Prosthetic rehabilitation of a patient with ectrodactyly-ectodermal dysplasia-cleft lip/palate syndrome through a hybrid workflow: A case report with 2-year follow-up.

AIM: Ectrodactyly-ectodermal dysplasia-cleft lip/palate (EEC) syndrome is a rare genetic disorder that affects ectodermal derived structures, including teeth, nails, hair, and sweat glands. Prosthetic rehabilitation of patients with EEC syndrome is essential towards improving their overall quality of life. METHODS AND RESULTS: In the case shown, a telescopic retained overdenture was made on the lower jaw based on digital impression of a patient with EEC syndrome associated with cleft lip and cleft palate. Due to the congenital anomalies and limited mouth opening, the impression was taken with intraoral scanner, and after designing the telescopes on the digital model, the primary and secondary telescopes were confectioned by selective laser sintering. CONCLUSION: Combining digital dental technology and conventional clinical prosthetic treatment methods, results in a well-functioning overdenture even in such complicated situations. The prosthodontic rehabilitation of patients with ECC helps to restore the masticatory and phonetic functions, increases the patient's self-esteem, and prevents further psychological trauma caused by hypodontia.

Maternal factors in the origin of cleft lip/cleft palate: A population-based case-control study.

OBJECTIVES: Orofacial clefts are among the most common birth defects, with an estimated worldwide incidence of around 1.5-1.7 per 1000 live-born babies. The most frequent form of orofacial clefts is cleft lip with or without cleft palate (CL ± CP). The role of environmental factors in the development of clefts is unclear in most patients, thus the aim of this study was to estimate possible maternal risk factors in the origin of CL ± CP. MATERIALS AND METHODS: 1648 CL ± CP cases, 2654 matched controls and 57 231 population controls were evaluated from The Hungarian Case-Control Surveillance of Congenital Abnormalities. Maternal factors during the critical period in cases and controls were compared. Adjusted odds ratios (OR) with 95% confidence intervals (CI) were estimated in a multivariable conditional logistic regression model. RESULTS: Among socio-demographic data, we have found significant differences between maternal employment. The proportion of unskilled mothers (6.5%) were higher in the CL ± CP group than in controls (3.5%). Medically recorded maternal anaemia, excessive vomiting and threatened abortion were associated with a higher risk of CL ± CP. An elevated risk was also found in various acute illnesses such as influenza (OR: 2.4, 95% CI: 3.0-5.8), acute bronchitis (OR: 4.5, 95% CI: 1.6-12.6) and urinary tract infections (OR: 3.5, 95% CI: 2.0-6.0). Maternal migraine and essential hypertension occurred more frequently in the mothers of cases than in controls. CONCLUSION: The findings of this study suggest that maternal diseases and lifestyle factors during the first trimester play a significant role in the development of CL ± CP.

The Burden of Care in Nasoalveolar Molding Treatment in Cleft Patients.

Objectives This study, first in Hungary, examined the success of presurgical nasoalveolar molding (NAM) therapy in cleft patients from a caregiver's perspective and revealed factors that can cause inconvenience. Patients and Methods A survey-based study was performed using a 32-item questionnaire following NAM therapy. The survey was sent to families whose child underwent NAM therapy from 2010 until 2020 at the 1st Department of Paediatrics, Semmelweis University. The questions focused on four main parts: socioeconomic, origin of the cleft, difficulties of therapy, and self-assessment. Fifty-three families received the questionnaire, 17 of them completed it. Results The mean age was 5 ± 3.7 weeks when NAM therapy started. Fifty-eight percent of the patients were male and 42% female. Patients are living more than 60 km from the cleft center (59%). Patients had to make the journey between their residence and the cleft center ∼10 to 15 times. In most cases, NAM therapy was covered by health insurance (83%). The unilateral cleft and lip palate occurred 58%, while the bilateral were 42%. Thirty-five percent of the patients had an allergic reaction against the adhesive, and 35% were affected by wounds on their lips or noses. The way of feeding was variable. Seventeen percent of the parents were able to breastfeed. In all cases, parents were satisfied with the NAM therapy. Conclusions The present study highlighted the value of caregivers' role in NAM therapy. The burden of care is acceptable, caregivers have high compliance, and are determined to help the effectiveness of therapy. Limitations of this study include a single-institute data with a small number of cases.

Evaluation of 3D visualization, planning and printing techniques in alveolar cleft repair, and their effect on patients' burden.

BACKGROUNDS: The aim of this study was to describe our auxiliary surgical techniques in alveolar cleft reconstruction and evaluate the patients burden after 3 years of surgical experience. METHODS: At the Cleft center, Semmelweis University Budapest, 28 patients underwent alveolar bone grafting between September 2017 and September 2020. We have used CBCT scans and computer software to perform 3D simulation of the alveolar defect and planning of patient- and defect specific grafts. An individual graft mold was designed for each alveolar defect and 3D printed for intra-operative use. During the surgical intervention, the mold was used to harvest the most appropriate amount and precise shape of graft material. We used cancellous iliac crest graft. After performing a descriptive statistical analysis of our patient group, we used a quality-of-life questionnaire to measure the patient's self-perception. RESULTS: In all cases the treatment sequence could be applied, planning and surgery was successful and uneventful. Patients did not experience more pain, bleeding or any feeding impairment. No inflammatory or wound healing reactions were observed. There were no major adverse effects causing permanent problems. CONCLUSIONS: Our follow-up and patient satisfaction questionnaire showed that our treatment sequence with auxiliary techniques brought no further burden to the patients care and hospitalization, even though it is more complex. Efficacy of this treatment method and improvement in graft integration has yet to be determined.

