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The International Journal of... Dec 2023Epidermolysis bullosa hereditaria (EBH) is a group of rare diseases characterized by a cutaneous-mucosal fragility with the formation of bullae, including the oral...
UNLABELLED
Epidermolysis bullosa hereditaria (EBH) is a group of rare diseases characterized by a cutaneous-mucosal fragility with the formation of bullae, including the oral mucosa. Therapeutic choices, especially prosthetic rehabilitation, must anticipate the worsening of the limitation of oral opening while respecting the functional and aesthetic expectations of the patients. This review on the oral prosthetic rehabilitation of patients with epidermolysis bullosa hereditaria (EBH) to study the level of evidence and quality of the presented available articles and establish clinical recommendations for the prosthetic management of these patients.
MATERIALS AND METHODS
An electronic search was done in July 2022 in five databases following PICOTS elements. The quality of the reports was established using the modified Pierson, Bradford Hills, and Ottawa Newcastle scale.
RESULTS
Data extracted from 19 case reports for protocolized analysis corresponded to 64 patients and 80 dental prostheses with almost 9 out of 10 patients being completely edentulous. The distribution of EBH types was 84% dystrophic, 10.5% junctional, and 5.5% simplex. The difficulties encountered by the authors synthetized in this review characteristically reflected those most likely encountered in dental practice. Most rehabilitations were implant-supported prostheses (85%) followed by removable dentures (10%) and finally dental-supported rehabilitations (5%). Fixed full-arch implant-supported prostheses represented 76.4% of implant-supported prostheses and this last prosthetic solution described showed the highest scientific quality.
CONCLUSIONS
In an individualized approach to treatment, we recommend that in cases of total edentulism, fixed full arch implant-supported prostheses are the most appropriate, as they allow the best computer-aided planning, design, manufacture, and fitting of the prosthesis in such a complex clinical context.
PubMed: 38096448
DOI: 10.11607/ijp.8791 -
JA Clinical Reports Jul 2023Freeman-Sheldon syndrome (FSS) is a rare disorder characterized by specific deformities of the extremities and face. There have been no reports of open-heart surgery in...
BACKGROUND
Freeman-Sheldon syndrome (FSS) is a rare disorder characterized by specific deformities of the extremities and face. There have been no reports of open-heart surgery in pediatric patients with FSS.
CASE PRESENTATION
We present the case of an 8-year-old girl with FSS who underwent atrial septal defect closure. Tracheal intubation was uncomplicated, although the patient had microstomia. Inhalational anesthetics and dopamine antagonists were avoided intraoperatively and perioperatively. We chose dexmedetomidine as an adjuvant for postoperative pain management contributing to adequate analgesia and early extubation without causing respiratory depression.
CONCLUSIONS
Anesthetic management of FSS requires consideration for airway management and prevention of malignant hyperthermia and respiratory complications. We successfully managed the case avoiding the use of malignant hyperthermia-triggering drugs.
PubMed: 37438578
DOI: 10.1186/s40981-023-00633-9 -
JAMA Dermatology Dec 2023
Topics: Humans; Hyaluronoglucosaminidase; Microstomia; Mixed Connective Tissue Disease; Scleroderma, Systemic
PubMed: 37851438
DOI: 10.1001/jamadermatol.2023.3893 -
Dermatologic Surgery : Official... Jun 2024Reconstruction of lower lip defects is challenging because of the functional and aesthetic demands of the lower face. We review the functional and aesthetic outcomes of...
BACKGROUND
Reconstruction of lower lip defects is challenging because of the functional and aesthetic demands of the lower face. We review the functional and aesthetic outcomes of the Karapandzic-type flaps for reconstructing lower lip defects.
METHODS
A retrospective review of patients who underwent repair using Karapandzic-type flaps.
RESULTS
Fifty patients with lower lip defects ranging from 20% to 95% (mean 59.2% ± 20%) were included. Eighteen patients (36%) were repaired using a bilateral flap, and 32 (64%) were reconstructed using a unilateral flap design. All patients had preservation of oral competency and a satisfactory aesthetic result. No patient complained of microstomia. A complication rate of 8% was noted ( n = 4) with postoperative wound infection and small areas of dehiscence. There was no statistically significant difference in complication rates in patients older than 75 years, in patients with a history of head/neck radiation, or in defects greater than 70% of lower lip breadth.
CONCLUSION
Karapandzic-type flaps are versatile and reliable for the reconstruction of a broad range of lower lip defects. This one-stage procedure can produce superior functional and aesthetic results as compared with other local and distant flaps with minimal risk of functional microstomia.
Topics: Humans; Retrospective Studies; Male; Female; Aged; Surgical Flaps; Middle Aged; Plastic Surgery Procedures; Lip Neoplasms; Aged, 80 and over; Esthetics; Lip; Adult; Treatment Outcome
PubMed: 38416801
DOI: 10.1097/DSS.0000000000004148 -
Special Care in Dentistry : Official... 2023Epidermolysis bullosa (EB) is a group of genetic disorders characterized by fragility of the skin and mucosal membranes. Dystrophic EB (DEB) is caused by mutations in...
Impression technique modification and oral contracture release surgery for orthodontic treatment in a patient with severe microstomia due to recessive dystrophic epidermolysis bullosa.
INTRODUCTION
Epidermolysis bullosa (EB) is a group of genetic disorders characterized by fragility of the skin and mucosal membranes. Dystrophic EB (DEB) is caused by mutations in the gene coding for type VII collagen. Among the most frequent oral manifestations in Recessive DEB (RDEB) are oral ulcers and blisters, absence of tongue papillae and palatal rugae, ankyloglossia, oral vestibule obliteration, and microstomia. The following report describes a modified impression technique used in a patient with severe RDEB and severe microstomia to obtain models for orthodontic treatment with aligners.
CASE REPORT
A 25-year-old female patient with severe RDEB was referred for orthodontic treatment. Severe microstomia (8 mm), hindered the use of conventional trays or intraoral scanners to design the aligners. Therefore, a contracture release surgery in combination with a modified impression technique was performed to obtain an optimal impression and subsequent aligners for orthodontic treatment.
DISCUSSION
This case presents an alternative strategy to provide orthodontic treatment with aligners in patients with severe microstomia due to severe RDEB. Reports of orthodontic treatment in people living with EB, especially in RDEB, are still rare, with few publications about fixed braces, early teeth extraction and removable devices, and none using aligners. Most of the impression techniques reported are aimed at oral rehabilitation. The multidisciplinary approach and impression technique reported should broaden the alternatives of orthodontic techniques provided to patients with EB and severe microstomia.
CONCLUSIONS
This article describes an oral contracture release surgery and modified impression technique for obtaining good quality impression for the design of orthodontic aligners in patients with severe microstomia due to severe RDEB.
PubMed: 36504454
DOI: 10.1111/scd.12808 -
The Journal of Prosthetic Dentistry Jul 2023Providing fixed implant-supported prostheses for edentulous patients with microstomia or limited mouth opening is challenging. The clinical treatment of a patient who...
Prosthetic rehabilitation of a completely edentulous maxilla with a sectioned implant-supported screw-retained prosthesis in a multiamputee patient with microstomia: A clinical report.
Providing fixed implant-supported prostheses for edentulous patients with microstomia or limited mouth opening is challenging. The clinical treatment of a patient who was a multiple amputee with microstomia and a complete loss of lips elasticity is described. A custom half-sectioned prosthesis was fabricated to resolve numerous difficulties. A screw-retained complete implant-supported prosthesis was developed from a monolithic zirconia framework supported by 4 implants and with digital scanning and computer-aided design and computer-aided manufacturing of the prosthesis. The protocol achieved a complete screw-retained implant-supported prosthesis and offered a solution for a patient with microstomia and limited mouth opening, leading to improved quality of life.
