Rozštěpy rtu a patra patří mezi nejčastější vrozené vady obličeje. Léčba rozštěpů je centralizovaná, multioborová a podílí se na ní plastický chirurg, ortodontista, anesteziolog, klinický logoped a další profese. Incidence rozštěpů rtu a patra zůstává stále přibližně stejná, mění se však přístup k jejich léčbě. Mezi moderní postupy léčby se stále více přidává 3D tisk a léčebné a výukové možnosti s ním spojené.

Cleft lip and cleft palate are among the most common congenital defects of the head. The treatment of clefts is centralized, multidisciplinary, and involves a plastic surgeon, orthodontist, anesthesiologist, clinical speech therapist, and other specialists. While the incidence of cleft lip and cleft palate remains approximately unchanged, the approach to their treatment is evolving. Modern treatment methods increasingly include 3D printing and the associated therapeutic and educational possibilities.

• MeSH
• 3D tisk MeSH
• lidé MeSH
• obturátory patra MeSH
• rozštěp patra * chirurgie   diagnostické zobrazování   MeSH
• rozštěp rtu * chirurgie   diagnostické zobrazování   MeSH
• zákroky plastické chirurgie MeSH
• zobrazování trojrozměrné MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• práce podpořená grantem MeSH
• přehledy MeSH

BACKGROUND: Ring 18 chromosome is a rare chromosomal aberration associated with a wide range of symptoms affecting all organ systems. One possible symptom associated with this condition is an orofacial cleft. However, to date, there are very few reported cases where the cleft has been surgically treated. CASE DESCRIPTION: In our case study, we present a female patient with Ring 18 chromosome who underwent cleft palate surgery at 14 months of age. Subsequently, a reoperation of the palate was necessary due to wound dehiscence. For the secondary reconstruction of the palate, the acellular dermal matrix (ADM) MatriDerm® was used to improve healing. The cleft palate surgery progressively improved her ability to take in food, allowing a transition from nasogastric tube feeding to oral intake. RESULTS: This is only the fourth reported case of a child with Ring 18 chromosome undergoing surgical correction of an orofacial cleft. Additionally, it is one of the first cases where an ADM MatriDerm® was used in the surgical correction of a cleft palate. In this study, we also present a comprehensive literature review, providing an overview of the various symptoms associated with this syndrome. CONCLUSION: Cleft palate surgery had a very positive effect on improving food intake in the patient with Ring 18 chromosome. The use of an acellular dermal matrix during the secondary cleft palate surgery led to improved healing and a good outcome.

• MeSH
• kojenec MeSH
• kruhové chromozomy * MeSH
• lidé MeSH
• lidské chromozomy, pár 18 genetika   MeSH
• rozštěp patra * genetika   chirurgie   MeSH
• Check Tag
• kojenec MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• přehledy MeSH

• MeSH
• kongresy jako téma MeSH
• kraniofaciální abnormality * MeSH
• ortodoncie MeSH
• rozštěp patra MeSH
• Publikační typ
• zprávy MeSH

• Klíčová slova
• orofaciální rozštěp,
• MeSH
• kraniofaciální abnormality * diagnóza   klasifikace   psychologie   MeSH
• kvalita života * MeSH
• lidé MeSH
• mladiství MeSH
• průzkumy a dotazníky MeSH
• rozštěp patra diagnóza   psychologie   MeSH
• rozštěp rtu diagnóza   psychologie   MeSH
• vrozené vady diagnóza   klasifikace   psychologie   MeSH
• Check Tag
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• klinická studie MeSH