Maternal-related factors in the origin of isolated cleft palate-A population-based case-control study.

OBJECTS: Isolated cleft palate (CPO) is the rarest form of oral clefting affecting 1-25 per 10 000 newborns worldwide. There is increasing evidence for the different pathogenetic backgrounds of CPO and cleft lip with or without cleft palate. The role of environmental factors in the origin of non-syndromic and syndromic CPO is unclear in most patients. The aim of this study was to estimate possible maternal risk factors in the origin of CPO. SETTING AND SAMPLE POPULATION: The Hungarian Case-Control Surveillance of Congenital Abnormalities contains data of 32 345 birth defect cases and 57 231 control newborns. The study samples included 751 cases with isolated CPO, 1196 matched controls and 57 231 population controls. MATERIAL AND METHODS: Maternal diseases during pregnancy in cases and population controls were compared, and adjusted ORs with 95% CI were calculated in a multivariable unconditional logistic regression model. RESULTS: Beyond the well-known robust female excess (58.9%)-maternal smoking (OR with 95% CI: 2.34, 1.94-2.81) medically recorded maternal anaemia, threatened abortion and excessive vomiting in pregnancy were associated with a higher risk for CPO in the offspring. An elevated risk was found in Graves' disease (OR: 4.30, 1.74-10.62), epilepsy (OR: 4.64, 2.44-8.82), migraine (OR: 2.82, 1.18-6.76) and essential hypertension (OR: 2.33, 1.32-4.10). Among acute diseases common cold (OR: 4.94, 3.48-7.03), acute respiratory infections (OR: 4.20, 1.49-11.82), influenza (OR: 2.95, 1.75-4.95), pulpitis (OR: 7.85, 2.80-22.03), cholecystitis (OR: 3.15, 1.16-8.60), acute urinary tract infections (OR: 4.08, 2.22-7.49) and pelvic inflammatory diseases (OR: 3.93, 1.62-9.53) during pregnancy also were associated with an increased risk for developing CPO. CONCLUSION: The findings of this study suggest that maternal diseases and lifestyle factors during the first trimester play a significant role in the development of isolated cleft palate.

Histologic Evaluation of Human Intrabony Periodontal Defects Treated with Deproteinized Bovine Bone Mineral in Combination with Orthodontic Tooth Movement: A Case Series.

The aim of this case series was the histologic evaluation of guided tissue regeneration utilizing deproteinized bovine bone mineral (DBBM) when regenerative surgery was combined with (test) or without (control) early orthodontic tooth movement. Core biopsy samples were harvested from previously defected sites after 9 months. The histologic section showed integration of DBBM particles in newly formed bone in the apical and middle thirds of the defect, while in the coronal part, graft materials were mainly embedded in connective tissues in the control patient. DBBM particles showed partial resorption with more de novo bone formation in test samples.

Clinical Management of BCLP With a Severe Hypoplastic and Retruded Premaxilla.

Bilateral cleft lip and palate with a severe hypoplastic and backward rotated premaxilla and lack of soft tissues is a rare congenital facial deformity. No treatment protocol for this type of cleft is widely accepted. In patient with bilateral cleft lip and palate, the premaxilla was protracted by nasoalveolar molding before lip surgery. The nasal tip was elevated and the columella lengthened by nasal components incorporated into the palatal guidance plate. After 4 months of nasoalveolar molding, surgery could be performed without complications. Postoperative use of a guidance plate prevented relapse of the premaxillary segment, the nasal conformers maintained the nostril form.

Three-dimensional simulation of the nasoalveolar cleft defect.

This study describes the planning process for a three-dimensional (3D) model of a nasoalveolar bone graft in patients with unilateral cleft lip and palate. A 3D reconstruction of the alveolar cleft based on cone-beam computed tomography was performed in 10 patients. Graft models were planned using a 3D planning software (iPlan ENT 3.0, Brainlab, Feldkirchen, Germany) and printed using a 3D printer (Objet30 Pro, Objet Ltd., Rehovot, Israel). A reproducible, step-by-step planning method was established, which is manual rather than automatic. Still, the 3D visualization and a life-size graft template could be useful during secondary alveolar osteoplasty.

Management of prominent premaxilla in bilateral cleft lip and alveolus.

Our aim was to describe the early management protocol of the prominent premaxilla in bilateral cleft lip and alveolus and its rationale, as used in the Cleft Centre at the 1st Department of Pediatrics and at the Department of Pedodontics and Orthodontics at the Semmelweis University Budapest. The non-surgical and surgical procedures included lip taping, nasoalveolar molding, lip adhesion and definitive one-stage lip closure. With this treatment sequence, arch management was satisfactory and at the time of the definitive lip closure the position of the premaxilla did not interfere with adequate surgical repair.