Maxillomandibular Fusion Along with Fusion of Dorsal Surface of the Tongue to the Hard Palate-A Case Report.

RATIONALE: This case report presents a rare combination of congenital anomalies in an otherwise healthy male infant born at 36 weeks. The infant was diagnosed with congenital maxillomandibular synechia, ectrodactyly, and ankyloglossia superior syndrome (ASS). PATIENT CONCERNS: Inability to open the mouth completely, feeding challenges, and a cleft palate. The infant was stabilized through successful positive pressure ventilation via a face mask at birth and enteral feeding was initiated via a feeding gastrostomy. EXAMINATION: Diagnostic tests revealed a midline palatal cleft, hypoplastic jaws, persistent metopic suture, and a bony fusion at the midline. TREATMENT: Sectioning of the bony spur along the midline and achieving a mouth opening of 2 cm post-manipulation. The patient is under follow-up, with future treatment plans including cleft palate correction at 12 months and potential frontomandibular and lower jaw advancement depending on growth trajectories. TAKEAWAY LESSONS: This case underscores the complexity of managing multiple congenital anomalies and the need for individualized treatment plans.

The role of MEG3 in the proliferation of palatal mesenchymal cells is related to the TGFß/Smad pathway in TCDD inducing cleft palate.

Cleft palate (CP) is a common birth defect with a high incidence of occurrence in humans. The 2, 3, 7, 8-tetrachlorodibenzo-p-dioxin (TCDD) is a highly toxic halogenated aromatic hydrocarbon, with a strong CP effect on mice. Increasing recent evidences have shown that long-noncoding RNAs (lncRNAs) play an important role in several diseases, including CP. However, there is a paucity of studies on the role of lncRNA MEG3 in the occurrence and development of TCDD-induced CP. In this study, the relationship between MEG3 and the proliferation of palatal mesenchymal cells and the underlying molecular mechanism were studied by establishing fetal CP with TCDD (64 µg/kg) in C57BL/6N mice. The results revealed that MEG3 was highly expressed during the critical period of CP formation and that the fetal mesenchymal proliferation was significantly inhibited at certain critical periods in the mice receiving TCDD. In addition, we noted a possibility of a crosstalk between MEG3 and the TGF-ß/Smad pathway, such that the inhibition of the TGF-ß/Smad pathway was induced by TCDD. Cumulatively, our study suggests that TCDD-induced CP may be caused by MEG3 inhibition of the proliferation of palatal mesenchymal cells involving the TGFß/Smad pathway, which may provide a novel perspective to understand the pathogenesis of CP.

Correlations between dental wear and oral cavity characteristics: Mandibular torus, palatine torus, and oral exostoses.

OBJECTIVE: This study evaluates the correlations amongst mandibular torus, palatine torus, oral exostoses to dental wear/loss and temporomandibular damage. METHODS: The sample consists of 504 skulls from the Hamann-Todd Osteological Collection; 223 African American and 281 European Americans aged between 30 and 80 years. The sample was analyzed using Pearson's Chi-square for significance of sex, age, ancestry, and wear as well as the interactions between the demographic variables and the presence of mandibular torus, palatine torus and oral exostoses. RESULTS: Wear was statistically significant by age and sex but not ancestry. The maxillary exostoses varied significantly by age, ancestry and wear but not sex. Mandibular torus frequencies varied significantly by wear, sex and ancestry. The palatine torus varied significantly across wear groups, sex and ancestry. DISCUSSION: The etiology of nonmetric oral cavity characteristics, mandibular torus, palatine torus and oral exostosis, is complex. The degree to which traits' presence and expression is the result of genetic and environmental interactions is not fully understood. More than age, sex or ancestry, the degree of dental wear and tooth loss influences the presence and expression of the oral cavity traits. The sample can be characterized as the presence of exostoses in higher frequencies in young African American males with little tooth loss. Males of both ancestral groups with heavy wear have higher frequencies of mandibular tori than females. The palatine torus is more common in edentulous European American females.

The Effect of Surgical Timing on 30-Day Outcomes in Cleft Palate Repair.

BACKGROUND: The optimal age for cleft palate repair continues to be debated, with little discussion of surgical risk related to operative timing. This study of 3088 cleft palate patients analyzed the impact of surgical timing on perioperative and 30-day postoperative outcomes. METHODS: Primary cleft palate repairs were identified in the National Surgical Quality Improvement Program database from 2012 to 2015. Data were combed for total postoperative complications, rates of readmission and reoperation, operating room time, and length of stay. Bivariate analyses were performed comparing 3-month periods from months 6 to 18, and months 0 to 5, 18 to 23, 24 to 29, and 30 to 59. RESULTS: Despite a higher proportion of isolated soft palate closure, children operated on before 6 months had a higher complication rate than children at other ages (7.1 percent versus 3.2 percent; OR, 2.4; p = 0.04), and higher rates of both readmission (3.6 percent versus 1.4 percent; OR, 3.6; p = 0.02) and reoperation (2.4 percent versus 0.5 percent; OR, 4.7; p = 0.04). There were no differences in short-term outcomes for any other age group younger than 5 years, and no differences in hospital length of stay among any age groups. CONCLUSIONS: The authors' findings suggest a relative contraindication to operation before 6 months. As there were no differences between any other age groups, long-term speech optimization should continue to be the primary consideration for operative planning. These findings improve the current rationale for palatoplasty timing, and can aid surgeons and parents in the surgical decision-making process. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Giant torus mandibularis causing submandibular duct obstruction and sialadenitis.

Torus mandibularis is a benign osseous overgrowth arising from the lingual surface of the mandible. It is a common, incidental finding on imaging due to its relatively high prevalence. In the majority of cases, mandibular tori are asymptomatic. We report a novel presentation of a giant torus mandibularis causing bilateral obstruction of the submandibular ducts and consequent sialadenitis. Our patient presented with progressive pain centered in the floor of his mouth and had bilateral submandibular glandular enlargement on exam. Computed tomography showed a giant right torus mandibularis, which was causing obstruction and dilation of the bilateral submandibular ducts. Although conservative management was attempted, he ultimately underwent surgical resection of his torus with symptomatic improvement. This patient highlights a novel complication of torus mandibularis and illustrates successful treatment. Though not previously described, this complication may be underreported and should be considered in the appropriate clinical setting.

Incidence and morphology of the incisive suture in CT scanning of young children and human fetuses.

PURPOSE: Incisive suture is a suture classically described on the oral face of the palate in fetuses and young children. The aim of our study was to describe the evolution of the incisive suture in human fetuses and to evaluate the incidence of this suture in a population of young children under 4 years, to determine if there is a possibility of improving the anterior growth of the maxilla, by stimulation of this suture. METHODS: One hundred and thirty CT scan images of patients aged from birth to 48 months have been studied and nine fetal palates aged from 18 to 26 weeks of development, have been scanned using high-resolution X-ray micro-computed tomography RESULTS: The CT scan images of patients showed that an incisive suture was present in 33/130 cases (25,4%). All the patients with a suture were under 2 years old. The fetal palate study showed that the suture was present in the inferior aspect of the palate (oral cavity) in all cases. The incisive suture increased from 18 to 24 weeks. At 26 weeks it stopped growing although the intercanine length increased. Considering the closure of the suture in a vertical plane, our study on fetuses has shown that the incisive suture is closing from its superior side (nasal side) to its inferior side. CONCLUSIONS: Considering all these results it appears to us that the incisive suture is partially ossified after birth, it cannot be stimulated by orthodontic appliances.

Hard palate defect repair by using haired angularis oris axial pattern flaps in dogs.

OBJECTIVE: To describe the repair of large hard palate defects with a haired angularis oris axial pattern flap (HAOF) and to report the postoperative outcome. STUDY DESIGN: Anatomical cadaver study and short case series. ANIMALS: One cadaver and three dogs with neoplasia of the caudal hard palate. METHODS: Large caudal and central hard palate defects were reconstructed by using the HAOF. The flap, composed of full-thickness skin and buccal mucosa, was rotated at the base of the angularis oris artery. The haired surface of the skin flap was placed facing into the oral cavity. RESULTS: Reconstruction of defects extending as far rostral as the maxillary canine tooth were feasible on the cadaver. The flap in dog 1 healed without complication and was intact at day 649 after surgery. Two minor areas of dehiscence were noted in dog 2, without further evidence of complications at 1331 days after surgery. Dog 3 had upper respiratory obstructive noise due to swelling immediately after surgery. These signs resolved, but local recurrence of the tumor prompted euthanasia at day 86. CONCLUSION: Anatomical studies provide evidence that the HAOF can be used to reconstruct caudal and central hard palate defects extending to the maxillary canine teeth. Its clinical use led to successful closure of such defects in three dogs. CLINICAL SIGNIFICANCE: HAOF provides an alternative for reconstruction of large defects of the caudal and central hard palate.

Single median maxillary central incisor syndrome and variant in SMO gene associated with SHH pathway.

Solitary median maxillary central incisor syndrome (SMMCI) is a rare congenital oronasal-dental midline anomaly. The aim of this paper is a presentation of a patient with SMMCI without other visible dentofacial anomalies, with a potentially new molecular etiology consisting of a gene-gene reaction and conservative therapeutic approach to nasal obstruction. Potentially pathogenic variants in the SMO gene (p.Gly422Glu) and in P2RY13 gene (p.Trp205*) inherited from the probant's father, and in the PLD2 gene (p.Gln319fs), inherited from the mother were found. A multidisciplinary approach is necessary for the management of patients with SMMCI, including a genetic consultation with genetic tests.

