language therapists. Patients with motor neurone disease may present to a generalist for routine dental care, and GDPs should be aware of the challenges of managing the dental work for this group of people.

Velopharyngeal Incompetence in Children With 22q11.2 Deletion Syndrome: Velar and Pharyngeal Dimensions.

The surgical management of velopharyngeal incompetence (VPI) in children with 22q11.2 deletion syndrome (22q11.2 DS) is challenging. There are numerous approaches and children often undergo more than one operation. Our aim was to develop a method using images from routine lateral videofluoroscopy to study the dimensions of the velopharynx in this cohort.We analyzed 22 pre-operative lateral videofluoroscopy recordings of children with 22q11.2 DS and VPI. Fourteen had a submucous cleft palate (SMCP) and 8 had no obvious palatal abnormality but who were subsequently labelled as having an occult submucous cleft palate (OSMCP). The control data were 10 historic records of children with cleft lip and an intact palate. The authors identified key points on radiographs of the velum at rest and when elevated to measure the total velar length, functional velar length and pharyngeal depth and compared them ratiometrically.The intra-observer reliability was > 0.9 whereas the inter-observer reliability was > 0.74. The velopharyngeal depth/total velar length was significantly greater in 22q11.2 DS than the control group P < 0.001. There was no difference between SMCP and OSMCP patients, P = 0.556. There was no difference in the functional velar length/total velar length between 22q11.2 DS and controls (P = 0.763).In this study, the authors demonstrate a reliable method to gain useful ratiometric measurements of the velopharynx. This may help with treatment planning. Children with 22q11.2 DS and VPI have a larger velopharyngeal depth/total velar length ratio that may explain some of the difficulty in management.

A new two-dimensional sonographic approach to the assessment of the fetal hard and soft palates.

Facial clefts are among the most common congenital defects. Ultrasound (US) imaging of secondary fetal palate, especially the detection of isolated defects, remains challenging. Currently described two-dimensional (2D) and three-dimensional methods are technically demanding and impractical for application during routine fetal anatomy evaluation. As an adjunct method, magnetic resonance imaging can provide additional information but has its limitations. We present a novel 2D US approach using axial and sagittal planes to evaluate the fetal palate and demonstrate the main differences between an intact palate, isolated cleft palate, and a cleft lip with cleft palate.

Cleft Palate

A cleft of the palate represents one of the most common congenital anomalies of the craniofacial region. Palatal clefting can occur in combination with a cleft of the lip and alveolus or as an isolated finding and can vary significantly in severity. The intact palate is a structure that separates the oral and nasal cavities, and the function of the palate is to close off the nasal cavity during deglutition and to regulate the flow of air between the nose and mouth during speech production. An unrepaired cleft palate can thus result in nasal regurgitation of food and liquid, early feeding difficulties, and impaired speech development. The goals of surgical repair are to restore palatal integrity by closing the cleft defect and repairing the musculature to allow for normal function during speech. The secondary goal of cleft palate repair is to minimize deleterious effects on growth of the palate and face, which can be impacted by standard surgical interventions. This review describes two of the most commonly performed cleft palate repair techniques in use today, as well as highlighting special anatomic considerations, summarizing perioperative care, and reviewing postoperative complications and their management. This review contains 11 figures, 2 videos, 3 tables and 63 references Key words: cleft, cleft team, Furlow, orofacial, oronasal fistula, palatoplasty, speech, submucous cleft, velopharyngeal insufficiency

Cleft Palate

A cleft of the palate represents one of the most common congenital anomalies of the craniofacial region. Palatal clefting can occur in combination with a cleft of the lip and alveolus or as an isolated finding and can vary significantly in severity. The intact palate is a structure that separates the oral and nasal cavities, and the function of the palate is to close off the nasal cavity during deglutition and to regulate the flow of air between the nose and mouth during speech production. An unrepaired cleft palate can thus result in nasal regurgitation of food and liquid, early feeding difficulties, and impaired speech development. The goals of surgical repair are to restore palatal integrity by closing the cleft defect and repairing the musculature to allow for normal function during speech. The secondary goal of cleft palate repair is to minimize deleterious effects on growth of the palate and face, which can be impacted by standard surgical interventions. This review describes two of the most commonly performed cleft palate repair techniques in use today, as well as highlighting special anatomic considerations, summarizing perioperative care, and reviewing postoperative complications and their management. This review contains 11 figures, 2 videos, 3 tables and 63 references Key words: cleft, cleft team, Furlow, orofacial, oronasal fistula, palatoplasty, speech, submucous cleft, velopharyngeal insufficiency

The New Anatomical Classification System for Orbital Exenteration Defect.

