Oral surgery with nasotracheal intubation in a patient with ectodermal dysplasia.
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ABSTRACT:
SUBMITTER: Nanegrungsunk D
PROVIDER: S-EPMC7377268 | biostudies-literature |
REPOSITORIES: biostudies-literature
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| S-EPMC3275962 | biostudies-literature
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| S-EPMC1235496 | biostudies-literature
Project description:This paper describes odontomicronychial dysplasia, a pure ectodermal dysplasia of the 2-3 subgroup of group A. It is characterised by precocious eruption and shedding of deciduous dentition, precocious eruption of secondary dentition with short, rhomboid roots, and short, thin, slow growing nails. This condition probably results from an autosomal recessive gene.
| S-EPMC1051874 | biostudies-other
Project description:IntroductionAn analysis was made of three different syndromes associated with p63 gene mutations, known as ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC), ankyloblepharon-ectodermal dysplasia clefting syndrome (AEC or Hay-Wells) and Rapp-Hodgkin syndrome (RHS). The postoperative complications associated with their cleft reconstructions were also evaluated.Materials and methodsExtensive demographic information, in particular of the clinical appearances, associated malformations, and the types and complications of the reconstructive surgical procedures, were recorded of these syndromic cases occurring in a database of 3621 facial cleft deformity patients. The data was analyzed using the Microsoft Excel program.ResultsA total of 10 (0.28%) cases of p63 associated syndromes were recorded: EEC (6), RHS (3), and AEC (1). The following clinical cleft appearances were noted - EEC = 6: CLA 1 -right side unilateral (female); CLAP 4 - right side (1) + left side (1) unilateral (male + female); bilateral (2) (males); hPsP 1 (female) (divided in 3 Black, 2 White, 1 Indian); RHS = 3: CLAP 2 (White males); hPsP 1 (White female); AEC = 1: CLAP bilateral (White male). Other features of the syndromes were: skin, hand, foot, tooth, hair and nail involvement, and light sensitivity. Postoperative complications included: (i) stenosis of nasal opening, especially after reconstruction of the bilateral cleft lip and the columella lengthening (2 cases), (ii) premaxilla-prolabium fusion (2 cases), (iii) repeated occurrence of oro-nasal fistula in the hard palate (4 cases), and (iv) dysgnathial development of midfacial structures (3 cases).DiscussionThree different p63 associated syndromes (EEC, AEC, and RHS) were diagnosed (0.27% of the total facial cleft deformities database). The majority of the cases presented with a bilateral CLAP in males only. A number of females and males had unilateral CLA. The hPsP-cleft was recorded in females only. The associated ectodermal component most probably had a profoundly negative influence on postoperatively wound healing, which was observed in particular at the nasal openings, the premaxilla sulcus and in the hard palate mucosa. The reconstruction of p63 associated syndromes is a greater challenge than the usual cleft reconstruction to the surgeon.
| S-EPMC3645613 | biostudies-literature
Project description:Ectodermal dysplasia (ED) refers to a heterogeneous group of genetic diseases of the skin, skin appendages, and teeth. People with ED experience a poorer oral health-related quality of life (OHRQoL) compared to the general population. The aim of this study was to examine the OHRQoL of people with ED and to measure their objective physical oral health to confirm or disprove evidence of poorer oral health in this population. To determine OHRQoL, the German version of the 14-item Oral Health Impact Profile (OHIP-14G) was used. All the participants in the study were clinically examined, and the measured parameters were recorded using the Physical Oral Health Index (PhOX). In total, 10 male and 11 female participants, with an average age of 22.0 ± 9.0 years, were included in this study. The OHIP-14G summary score was 23.9 (±15.2) points (range: 0-56 points). The PhOX summary score was 61.2 (±5.1) points (range: 22-80 points). The findings indicated that both the OHRQoL and physical oral health of the participants were highly impaired and that their objective and subjective oral health were worse than those of the general population in Germany.
| S-EPMC7796382 | biostudies-literature
Project description:Ectodermal dysplasia (ED) is a hereditary genetic disorder that manifests a variety of deformities in one or more of the ectodermal derivatives. Ectodermal derivatives originate from ectodermal layers during embryonic development, such as skin, nails, hair, teeth, and exocrine glands. Over 150 variants of ED are reported in the literature. It has an incidence of seven in every 100,000 live births. There are two types of ED, which are hypohidrotic (anhidrotic) and hydrotic. The types are classified according to the degree of function of the sweat glands. This report discusses the case of a 13-month-old Saudi girl with typical features of ectodermal dysplasia who presented to a dermatology clinic.