Unique air inclusions within the nasopalatine duct indicating its presence radiographically: a case presentation.

The anterior maxilla is characterized by the nasopalatine canal that originates bilaterally from the anterior nasal floor, subsequently fuses, and terminates at the incisive foramen in the anterior palate. Embryologically, this structure forms within the primary palate, and contains the neurovascular bundle, but also continuous epithelialized bands. The latter, termed nasopalatine ducts, usually degenerate and/or obliterate before birth. However, in some individuals, the ducts may remain partially or completely patent. The present case report describes for the first time in the literature a rare finding of air inclusions within the anatomical area of the nasopalatine canal indicating the presence of a nasopalatine duct as visualized with cone beam computed tomography. The patient was asymptomatic and the radiographic findings were seen incidentally. An endoscopic inspection of the anterior nasal cavities confirmed the presence of the nasal openings of the partially patent nasopalatine ducts.

Patulous Eustachian tube and palatine defect in a Dachshund with chronic unilateral otitis externa and otitis media.

BACKGROUND: Patulous Eustachian tube (pET) is a rare dysfunction of the Eustachian tube described in humans. It is characterized by failure of the ET to close, resulting in unrestricted passage of air, sound and material between the nasopharynx and the middle ear. OBJECTIVE: To report a case of pET associated with otitis in a dog. ANIMAL: A 6-year old-female spayed Dachshund dog. METHODS AND MATERIALS: Otoscopic examination, cytological evaluation, culture and susceptibility, computerized tomography (CT), video-otoscopic flushing and surgery. RESULTS: Left ear otoscopic examination revealed erythema, purulent frothy discharge, ceruminous gland hyperplasia, stenosis and a partial tear of the tympanum. Cytological evaluation from the left external canal showed neutrophils, macrophages, rods and cocci. Aerobic culture showed predominantly multidrug-resistant Pseudomonas aeruginosa. The CT findings of the left ear included chronic changes in the external canal, marked lysis of the tympanic bulla and marked dilation of the ET. During video-otoscope flushing, saline drained through the mouth. Bilateral incomplete hypoplasia of the soft palate was noted. Total ear canal ablation and bulla osteotomy with ET dissection were curative. Histopathological findings were compatible with chronic otitis externa (OE) and media. CONCLUSION AND CLINICAL IMPORTANCE: To the best of the authors' knowledge, this is the first case of pET described in animals. The ET dysfunction and palatine defect were likely the cause of the otitis in this dog. Clinicians should investigate pET in animals with signs of OE characterized by frothy liquid and food fragments in the ear canal in addition to sneezing after drinking water.

The use of torus palatinus in the identification of unknown skeletal remains.

Torus palatinus (TP) is a benign, bony outgrowth located on the hard palate. Variation in this trait occurs, ranging from absent to a protuberance several millimeters in length. If a TP becomes too large, it can interfere with daily activities and thus warrant medical attention. This case utilised the unique morphology of a TP after surgical invention to identify the unknown skeletal remains of a woman missing for six years. Even though there is still much unknown regarding the aetiology, occurrence and prevalence of TP, this study demonstrates the usefulness of anatomical variants in the identification of unknown skeletal remains.

Torus palatinus / Torus Palatinus

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Supernumerary incisive canals in a cadaver: a rare anatomical variation.

We present a rare anatomical variation in which an additional incisive canal was observed during the routine dissection of the palatal region in a cadaver. The diameter of the incisive and additional incisive canals measured 4.59 mm and 0.91 mm, respectively. Even though this variation is rare, oral surgeons should be aware of such anatomical variations for a better understanding.

Torus mandibularis affects the severity and position-dependent sleep apnoea in non-obese patients.