The unique anatomy of the orbita and the different behavior of each malignant tumor cause us to perform the various types of orbital exenteration that yields to varying defect each of which has own specific demands in terms of the reconstruction. Current classification of orbital exenteration defects seems not to be adequate to provide detailed description. This study reviews 50 exenteration defects to offer a more effective anatomical classification system. Over a 15 years period, 50 orbital exenteration defects in 47 patients were reconstructed. Defects were categorized according to the resected orbital wall, dura, and ethmoid resection. If the maxillectomy was performed, A or B was added to define the type of maxillectomy as partial (intact palate) or total maxillectomy, respectively. According to these criteria, 4 types of defect patterns were determined including Type 0 (n = 5) with intact orbital wall, Type I (n = 9) with sino-orbital fistula, Type II (n = 4) with crania-orbital fistula with intact dura, Type III (n = 6) with crania-orbital fistula associated with dura defect, and Type IV (n = 8) with cranio-nasal-orbital fistula. There were 12 partial (A) and 6 total maxillectomy (B) defects along with the orbital exenteration. There was no major complication except one. The minor wound-healing problems occurred in 7 patients. Nine patients (19%) used prosthesis. Twenty-two (46.8%) patients chose a patch to cover the area. The remaining 16 patients were not able to use any type of prosthesis because of the reconstruction methods. The authors believe that the authors' anatomical classification system provides more precise description of the defect which eventually enhances the success rate of both reconstruction and resection.

Promoting Breast Milk Nutrition in Infants With Cleft Lip and/or Palate

To the Editor: Burca and colleagues1 are to be congratulated for their efforts to promote breast milk for infants with cleft lip and/or cleft palate (CL/P). They nicely outline the important benefits of breast milk for this vulnerable population, and we commend the authors for their position that human milk “should be central to the care of these infants.” However, we are concerned with several recommendations related to how human milk is delivered. First and most concerning is the statement by the authors that “breastfeeding usually/sometimes works well” for an infant with a cleft palate and recommendations in this regard (see Table 1 in Burca et al1). We caution the authors to avoid giving the impression that children with isolated cleft palate are likely to feed effectively or fully at the breast. The vast majority of children with a cleft palate do not feed well at the breast and require an adaptive feeding method. Efforts should focus on ways to improve availability of breast milk for these patients, beginning with support and education of parents during the prenatal visit, and continuing through the perinatal period and infancy. The advice that an infant with a cleft palate can breastfeed shows a misunderstanding of the sucking mechanics of an infant with a cleft palate, which is distinct from an infant with a cleft lip. As the authors point out, an infant with a cleft lip and intact palate will most likely be able to feed at the breast effectively. A lactation consultant can assist the mother with positioning to assist with lip seal, which often, but not always, allows breastfeeding. With a cleft palate, the infant is unable to generate sufficient intraoral suction to breastfeed effectively. Regardless of size or location of the cleft palate (as stated in the article), the presence of the cleft palate prohibits the infant from isolating the oral cavity from the nasal sinuses, thereby preventing the infant from developing the negative pressure vacuum needed to draw milk from the breast.2 There are only extremely rare cases where the mother and infant can work together so the infant may feed directly and fully from the breast. Second, the authors state that “infants with CL/P consume less milk, gain less weight, and measure lower on growth curves” than infants without CL/P. Nutrition in children with oral clefts has not been well studied. There are many potential confounders that impact the published studies suggesting suboptimal growth in children with clefts, and some studies have shown that children with clefts do have adequate growth whether or not the palate is involved.3 Given the mostly small or anecdotal studies in this field, the reason for poor growth may stem from infants who are not given appropriate feeding methods to compensate for the cleft or have other malformations or medical problems leading to inadequate nutritional status. Most infants with clefts who are managed with the proper feeding tools grow at the same rate as an infant without a cleft. Numerous adaptive bottles are available for infants with clefts, which assist with milk flow so the infant can feed well without needing to create a vacuum. Assistive devices may also be used at the breast but require careful instruction to ensure adequate milk intake. If there is a delay in diagnosing the cleft palate or implementing the correct feeding adaptation, there will be a risk of infant weight loss and diminishing maternal milk supply if milk expression is not started quickly after birth. Lastly, the authors state that “feeding human milk by bottle is the preferred approach if direct breastfeeding is not possible.” This is true for infants in high-resource settings. In low-resources settings, however, the World Health Organization and United Nations Children's Fund4,5 recommend cup feeding for infants unable to breastfeed as the safest tool. Because 94% of children born with an oral cleft are born in a developing country,6 this advice should be included in guidance on adaptive equipment to support feeding. We do applaud the authors for their efforts but believe it is imperative that the clinical community clearly understand the differences in the potential for infants with various types of clefts to feed at the breast. What does not differ is the ability of these infants to benefit from breast milk feeding. Future efforts should focus on interventions to support mothers in providing this valuable source of nutrition to their infants. Emily Gallagher, MD, MPH [email protected] Christy McKinney, PhD, MPH Robin Glass, MS, OTR, IBCLC Seattle Children's Hospital Craniofacial Center,Seattle, Washington

