Cases reported "Cleft Lip"

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1/9. Working with parents to promote health.

    A cleft lip, with or without cleft palate, is the most common craniofacial malformation. The issue of whether health can be attained if the person has a physical defect is discussed. health education can be considered as a means whereby individuals learn to maintain, restore and promote health. Consideration is given to health promotion and the prevention of ill health in relation to infant feeding. Skill teaching, if successfully completed, may be regarded as a fundamental cornerstone to health promotion.
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ranking = 1
keywords = physical
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2/9. A surgical simulator for cleft lip planning and repair.

    The objective of this project was to develop a computer-based surgical simulation system for cleft lip planning and repair. This system allows the user to interact with a virtual patient to perform the traditional steps of cleft-lip repair. The system interfaces to force-feedback (haptic) devices to track the user's motion and provide feedback during the procedure, while performing real-time soft-tissue simulation. An eleven-day old unilateral cleft-lip and palate patient was previously CT scanned for ancillary diagnostic purposes using standard imaging protocols and 1mm slices. High-resolution 3D meshes were automatically generated from this data using the ROVE software created in our lab. The resulting 3D meshes of bone and soft-tissue were instilled with physical properties of soft tissues for purposes of simulation. Once these preprocessing steps were completed, the patient's bone and soft-tissue data are presented on the computer screen in stereo and the user can freely view, rotate, and otherwise interact with the patient's data in real-time. The user is prompted to select anatomical landmarks on the patient data for preoperative planning purposes, then their locations are compared against that of a "gold standard" and a score, derived from their deviation from that standard and time required, is generated. The user can then move a haptic stylus and guide the motion of the virtual cutting tool. The soft tissues can thus be incised using this virtual cutting tool, moved using virtual forceps, and fused in order to perform any of the major procedures for cleft-lip repair. Real-time soft tissue deformation of the mesh realistically simulates normal tissues and haptic-rate (>1kHz) force-feedback is provided. The surgical result of the procedure can then be immediately visualized and the entire training process can be repeated at will. A short evaluation study was also performed. Two groups (nonmedical and plastic surgery residents) of six-people each performed the anatomical marking task of the simulator four times. Results showed that the plastic surgery residents scored consistently better than the people without medical background. Every person's score increased with practice, and the length of time needed to complete the eleven markings decreased. The data was compiled and showed which specific markers consistently took users the longest to identify as well as which locations were hardest to accurately mark. Our findings suggest that the simulator is a valuable training tool, giving residents a way to practice anatomical identification for cleft lip surgery without the risks associated with training on a live patient. Educators can also use the simulator to examine which markers are consistently problematic, and modify their training to address these needs.
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ranking = 1
keywords = physical
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3/9. A surgical simulator for planning and performing repair of cleft lips.

    The objective of this project was to develop a computer-based surgical simulation system for planning and performing cleft lip repair. This system allows the user to interact with a virtual patient to perform the traditional steps of cleft-lip repair (rotation-advancement technique). MATERIALS AND methods: The system interfaces to force-feedback (haptic) devices to track the user's motion and provide feedback during the procedure, while performing real-time soft-tissue simulation. An 11-day-old unilateral cleft lip, alveolus and palate patient was previously CT scanned for ancillary diagnostic purposes using standard imaging protocols and 1mm slices. High-resolution 3D meshes were automatically generated from this data using the ROVE software developed in-house. The resulting 3D meshes of bone and soft tissue were instilled with physical properties of soft tissues for purposes of simulation. Once these preprocessing steps were completed, the patient's bone and soft tissue data are presented on the computer screen in stereo and the user can freely view, rotate, and otherwise interact with the patient's data in real time. The user is prompted to select anatomical landmarks on the patient's data for preoperative planning purposes, then their locations are compared against that of a 'gold standard' and a score, derived from their deviation from that standard and time required, is generated. The user can then move a haptic stylus and guide the motion of the virtual cutting tool. The soft tissues can thus be incised using this virtual cutting tool, moved using virtual forceps, and fused in order to perform any of the major procedures for cleft lip repair. Real-time soft tissue deformation of the mesh realistically simulates normal tissues and haptic-rate (>1 kHz) force-feedback is provided. The surgical result of the procedure can then be immediately visualized and the entire training process can be repeated at will. A short evaluation study was also performed. Two groups (non-medical and plastic surgery residents) of six persons each performed the anatomical marking task of the simulator four times. RESULTS: Results showed that the plastic surgery residents scored consistently better than the persons without medical background. Every person's score increased with practice, and the length of time needed to complete the 11 markings decreased. The data was compiled and showed which specific markers consistently took users the longest to identify as well as which locations were hardest to accurately mark. CONCLUSION: These findings suggest that the simulator is a valuable training tool, giving residents a way to practice anatomical identification for cleft lip surgery without the risks associated with training on a live patient. Educators can also use the simulator to examine which markers are consistently problematic, and modify their training to address these needs.
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ranking = 1
keywords = physical
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4/9. Submandibular cystic hygroma resembling a plunging ranula in a neonate. review and report of a case.