OBJECTIVE: Various anatomical structures of upper airway and physical differences are known to be risk factors for obstructive sleep apnoea (OSA). Torus mandibularis is a structure that can appear on the inside of the mandible. Therefore, it is possible for tori to influence airway volume by occupying the space for tongue and cause sleep apnoea. The purpose of this study is to investigate the effect of torus mandibularis on the severity of OSA as one of the craniofacial risk factors. DESIGN: Retrospective case-control study. SETTING: University-based tertiary medical centre. PARTICIPANTS: Adult patients over 19-years-old who visited outpatient clinics with complaints of sleep-disordered breathing symptoms between January 2010 and December 2017 were investigated. MAIN OUTCOME MEASURES: The presence of torus mandibularis in oral cavity was confirmed by physical examination or CT image. We analysed demographic findings including age, sex, medical history, previous operation history, physical findings of upper airway and result of polysomnography. To evaluate the effect of torus mandibularis on OSA, polysomnography data of the two groups according to presence or absence of torus mandibularis were compared and analysed. RESULTS: Two-hundred and thirty-two OSA patients with BMI <25 were divided into two groups, according to either the presence or absence of torus mandibularis. We analysed 138 patients of control group and 94 of torus mandibularis group. Apnoea-hypopnoea index (AHI) was 18.8 ± 14.9 in control group and 25.1 ± 18.4 in torus mandibularis group (P = 0.006). Respiratory disturbance index (RDI) was 23.1 ± 14.7 in control group and 27.9 ± 18.4 in torus mandibularis group (P = 0.035). Supine AHI showed 26.6 ± 20.3 in control group and 32.5 ± 22.6 in torus mandibularis group (P = 0.039). Patients with torus mandibularis had a trend of increase in proportion according to the severity of sleep apnoea, such as AHI (P = 0.007) or RDI (P = 0.034). CONCLUSIONS: We newly found that the presence of torus mandibularis affects not only severity of OSA and also position-dependent OSA. These results support the necessity of torus mandibularis evaluation in OSA patients, and further study is also required to investigate its consequence in the surgical outcome.

Untreated incomplete isolated cleft palate: cadaveric findings.

Isolated cleft palate without cleft lip is a rare deformity. Cleft lip and cleft palate can sometimes develop in combination with a syndrome due to genetic causes. Affected patients have morbidity through life from birth and experience comprehensive treatment for such clefts including surgery. It is extremely rare that the untreated clefts are found during routine cadaveric dissection, since many patients have treatment for clefts in developed countries. Herein, we present a case of an untreated incomplete isolated cleft palate on the right side of the maxilla. An oronasal fistula was found in the same location as the missing right maxillary lateral incisor, and the soft palate was considerably intruding into the hard palate but without penetration into the nasal cavity. The right incisivus labii superioris muscle forming the oral vestibule was incompletely torn with two fistulae. An incomplete bony defect was found on the right maxilla without oronasal or oroantral fistula. This paper may contribute to evaluating the disturbed site of the incomplete isolated cleft palate with no treatment.

Ankyloglossum Superius Syndrome compromising a neonatal airway: Considerations in congenital oral airway obstructions.

We report the case of a 37-week old newborn presenting on day 1 of life with an apparent congenital fusion of the tongue to the hard palate, consistent with Ankyloglossum Superius syndrome. Physical exam along with endoscopy showed apparent fusion of the floor of the mouth to the anterior hard palate displacing the tongue into the nasal cavity and obstructing the oral airway. The child was nasotracheally intubated and brought to the operating room for lysis of the fusion under binocular microscopy. We review the literature on this rare condition and provide an algorithm for evaluating the neonatal airway in the setting of congenital oral abnormalities.

Sleep-disordered breathing, craniofacial development, and neurodevelopment in premature infants: a 2-year follow-up study.

INTRODUCTION: Sleep problems, neuro-developmental development, and sleep-disordered-breathing (SDB), are reported as more prevalent in premature infants than in full-term infants. We investigated the relationship between neuro-development, and SDB in preterm infants at 24 months corrected age (CA) with a narrow palatal presentation over time. METHODS: We enrolled infants 40 weeks or younger at birth collecting obstetric and birth data. Participants were followed up at 6, 12, 18, and 24 months CA. We evaluated craniofacial development by inspecting and photo documenting hard palate; sleep using sleep diary, actigraphy and night-time polysomnography-PSG-; and development using Bayley- Scales-of-Infant-Development and Denver-Developmental-Screening-Test (DDST) at each visit and comparing results at six months and two years. RESULTS: 244 premature infants [139 (57.0%) boys, [at birth: mean gestational age-GA- 31.5 ± 3.2 weeks, 1691.9 ± 593.9 g, 40.2 ± 5.2 cm], and 30 full term infants (50% boys), [mean GA 39.3 ± 1.0 weeks, 3131.0 ± 390.0 g, and 49.38 ± 2.0 cm] were enrolled in the study. At 6 and 24 months, 65.2% premature infants had a narrow hard palate (NHP). At 24 months, 79% had an apnea-hypopnea- index (AHI) > 1 events/hour at PSG, with a mean AHI of 3.00 ± 2.95. Only 10% of full term infants had NHP at birth and the mean AHI was 0.5 ± 0.2 event/hour at 24 months. CONCLUSION: Preterm infants have a higher occurrence of NHP at birth. At two years of age they have more sleep problems, most commonly associated with obstructive-SDB, and a higher rate of development delays. Frequency of NHP is still abnormally high, suggesting not only abnormal orofacial growth over-time, but also impact of this abnormal growth in the genesis of the obstructive-SDB.