Retraction of the Soft Palate During a Modified Hynes Pharyngoplasty: A Technical Note.

Surgical management of velopharyngeal insufficiency by construction of sphincter pharyngoplasty is well described in the medical literature. Hynes advocated splitting an intact soft palate when it would be helpful for better exposure of the posterior pharyngeal wall for flap inset. We describe a modification to the Hynes pharyngoplasty whereby the soft palate is retracted upward, giving the operator unrestricted surgical access to the salpingopharyngeus muscles and their overlying mucosa. This allows the surgeon to raise and inset the flaps, as described by Hynes, without the need to divide the soft palate. The retraction catheters avoid the need for splitting a soft palate, which has been optimized by either a Furlow or soft palate re-repair in the past, avoiding unnecessary compromise of the integrity and architecture of the soft palate.

Defining failure and its predictors in mandibular distraction for Robin sequence.

Robin sequence (RS) is defined as the triad of micrognathia, glossoptosis and airway obstruction. A popular surgical treatment is mandibular distraction osteogenesis (MDO). In this study, it is demonstrated that the associated variables change, dependent on the manner in which failure is defined. These multiple failure outcomes are used to construct a scoring system to predict MDO failure. A retrospective database of neonatal MDO patients was constructed. Failure outcomes studied included tracheostomy; a decrease in the apnea-hypopnea index (AHI) but an AHI >20; and death. A combination of bivariate and regression analysis was used to produce significantly associated variables and a scoring system. Statistical analysis demonstrated the association of gastroesophageal reflux; age >30 days; neurologic anomaly; airway anomalies, other than laryngomalacia; an intact palate; and pre-operative intubation on the outcome variables studied. Multiple scoring systems were produced with reasonable sensitivity, specificity, and positive and negative predictive value. When reporting surgical outcomes of MDO in the setting of RS, it is important to consider the AHI as well as avoidance of tracheostomy as an outcome variable. Incomplete amelioration of AHI accounts for half of the patients with a problem after MDO. The predictive scores presented will be used and validated on a larger, prospectively collected dataset.

Cellular and Molecular Mechanisms of Palatogenesis.

Palatogenesis involves the initiation, growth, morphogenesis, and fusion of the primary and secondary palatal shelves from initially separate facial prominences during embryogenesis to form the intact palate separating the oral cavity from the nostrils. The palatal shelves consist mainly of cranial neural crest-derived mesenchymal cells covered by a simple embryonic epithelium. The growth and patterning of the palatal shelves are controlled by reciprocal epithelial-mesenchymal interactions regulated by multiple signaling pathways and transcription factors. During palatal shelf outgrowth, the embryonic epithelium develops a "teflon" coat consisting of a single, continuous layer of periderm cells that prevents the facial prominences and palatal shelves from forming aberrant interepithelial adhesions. Palatal fusion involves not only spatiotemporally regulated disruption of the periderm but also dynamic cellular and molecular processes that result in adhesion and intercalation of the palatal medial edge epithelia to form an intershelf epithelial seam, and subsequent dissolution of the epithelial seam to form the intact roof of the oral cavity. The complexity of regulation of these morphogenetic processes is reflected by the common occurrence of cleft palate in humans. This review will summarize major recent advances and discuss major remaining gaps in the understanding of cellular and molecular mechanisms controlling palatogenesis.