OBJECTIVES: This study evaluated maxillary growth and dental arch relationships at 5 and 10 years of age in patients with unilateral cleft lip and palate (UCLP) who underwent early cleft lip and palate surgery. METHODS: 28 patients with UCLP who underwent cleft lip surgery in neonatal age and cleft palate surgery at average age of 7 months without orthodontic treatment (intervention group) were measured for intercanine and intermolar distances and for dental arch length. These measurements were compared with those of 30 healthy participants in a control group. Dental arch relationships in the intervention group were evaluated by 5-YO index at 5 years and the GOSLON Yardstick score at 10 years of patients' age. RESULTS: Patients in the intervention group had significantly shorter mean intercanine distance and arch length than control patients at both 5 and 10 years of age (p<.001 for all). There were no significant differences in intermolar distance at both 5 (p = .945) and 10 years (p = .105) of patients' age. The average 5YO index increased from 2.46 to an average GOSLON 10-year score of 2.89 in intervention group. CONCLUSION: Intercanine distance and dental arch length of patients with UCLP are significantly reduced at 5 and 10 years after early cleft lip and palate surgeries compared to the healthy population. Dental arch relationships at 5 and 10 years of patients with UCLP show comparable outcomes to those reported by other cleft centers. CLINICAL SIGNIFICANCE: This study evaluates maxillary growth in UCLP patients 5 and 10 years of age who underwent early primary lip and palate surgery.

• MeSH
• dítě MeSH
• lidé MeSH
• maxila * chirurgie   růst a vývoj   MeSH
• maxilofaciální vývoj MeSH
• předškolní dítě MeSH
• průřezové studie MeSH
• rozštěp patra * chirurgie   MeSH
• rozštěp rtu * chirurgie   MeSH
• studie případů a kontrol MeSH
• zubní oblouk * růst a vývoj   patologie   chirurgie   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

V současnosti se u nás se rodí v průměru jedno dítě s rozštěpem na 500 až 700 zdravých dětí. Aby tyto děti mohly přežít a začlenit se do běžného života, potřebují ke svému přežití specializovanou péči; s jednou její nezbytnou součástí seznamuje čtenáře tato kniha.

• Klíčová slova
• Stomatologie,
• MeSH
• ortodontické aparáty MeSH
• rozštěp patra komplikace   MeSH
• rozštěp rtu komplikace   MeSH

• NLK Obory
• ortodoncie

CDK13-related disorder, also known as congenital heart defects, dysmorphic facial features and intellectual developmental disorder (CHDFIDD) is associated with mutations in the CDK13 gene encoding transcription-regulating cyclin-dependent kinase 13 (CDK13). Here, we focused on the development of craniofacial structures and analyzed early embryonic stages in CHDFIDD mouse models, with one model comprising a hypomorphic mutation in Cdk13 and exhibiting cleft lip/palate, and another model comprising knockout of Cdk13, featuring a stronger phenotype including midfacial cleft. Cdk13 was found to be physiologically expressed at high levels in the mouse embryonic craniofacial structures, namely in the forebrain, nasal epithelium and maxillary mesenchyme. We also uncovered that Cdk13 deficiency leads to development of hypoplastic branches of the trigeminal nerve including the maxillary branch. Additionally, we detected significant changes in the expression levels of genes involved in neurogenesis (Ache, Dcx, Mef2c, Neurog1, Ntn1, Pou4f1) within the developing palatal shelves. These results, together with changes in the expression pattern of other key face-specific genes (Fgf8, Foxd1, Msx1, Meis2 and Shh) at early stages in Cdk13 mutant embryos, demonstrate a key role of CDK13 in the regulation of craniofacial morphogenesis.

• MeSH
• cyklin-dependentní kinasy metabolismus   genetika   MeSH
• embryo savčí metabolismus   MeSH
• embryonální vývoj * genetika   MeSH
• fenotyp MeSH
• lebka embryologie   patologie   MeSH
• mentální retardace genetika   MeSH
• modely nemocí na zvířatech * MeSH
• mutace genetika   MeSH
• myši MeSH
• nervus trigeminus embryologie   MeSH
• neurogeneze * genetika   MeSH
• obličej embryologie   abnormality   MeSH
• protein doublecortin MeSH
• rozštěp patra genetika   patologie   embryologie   MeSH
• rozštěp rtu genetika   patologie   embryologie   MeSH
• vývojová regulace genové exprese * MeSH
• zvířata MeSH
• Check Tag
• myši MeSH
• zvířata MeSH
• Publikační typ
• časopisecké články MeSH

