scholarly journals Oral-Facial-Digital Syndrome Type IX with Subfoveal Drusenoid Deposit

2020 ◽  
Vol 09 (04) ◽  
pp. 289-292
Author(s):  
Jong In You ◽  
Seul Ki Bang ◽  
Seung-Young Yu ◽  
Kyung Hyun Jin

AbstractWe describe a patient with oral-facial-digital syndrome (OFDS) with the following anomalies: cleft lip, cleft palate, micrognathia, hypertelorism, nasal septum deviation, thumb polydactyly in the right hand, and partial agenesis of the corpus callosum. In addition, the patient had optic disc coloboma in the left eye and subfoveal drusenoid deposit in the right eye, features of OFDS type IX. Subfoveal drusenoid deposit has not been previously reported in OFDS type IX. Evaluation of the fundus is necessary for diagnosis of OFDS.

Author(s):  
Cesar B. Albuquerque ◽  
Nataly R. M. Zambrana ◽  
Jéssica R. M. Zambrana ◽  
Rodrigo A. Ribeiro ◽  
Daniela M. R. A. Salgado ◽  
...  

Introduction: The Haller Cells are described as aerated ethmoidal cells, located in the inferior margin of the orbit, near the ostia of the maxillary sinuses. The Cone Beam Computed Tomography (CBCT) is widely used in dentistry, allowing acquisition of images of the craniofacial region. Objective: to identify in CBCT exams the Haller´s cell relationship with the following conditions: sinus disease, bony nasal septum deviation, endodontic treatment and periapical lesions. Materials and methods: 99 CBCT exams were used, from which 51 were included in the research criteria. The images were analyzed in the Xelis Dental® software to identify the presence or absence of the Haller Cell, as well as the relationship with the mentioned conditions. Results: Among the 51 CFCT exams evaluated, 35.3% presented Haller´s Cell on the right side and 23.5% on the left side. Whereas the relationship of the Haller Cells with one or more alterations mentioned here, on the right side we had it in 72% of the cases, while on the left side such relationship was present in 75% of the cases. Conclusion: CBCT exams that present the infraorbital margin allow to verify the presence or absence of the Haller Cell. In this sample, we verified a greater presence of endodontic cases, bony nasal septum deviation and sinus disease cases in individuals who presented Haller Cells.


2021 ◽  
pp. 105566562110232
Author(s):  
Maryam Paknahad ◽  
Aida Pourzal ◽  
Mohammad Mahjoori-Ghasrodashti ◽  
Leila Khojastepour

Objectives: The detection of anatomical variation impairments in patients with cleft lip and palate (CLP) is crucial in combined orthodontic and maxillofacial surgical treatments. Therefore, this study aimed at evaluating the anatomical maxillary sinus characteristics, nasal septum deviation (NSD), and mucosal thickening of the maxillary sinus in patients with CLP using cone beam computed tomography (CBCT). Design: The CBCT images were classified into 3 groups of unilateral cleft lip and palate (UCLP; n = 40), bilateral cleft lip and palate (BCLP; n=14), and noncleft (control; n = 54). Subsequently, the maxillary sinus linear dimensions were assessed. Height, width, depth, infundibulum height, and ostium width were assessed as quantitative measures, whereas nasal septum deviation and mucosal thickening were assessed as qualitative measures. One-way analysis of variance and χ2 tests were utilized to identify any significant differences among the groups regarding the aforementioned variables. Results: Significant differences were observed among the groups regarding maxillary sinus height and depth, NSD, and mucosal thickening. Moreover, UCLP and BCLP groups showed higher incidence of NSD and mucosal thickening. However, the size of maxillary sinus height and depth was lower in the UCLP and BCLP groups. Conclusion: The results showed that BCLP and UCLP groups obtained lower maxillary sinus height and depth compared to the control group. On the other hand, incidence of the NSD and mucosal thickening was significantly higher in UCLP and BCLP groups than those in the control group.


2021 ◽  
Vol 16 (8) ◽  
pp. 2211-2213
Author(s):  
Prajina Pradhan ◽  
Subash Phuyal ◽  
Ritesh Lamsal ◽  
Pooja Agrawal ◽  
Raju Paudel

2021 ◽  
pp. 105566562110577
Author(s):  
Jaideep Singh Chauhan ◽  
Sarwpriya Sharma

Objective: To analyse the morphological presentation of orofacial clefts, gender, syndromes and systemic anomalies associated with them. Design: This was an epidemiological study performed in the patients who were registered for cleft lip and palate surgeries in our centre. The data was evaluated both retrospectively as well as prospectively. Patients/ Participants: The patients registered from November 2006 to April 2021 were studied. Out of 5276 patients, data of 5004 cases were analysed, rest 272 patients were excluded due to lack of information. Statistical analysis and Chi square test were applied. Results: Cleft deformities were more common in males than females. Cleft lip with palate was the commonest phenotype (52.2%). It was followed by isolated cleft lip (22.9%), isolated cleft palate (22.1%), rare clefts (1.62%) and syndromic clefts (1.18%). Unilateral variants were more frequent than bilateral. In unilateral, left side was more common than the right side. Among bilateral, most of the cases had premaxillary protrusion. In the present study, 3.46% of all the patients had associated anomalies affecting their other organs. Less common cleft phenotypes like microform cleft lip and submucous cleft palate ± bifid uvula showed frequency of 0.62% and 0.64% respectively. Conclusion: Thorough examination of cleft deformity should be done as it may appear as an isolated deformity or part of a syndrome and have associated systemic anomalies. This may help us to deliver comprehensive care to the patients and can prevent potential operative complications.


2019 ◽  
Vol 30 (2) ◽  
pp. 433-436 ◽  
Author(s):  
Imran Aydoğdu ◽  
Yavuz Atar ◽  
Zeynep Aydoğdu ◽  
Ziya Saltürk ◽  
Enes Ataç ◽  
...  

2019 ◽  
Vol 30 (5) ◽  
pp. 1605-1608
Author(s):  
Israfil Orhan ◽  
Tugrul Ormeci ◽  
Nagihan Bilal ◽  
Saime Sagiroglu ◽  
Adem Doganer

2013 ◽  
Vol 130 (6) ◽  
pp. 359-361
Author(s):  
C.-A. Righini ◽  
I. Atallah ◽  
E. Reyt

Sign in / Sign up

Export Citation Format

Share Document