The causes are injuries, maternal diseases transferred during pregnancy, endocrine and metabolic disorders, congenital disorders of osteogenesis , including genetic effects on the fetus with toxins during pregnancy, especially in the first 10 weeks , damage to the growth areas of the skull, and a change in functional load. The formation of the gill apparatus and its derivative organs and tissues of the maxillofacial region is disrupted.
Klein (1968) compiled a list of syndromes involving tissue derived from the first and second branchial arches. He identified 20 major syndromes: otocephaly ; dysostosis – craniofacial; mandibular; mandibular ear; eye- ear; mandibular- eye- facial dysmorphia ; mandibular- eye- facial dyscephaly ; eye- vertebral dysplasia; ear-vertebral dysplasia; cranial-eyelid-iris – skin dysplasia with deafness; cervico-ocular facial dysmorphia with deafness; facial nerve palsy, deafness, pre-growths , pre-aural and cervical fistulas; microgenia ; glossoptosis , cleft palate (Robin syndrome); eye – tooth- finger dysplasia with microphthalmia ; eye-phalangeal disco ; mandibular dysostosis with lack of limbs; roto- finger-facial dysostosis ; maxillofacial dysostosis ; acrolitic dysostosis ; craniomandibular-facial dysplasia. The most common craniofacial dysostoses , which are also indicated in the literature as syndromes of the first and second branchial arches, hemifascial dwarfism, lateral dysplasia of the face, craniofacial dwarfism. You should use the following classification developed by domestic researchers [ Kalamkarov X. A., 1967; Bezrukov V.M., 1981; Gunko V.I., 1982]. 1. Deformation of the jaw. 1.1. Macognathia (upper, lower, symmetric, asymmetric, of various departments or the entire jaw). 1.2. Micrognathia (upper, lower, symmetric, asymmetric, of various departments or the entire jaw). 1.3. Prognathy (upper, lower, functional, morphological). 1.4. Retrognathia (upper, lower, functional, morphological). 2. Combined deformations of the jaws (symmetric, asymmetric). 2.1. Upper micro, retrognathia , lower macro, prognathia ; 2.2. Upper macro, prognathia , lower micro, retrognathia ; 2.3. Upper and lower macrognathia . 3. Combined tooth abnormalities and jaw deformities. 4. Combined tooth abnormalities, deformations of the jaw and facial skeleton. 4.1. Symmetric: 4.1.1. Maxillofacial dysostosis ( Tricher -Collins syndrome , Franceschetti ); 4.1.2. Craniostenosis ( Aper syndrome , Cruson syndrome ); 4.1.3. Hypertelorism I-II-III degree; 4.2. Asymmetrical: 4.2.1. Otocraniostenoses of the I-II-III degree ( hemifacial microsomy ), eye- ear-vertebral dysplasia, Goldenbar syndrome ; 4.2.2. Hypertelorism I-II-IH degree.
Clinic of congenital craniofacial deformities
Aesthetic (change in face shape) and functional disorders prevail. Complaints about difficulty in biting and chewing food due to malocclusion. Difficult speech, breathing, functions of the digestive system, chewing muscles, and dysfunction of the temporomandibular joint were noted. Periodontal tissue diseases develop. The nature of the deformation determines the features of clinical manifestations.
The observance of a clear sequence in the examination of patients matters. It is necessary to study the local status, photographs of the face, models of the jaws, and carry out x-ray and functional studies. Inspection sequence: position of the head, face contours in the face and profile, its proportions, symmetry, correlation of dentition, shape, size, position of teeth, periodontal condition, condition of the nasal cavity, nasal breathing, range of motion of the lower jaw, size of the tongue, its position, articulation, structural features and function of hard and soft palate, state of pain sensitivity.
Treatment of congenital craniofacial deformities
Osteo-reconstructive operations: osteotomy of the maxillary complex with subsequent movement of the osteotomy fragment to the correct position on the bite; flat osteotomy of the branches of the lower jaw; alloplasty of the condylar process, branch, lower jaw body with an orthotopic transplant; step genioplasty , contour plastic. Patients require rational immobilization, orthodontic and orthopedic treatment, dispensary observation.
Follicular erythromelanosis of the face and neck
A skin disease characterized by a clinical triad: well-defined erythema, hyperpigmentation and follicular pattern on the face and neck.
Etiology follicular erythromelanosis of the face and neck
Autosomal recessive hereditary disease or acquired autonomous neuronal dysfunction. The connection with the influence of drugs, chemicals, the environment or ultraviolet radiation is not always possible to identify.
Clinic of follicular erythromelanosis of the face and neck
A triad is characteristic – well-delimited erythema, hyperpigmentation and follicular pattern on the face and neck. Young people complain of persistent erythema on the cheeks and neck, not related to exposure to sunlight , the effects of cold and heat, physical exertion, medication, or redness per minute of embarrassment. In places of erythema, you can consider the pattern of thin blood vessels. With the help of diascopic examination, hyperpigmentation is detected in the basal layer. On the side surfaces of the face and neck, pronounced follicles are observed, and on the shoulders and back – keratosis pilaris .
Diagnosis of follicular erythromelanosis of the face and neck
1. Clinical triad.
2. Histological examination: somewhat orthohyperkeratotic stratum corneum, pigmentation is enhanced in the basal layer. Follicular horn plugs and pseudo- horn keratin cysts are visualized in the epidermis . Sebaceous glands are enlarged. The openings of the hair follicles are dilated. In addition, significantly dilated blood vessels and small perivascular lymphocytic infiltration are noted in the upper layer of the skin .
Differential diagnosis of follicular erythromelanosis of the face and neck
– Ulerythema oophryogenes – Atrophoderma vermiculatum – Lichen pilaris faciei – Poikiloderma Sivata ( Civatte ).
Treatment of follicular erythromelanosis of the face and neck
1. Locally – topical retinoids .
2. Ammonium lactate cream .
3. Hydroquinone (3%).