• MeSH
• dítě MeSH
• lidé MeSH
• rizikové faktory MeSH
• rozštěp patra * chirurgie   komplikace   MeSH
• rozštěp rtu * chirurgie   komplikace   MeSH
• stupeň závažnosti nemoci * MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: Orofacial clefts are the most common congenital abnormalities. Cleft lip reconstruction is performed mostly in 3 months of life including the neonatal period. The consumption of opioids during anesthesia is one of the monitored parameters of anesthesia safety. We investigated the effect of using an infraorbital nerve block for reducing opioid consumption during cleft lip surgery in neonates. PATIENTS/METHODS: Overall, 100 patients who underwent primary cleft lip surgery in neonatal age between 2018 and 2021 were included in the study. The primary outcome was to compare opioid requirements during cleft lip surgery with and without using regional anesthesia. Secondary outcomes included a first oral intake from surgery between neonates with and without regional anesthesia and complications rate of infraorbital nerve block. RESULTS: Data from 100 patients (46 patients with and 64 without regional anesthesia) were retrospectively analyzed and classified into two groups according to whether regional anesthesia during neonatal cleft lip surgery had been performed or not. The use of infraorbital block was found to be positively correlated with lower doses of opioids used during the general anesthesia for the surgery (mean 0.48 μg/kg vs 0.29 μg/kg, p < 0.05). The postoperative course was evaluated based on the interval from surgery to first oral intake which was statistically insignificant shorter (p = 0.16) in the group of patients using regional anesthesia. No complications were recorded in the group of patients with regional anesthesia. CONCLUSIONS: Regional anesthesia is associated with reduced opioid consumption during anesthesia thereby increasing the safety of anesthesia in neonates. GOV IDENTIFIER: NCT06067854https://clinicaltrials.gov/study/NCT06067854?cond=NCT06067854&rank=1.

• MeSH
• lidé MeSH
• nervová blokáda * metody   MeSH
• novorozenec MeSH
• opioidní analgetika * terapeutické užití   MeSH
• pooperační bolest farmakoterapie   prevence a kontrola   MeSH
• retrospektivní studie MeSH
• rozštěp patra * chirurgie   MeSH
• rozštěp rtu * chirurgie   MeSH
• svodná anestezie * metody   MeSH
• zákroky plastické chirurgie metody   škodlivé účinky   MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

OBJECTIVE: To assess modelled facial development of infants with unilateral cleft lip (CL) and cleft lip and palate (UCLP) compared to controls up to two years of age. DESIGN AND PARTICIPANTS: A total of 209 facial images of children (CL: n = 37; UCLP: n = 39; controls: n = 137) were obtained in four age categories (T0 = 0.2-0.5; T1 = 0.6-1.0; T2 = 1.1-1.5; T3 = 1.6-2.0 years) and were evaluated using stereophotogrammetry and geometric morphometry. All patients underwent lip surgery before T0, patients with UCLP underwent palatoplasty (T0, T1 before palatoplasty; T2, T3 after palatoplasty). RESULTS: In patients with CL, the forehead was significantly retracted (p ≤ 0.001), while the supraorbital and ocular regions were prominent (p ≤ 0.001). The oronasal region appeared convex (p ≤ 0.001). The lower lip and chin were non-significantly protruded. In patients with UCLP, a significantly retracted forehead and prominent supraorbital region were apparent (p ≤ 0.001). A retrusive oronasal region (p ≤ 0.001) was observed in the middle face. The chin was anteriorly protruded (p ≤ 0.01). No progression of deviations was found with increasing age. After the first year, a slight improvement in the morphological features became apparent. The shape variability of the clefts and controls overlapped, suggesting a comparable modelled facial development. CONCLUSIONS: The facial morphology of individuals with cleft was comparable to the norm. Shape deviation was apparent in the oronasal region, forehead, and chin, which minimised with increasing age even in complete clefts.

• MeSH
• fotogrammetrie * metody   MeSH
• kefalometrie MeSH
• kojenec MeSH
• lidé MeSH
• maxilofaciální vývoj MeSH
• obličej anatomie a histologie   abnormality   MeSH
• předškolní dítě MeSH
• průřezové studie MeSH
• rozštěp patra * chirurgie   diagnostické zobrazování   patologie   MeSH
• rozštěp rtu * chirurgie   patologie   MeSH
• studie případů a kontrol MeSH
• zobrazování trojrozměrné * MeSH
• Check Tag
• kojenec MeSH
• lidé MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH