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
• Printing, Three-Dimensional MeSH
• Humans MeSH
• Palatal Obturators MeSH
• Cleft Palate * surgery   diagnostic imaging   MeSH
• Cleft Lip * surgery   diagnostic imaging   MeSH
• Plastic Surgery Procedures MeSH
• Imaging, Three-Dimensional MeSH
• Check Tag
• Humans MeSH
• Publication type
• Research Support, Non-U.S. Gov't MeSH
• Review 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
• Infant MeSH
• Ring Chromosomes * MeSH
• Humans MeSH
• Chromosomes, Human, Pair 18 genetics   MeSH
• Cleft Palate * genetics   surgery   MeSH
• Check Tag
• Infant MeSH
• Humans MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Case Reports MeSH
• Review MeSH

• MeSH
• Congresses as Topic MeSH
• Craniofacial Abnormalities * MeSH
• Orthodontics MeSH
• Cleft Palate MeSH
• Publication type
• News MeSH

• Keywords
• orofaciální rozštěp,
• MeSH
• Craniofacial Abnormalities * diagnosis   classification   psychology   MeSH
• Quality of Life * MeSH
• Humans MeSH
• Adolescent MeSH
• Surveys and Questionnaires MeSH
• Cleft Palate diagnosis   psychology   MeSH
• Cleft Lip diagnosis   psychology   MeSH
• Congenital Abnormalities diagnosis   classification   psychology   MeSH
• Check Tag
• Humans MeSH
• Adolescent MeSH
• Male MeSH
• Female MeSH
• Publication type
• Clinical Study 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
• Child MeSH
• Humans MeSH
• Maxilla * surgery   growth & development   MeSH
• Maxillofacial Development MeSH
• Child, Preschool MeSH
• Cross-Sectional Studies MeSH
• Cleft Palate * surgery   MeSH
• Cleft Lip * surgery   MeSH
• Case-Control Studies MeSH
• Dental Arch * growth & development   pathology   surgery   MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Male MeSH
• Child, Preschool MeSH
• Female MeSH
• Publication type
• Journal Article 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.

• Keywords
• Stomatologie,
• MeSH
• Orthodontic Appliances MeSH
• Cleft Palate complications   MeSH
• Cleft Lip complications   MeSH

• NML Fields
• 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
• Cyclin-Dependent Kinases metabolism   genetics   MeSH
• Embryo, Mammalian metabolism   MeSH
• Embryonic Development * genetics   MeSH
• Phenotype MeSH
• Skull embryology   pathology   MeSH
• Intellectual Disability genetics   MeSH
• Disease Models, Animal * MeSH
• Mutation genetics   MeSH
• Mice MeSH
• Trigeminal Nerve embryology   MeSH
• Neurogenesis * genetics   MeSH
• Face embryology   abnormalities   MeSH
• Doublecortin Protein MeSH
• Cleft Palate genetics   pathology   embryology   MeSH
• Cleft Lip genetics   pathology   embryology   MeSH
• Gene Expression Regulation, Developmental * MeSH
• Animals MeSH
• Check Tag
• Mice MeSH
• Animals MeSH
• Publication type
• Journal Article MeSH

• MeSH
• Child MeSH
• Humans MeSH
• Risk Factors MeSH
• Cleft Palate * surgery   complications   MeSH
• Cleft Lip * surgery   complications   MeSH
• Severity of Illness Index * MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Male MeSH
• Female MeSH
• Publication type
• Journal Article 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
• Humans MeSH
• Nerve Block * methods   MeSH
• Infant, Newborn MeSH
• Analgesics, Opioid * therapeutic use   MeSH
• Pain, Postoperative drug therapy   prevention & control   MeSH
• Retrospective Studies MeSH
• Cleft Palate * surgery   MeSH
• Cleft Lip * surgery   MeSH
• Anesthesia, Conduction * methods   MeSH
• Plastic Surgery Procedures methods   adverse effects   MeSH
• Check Tag
• Humans MeSH
• Male MeSH
• Infant, Newborn MeSH
• Female MeSH
• Publication type
• Journal Article 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
• Photogrammetry * methods   MeSH
• Cephalometry MeSH
• Infant MeSH
• Humans MeSH
• Maxillofacial Development MeSH
• Face anatomy & histology   abnormalities   MeSH
• Child, Preschool MeSH
• Cross-Sectional Studies MeSH
• Cleft Palate * surgery   diagnostic imaging   pathology   MeSH
• Cleft Lip * surgery   pathology   MeSH
• Case-Control Studies MeSH
• Imaging, Three-Dimensional * MeSH
• Check Tag
• Infant MeSH
• Humans MeSH
• Male MeSH
• Child, Preschool MeSH
• Female MeSH
• Publication type
• Journal Article MeSH