PubMed: 37442751
DOI: 10.1016/j.prosdent.2023.05.035 -
Indian Journal of Plastic Surgery :... Dec 2023Management of post-electric burn microstomia is a challenging task, especially in children, as it causes difficulty in feeding and airway problems (secondary to nasal...
Management of post-electric burn microstomia is a challenging task, especially in children, as it causes difficulty in feeding and airway problems (secondary to nasal airway blockage). The recreated defect is often full thickness and requires full-thickness tissue for reconstruction. The free flap can provide adequate normal tissue for the restoration of functions and aesthesis of the perioral region. However, performing free flaps in children is equally demanding due to small-diameter vessel anastomosis and postoperative monitoring. We present a case of postburn microstomia that was managed by contracture release and reconstruction by free radial artery forearm flap in a 1-year-old child. Postoperatively, at 6 months of follow-up, the flap settled well and the child was able to open his mouth fully with good aesthetic outcome. The free flap can be considered a good and safe option for perioral contracture release and reconstruction for better functional and aesthetic outcomes.
PubMed: 38105870
DOI: 10.1055/s-0043-1776008 -
Clinical Case Reports Nov 2023This study describes prosthetic rehabilitation an edentulous patient with microstomia. Maxillary preliminary and definitive impression were made by intraoral scanning...
This study describes prosthetic rehabilitation an edentulous patient with microstomia. Maxillary preliminary and definitive impression were made by intraoral scanning and custom 2-piece impression tray respectively to fabricate conventional denture.
PubMed: 37900713
DOI: 10.1002/ccr3.7904 -
Journal of Prosthodontics : Official... Apr 2024Microstomia presents a challenge for the patient and dental provider. This report describes a partial digital workflow for the fabrication of a mandibular complete...
Microstomia presents a challenge for the patient and dental provider. This report describes a partial digital workflow for the fabrication of a mandibular complete denture for a patient with microstomia. Computer-aided design and computer-aided manufacturing technology was utilized to 3D print a sectional custom tray with a unique design. The sectional custom tray was used to make a conventional border molded impression of the edentulous arch to fabricate a flexible complete mandibular denture for a 58-year-old female patient with scleroderma and microstomia. This treatment resulted in a successful prosthetic outcome and high patient satisfaction.
Topics: Female; Humans; Middle Aged; Denture Design; Microstomia; Dental Impression Technique; Denture, Complete; Mouth, Edentulous; Computer-Aided Design
PubMed: 37927117
DOI: 10.1111/jopr.13789 -
International Journal of Clinical... Aug 2023Moebius syndrome (MBS) is a rare congenital nonprogressive neuromuscular disorder characterized by partial or complete, unilateral or bilateral paralysis of the facial...
UNLABELLED
Moebius syndrome (MBS) is a rare congenital nonprogressive neuromuscular disorder characterized by partial or complete, unilateral or bilateral paralysis of the facial (VII) and abducens (VI) cranial nerves (CNs). In this syndrome bilateral facial palsy may occur with the involvement of VII CN and impaired eye movements can be there because of the involvement of VI CN. It can also be associated with other CN palsies, orofacial anomalies, and limb defects. MBS is diagnosed exclusively on the basis of clinical criteria, although causative genetic patterns are being documented in recent studies. The present case report describes the dental management of a 5-year-old child diagnosed with MBS. The child presented with the abnormal shape of legs, incomplete eye closure during sleep, inability to smile, lingual hypoplasia, microstomia, and hypoplastic teeth among other dental-skeletal abnormalities, and a clinical diagnosis of MBS was made.
HOW TO CITE THIS ARTICLE
Mittal M, Kumar A, Chopra R, Diagnosis and Dental Management of a Child with Moebius Syndrome: A Case Report. Int J Clin Pediatr Dent 2023;16(S-1):S109-S112.
PubMed: 37663216
DOI: 10.5005/jp-journals-10005-2626