| S-EPMC8759711 | biostudies-literature
Project description:Background: Intubation of neonates is difficult and hazardous. Factors associated with procedure-related adverse events and unsuccessful intubation attempts are insufficiently evaluated, especially during neonatal nasotracheal intubations. Objective: Aim of this study was to determine the frequency of tracheal intubation-associated events (TIAEs) during neonatal nasotracheal intubations and to identify factors associated with TIAEs and unsuccessful intubation attempts in our neonatal unit. Methods: This was a prospective, single-site, observational study from May 2017 to November 2019, performed at a tertiary care neonatal intensive care unit in a German academic teaching hospital. All endotracheal intubation encounters performed by the neonatal team were recorded. Results: Two hundred and fifty-eight consecutive intubation encounters in 197 patients were analyzed. One hundred and forty-eight (57.4%) intubation encounters were associated with at least one TIAE. Intubation inexperience (<10 intubation encounters) (OR = 2.15; 95% CI, 1.257-3.685) and equipment problems (OR = 3.43; 95% CI, 1.12-10.52) were predictive of TIAEs. Intubation at first attempt (OR = 0.10; 95% CI, 0.06-0.19) and videolaryngoscopy (OR = 0.47; 96% CI, 0.25-0.860) were predictive of intubation encounters without TIAEs. The first intubation attempt was commonly done by pediatric residents (67.8%). A median of two attempts were performed until successful intubation. Restricted laryngoscopic view (OR = 3.07; 95% CI, 2.08-4.53; Cormack-Lehane grade 2 vs. grade 1), intubation by pediatric residents when compared to neonatologists (OR = 1.74; 95% CI, 1.265-2.41) and support by less experienced neonatal nurses (OR = 1.60; 95% CI, 1.04-2.46) were associated with unsuccessful intubation attempts. Conclusions: In our unit, TIAEs and unsuccessful intubation attempts occurred frequently during neonatal nasotracheal intubations. To improve success rates, quality improvement und further research should target interprofessional education and training, equipment problems and videolaryngoscopy.
| S-EPMC8144442 | biostudies-literature
Project description:BackgroundNasotracheal intubation (NTI) is commonly performed in oromaxillofacial surgeries. We did this metanalysis to ascertain whether use of video laryngoscopy (VL) provided better NTI characteristics as compared to direct laryngoscopy (DL) in patients undergoing oromaxillofacial surgeries.MethodsWe performed a systematic search to identify randomized controlled trials comparing VL with DL for NTI in adults undergoing elective oromaxillofacial surgery. The primary outcome was time to intubation. Secondary outcomes included the first attempt success, overall success, incidence of nasal bleeding, Cormack and Lehane grade, and maneuvers required.ResultsOf the 456 studies identified following a systematic search, 10 were included. Meta-analysis showed a significantly lower time to tracheal intubation favoring VL (mean difference: -9.04, 95% CI [-12.71, -5.36], P < 0.001; I2 = 59%). VL was also associated with a greater first attempt success (relative risk [RR]: 1.10, 95% CI [1.04, 1.16], P = 0.001). Maneuvers to facilitate intubation were less with VL (RR: 0.22, 95% CI [0.10, 0.51], P < 0.001). There was no difference in overall intubation success (RR: 1.04, 95% CI [0.98, 1.10], P = 0.17). The incidence of bleeding did not differ between the DL and VL groups (RR: 0.59, 95% CI [0.32, 1.08], P = 0.09).ConclusionsEvidence as per this meta-analysis suggests VL leads to a shorter time to NTI, a greater first attempt success rate, and reduced need for maneuvers when compared to DL. The present study supports use of VL as a first line device for NTI in oral-maxillofacial surgeries in experienced hands.
| S-EPMC8497912 | biostudies-literature
Project description:Hypohidrotic ectodermal dysplasia (HED) is a type of genodermatosis characterized by the abnormal development of sweat glands, teeth, and hair. The most prevalent form of HED is X-linked hypohidrotic ectodermal dysplasia (XLHED), which is associated with mutations in the EDA gene. The aim of this case report was to describe a family with XLHED with emphasis on differences in orofacial features between members. Family members were systematically evaluated to characterize the pattern of inheritance and clinical features. Dental examination included evaluation of agenesis and abnormal teeth structure. The pedigree of the last seven generations of the family was constructed. Clinical examination and medical history revealed five males affected by HED and nine female as heterozygous carriers. The males exhibited the classic phenotype of XLHED, with dental abnormalities, hypohydrosis, and craniofacial dysmorphologies. The heterozygous carriers of the X-linked gene defect principally exhibited dental agenesis of the lateral maxillary incisors. Careful clinical examination, including dental evaluation, is an important way to detect heterozygous carriers of X-linked HED. Heterozygous parents of patients with HED may also show some features of the disorder. The identification of female carriers results in genetic counseling being offered to affected families, as well as providing adequate treatment as necessary and long-term follow-up of these patients.
| S-EPMC3500897 | biostudies-literature
Project description:PurposeThis study investigated the feasibility and efficacy of continuous noninvasive ventilation (NIV) support with 100% oxygen using a specially designed face mask, for reducing desaturation during fiberoptic bronchoscopy (FOB)-guided intubation in critically ill patients with respiratory failure.Materials and methodsThis was a single-center prospective randomized study. All patients undergoing FOB-guided nasal tracheal intubation were randomized to bag-valve-mask ventilation or NIV for preoxygenation followed by intubation. The NIV group were intubated through a sealed hole in a specially designed face mask during continuous NIV support with 100% oxygen. Control patients were intubated with removal of the mask and no ventilatory support.ResultsWe enrolled 106 patients, including 53 in each group. Pulse oxygen saturation (SpO2) after preoxygenation (99% (96%-100%) vs. 96% (90%-99%), p = .001) and minimum SpO2 during intubation (95% (87%-100%) vs. 83% (74%-91%), p < .01) were both significantly higher in the NIV compared with the control group. Severe hypoxemic events (SpO2 < 80%) occurred less frequently in the NIV group than in controls (7.4% vs. 37.7%, respectively; p < .01).ConclusionsContinuous NIV support during FOB-guided nasal intubation can prevent severe desaturation during intubation in critically ill patients with respiratory failure.Trial registrationClinicalTrials.gov, NCT02462668. Registered on 25 May 2015, https://www.clinicaltrials.gov/ct2/results?term=NCT02462668.
| S-EPMC7126932 | biostudies-literature