Midfacial Growth and Surgically Induced Growth Arrest

INTRODUCTION: Midfacial growth arrest is a common occurrence in patients who have had cleft palate operations but surprisingly, children whose cleft palates have not been surgically repaired have nearly normal midfacial growth.1,2 The mechanisms responsible for surgically induced midfacial growth arrest are unknown. We hypothesized that the midpalatal suture complex acts as a midfacial growth center. We examined the molecular signals and mechanical forces involved in forming this presumptive growth center then tested whether surgical disruption of the suture complex in neonatal mice contributed to growth arrest of the midface. MATERIALS AND METHODS: The distribution of Wnt and Hedgehog responsive cells during midpalatal suture development were mapped using Axin2LacZ/+, Gli1LacZ/+, and Ptc1LacZ/+ mice. FE modeling was used to generate strain maps of the forces acting on palate development and on the palate after surgery. Micro-CT, histology, qRT-PCR, and immunohistochemistry were used to quantify changes. RESULTS: The midpalatal suture forms between two intramembranous bones that in response to suckling and tongue forces, transform into endochondral growth sites, similar to the growth plates found on long bones. The prenatal distribution of Hedgehog and Wnt responsive cells suggests that this transformation involves both signaling pathways. The resulting cartilage growth plates of the midpalatal suture complex act as growth centers and mucoperiosteal denudation of the site in postnatal animals results in a growth arrest and premature fusion of the suture (Figures 1 and 2).Figure 1: Micro-CT of intact palate; yellow bracket demarcates the midpalatal suture.Figure 2: Micro-CT of injured palate.CONCLUSION: The midpalatal suture complex develops between the palatal processes that begin as intramembranous bones but transform into endochondral growth plates in response to mechanical forces. Raising a flap disrupts these growth plates and is sufficient to arrest growth. Although the suture complex regenerates, subsequent skeletal growth is compromised. These data strongly suggest that disruption of suture complexes, which have intrinsic growth potential, is a contributor for midfacial growth arrest associated with palatal reconstruction.

Obstructive sleep apnea syndrome in children with 22q11.2 deletion syndrome after operative intervention for velopharyngeal insufficiency.

Introduction: Surgical treatment of velopharyngeal insufficiency (VPI) in 22q11.2 deletion syndrome is often warranted. In this patient population, VPI is characterized by poor palatal elevation and muscular hypotonia with an intact palate. We hypothesize that 22q11.2 deletion patients are at greater risk of obstructive sleep apnea (OSA) after surgical correction of VPI, due, in part, to their functional hypotonia, large velopharyngeal gap size, and the need to surgically obstruct the velopharynx.Methods: We performed a retrospective analysis of patients with 22q11.2 deletion syndrome treated at a tertiary pediatric hospital between the years of 2002 and 2012. The incidence of VPI, need for surgery, post-operative polysomnogram, post-operative VPI assessment, and OSA treatments were evaluated.Results: Forty-three patients (18 males, 25 females, ages 1–14 years) fitting the inclusion criteria were identified. Twenty-eight patients were evaluated by speech pathology due to hypernasality. Twenty-one patients had insufficient velopharyngeal function and required surgery. Fifteen underwent pharyngeal flap surgery, three underwent sphincter pharyngoplasty, two underwent Furlow palatoplasty, and one underwent combined sphincter pharyngoplasty with Furlow palatoplasty. Of these, eight had post-operative snoring. Six of these underwent polysomnography (five underwent pharyngeal flap surgeries and one underwent sphincter pharyngoplasty). Four patients were found to have OSA based on the results of the polysomnography (average apnea/hypopnea index of 4.9 events/h, median = 5.1, SD = 2.1). Two required continuous positive airway pressure (CPAP) due to moderate OSA.Conclusion: Surgery is often necessary to correct VPI in patients with 22q11.2 deletion syndrome. Monitoring for OSA should be considered after surgical correction of VPI due to a high occurrence in this population. Furthermore, families should be counseled of the risk of OSA after surgery and the potential need for treatment with CPAP.

Signaling networks in palate development

Palatogenesis, the formation of the palate, is a dynamic process regulated by a complex series of context-dependent morphogenetic signaling events. Many genes involved in palatogenesis have been discovered through the use of genetically manipulated mouse models as well as from human genetic studies, but the roles of these genes and their products in signaling networks regulating palatogenesis are still poorly known. In this review, we give a brief overview on palatogenesis and introduce key signaling cascades leading to formation of the intact palate. Moreover, we review conceptual differences between pathway biology and network biology and discuss how some of the recent technological advances in conjunction with mouse genetic models have contributed to our understanding of signaling networks regulating palate growth and fusion. For further resources related to this article, please visit the WIREs website. The authors have declared no conflicts of interest for this article.

The transpalatal approach to repair of congenital Basal skull base cephaloceles.

Basal skull base herniations, including meningoceles and encephaloceles, are rare and may present with characteristic facial and neurologic features. The traditional craniotomy approach has known morbidity, and nasal endoscopy may not allow for control of large posterior basal defects, especially in newborns. We present two cases of successful repair of basal transsphenoidal meningoceles using an oral-transpalatal approach. The first patient with an intact palate presented with respiratory distress, and a palatectomy was performed for access to the skull base. The second patient had a large basal herniation that was reduced through a congenital midline cleft palate, and a calvarial bone graft was used to repair the defect. A literature search revealed 10 previous successful cases using the transpalatal repair, which allows for excellent access, low morbidity, and a team-oriented method to skull base surgery.

Three‐dimensional ultrasound demonstration of the fetal palate in high‐risk patients: the accuracy of prenatal visualization

The aim of this research was to evaluate the ability of three-dimensional (3D) ultrasound for demonstrating the palate of fetuses at high risk for cleft palate. Fifty-seven fetuses at high risk for cleft palate were referred for specialist ultrasound at 12-40 weeks' gestation. A detailed assessment of palate was made using both two-dimensional and 3D ultrasounds on the axial plane. Antenatal diagnoses were compared with postnatal findings. Cleft palate was suspected in 13 (22.8%); a normal palate was demonstrated in 38 (67%), and in six (10.2%), the palate view could not be obtained. Mean gestational age at the first visit was 27 weeks 6 days (range of 12-40 weeks 3 days). Examination after delivery revealed that one of the 38 fetuses with presumed normal palate had a cleft hard palate, and one had a cleft soft palate (false negative 5%). Among the 13 fetuses with suspected cleft palate, three had an intact palate (false-positive 23%). Sensitivity, specificity, positive predictive value, and negative predictive value of detection of palatal clefts were 71.4%, 91.9%, 62.5%, and 94.4%, respectively. Using 3D technology, we diagnosed a cleft palate in 83% of high-risk cases, with 5% false negative. 3D technology might produce some technical artifacts resulting in a 23% false-positive rate.

Visual Diagnosis: Multiple Joint Contractures in a Male Newborn

1. Lincy Cherian, MD* 2. Jacob J. Rosenberg† 1. *Pediatric Chief Resident, Nassau University Medical Center, East Meadow, NY. 2. †Associate Program Director, Department of Pediatrics, Nassau University Medical Center; Assistant Professor of Clinical Pediatrics, SUNY at Stony Brook, West Hempstead, NY. A male infant is born at 35 weeks' gestation to a 38-year-old G3P2 woman and is admitted to the neonatal intensive care unit (NICU) because of multiple joint contractures (Fig. 1 ). The mother received appropriate prenatal care during pregnancy, during which multiple fetal sonograms revealed a prominent renal pelvis, poor fetal motion and tone, persistent extension of the arms, flexion of the knees, and extension of one ankle. However, maternal morbid obesity limited the ability to achieve full-quality images. A maternal serum screen suggested that the fetus had trisomy 18, but the mother refused additional investigative studies, including amniocentesis. Figure 1. A term male newborn who has multiple joint contractures. Apgar scores are 8 at 1 minute and 8 at 5 minutes. Vital signs are normal for age, and the weight, length, and head circumference are within the 25th percentile for gestational age. Physical examination reveals a flat, open anterior fontanelle; normal-set ears; intact palate; normal cardiovascular, lung, and abdominal examination findings; and normal male genitalia. The infant's hips are rotated externally, the knees are flexed with limited range of motion (ROM) (Fig. 2 ), the …