    Cystic hygromas are large lymphangiomas that are most often found in the posterior triangle of the neck and the axilla in children. They are most frequently found before age 2 and may be massive. After upper respiratory infection, they may become infected and enlarged, causing dysphagia and toxemia. The diagnosis can usually be made by history and physical examination and confirmed by biopsy. Treatment is by surgical excision of small lesions and staged debulking excisions in more severe cases. A patient with a cystic hygroma having many clinical characteristics of a plunging ranula is presented. The cyst fluid was aspirated and analyzed for its amylase, sodium, potassium, chloride, urea nitrogen, glucose, and total protein content. The characteristics of the fluid were also compared with those of lymph and saliva. This report demonstrates the difficulty in determining the diagnosis of a tumor that has the clinical features of a cystic hygroma, as well as a plunging ranula. The necessity of a proper presurgical diagnosis is essential since the form of therapy for each is different and conflicting. A method that distinguishes between the cervical cystic hygroma and a plunging ranula by means of aspirated fluid is discussed.
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ranking = 1
keywords = physical
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5/9. neural tube defects and omphalocele in trisomy 18.

    A trisomy 18 fetus with severe congenital anomalies including craniorachischisis, large omphalocele, and bilateral cleft lip and palate is reported. The occurrence of neural tube defects and/or omphalocele in reported cases of trisomy 18 is discussed and the frequency of these anomalies in 85 trisomy 18 patients evaluated at indiana University School of medicine from 1963 to 1986 is reviewed. In this series of patients the frequency of neural tube defects was 7.0% and the frequency of omphaloceles was 5.9%. The percentage of these findings in our cases supports the premise that neural tube defects and omphaloceles are part of the trisomy 18 phenotype. Since fetuses with trisomy 18 are subject to early fetal loss or premature birth, the more subtle physical features of this condition may not be apparent. Thus, karyotyping of fetuses and premature infants with either neural tube defect or omphalocele should be considered.
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ranking = 1
keywords = physical
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6/9. Popliteal pterygium syndrome.

    A syndrome of popliteal pterygium, cleft lip-palate, lower lip pits, eyelid adhesions, genito-urinary anomalies and digital anomalies is presented as the Popliteal pterygium syndrome. The hereditary pattern appears to be an autosomal dominant trait with incomplete penetrance and variable expressivity. The pediatric otolaryngologist should be alert to this syndrome and will be consulted for airway and feeding problems as well as speech and hearing management. Because of the wide variability of expression of popliteal pterygium syndrome, careful physical evaluation of available family members should be made in sporadic cases to confirm the diagnosis in those presenting with minor manifestations and to discover less severely affected relatives of those full expression. Appropriate genetic counseling can then be given to these families.
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ranking = 1
keywords = physical
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7/9. cleft lip and palate, corneal opacities and profound psychomotor retardation. A newly recognized genetic syndrome?

    Clinical histories and physical features of two sisters affected with a previously unreported syndrome are presented with illustrations. The manifestations were profound postnatal growth and psychomotor retardation, hydrocephaly, cleft lip and palate, corneal opacities, central nervous system impairment, and genitourinary anomalies. Four other siblings, two males and two females and the parents of the affected were examined and found to be normal. Infectious, metabolic, and chromosomal etiologies were excluded by appropriate studies. The most likely pattern of inheritance is autosomal recessive.
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ranking = 1
keywords = physical
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8/9. Scarring folliculitis in the ectrodactyly-ectodermal dysplasia-clefting syndrome. Histologic, scanning electron-microscopic and biophysical studies of hair.

    Several clinical syndromes are characterized by ectodermal dysplasia (ED) in association with clefting of the lip and/or palate. In these syndromes, alopecia is primarily due to abnormalities of the hair shaft associated with increased hair fragility. scalp dermatitis is yet another peculiar finding, primarily seen in the ankyloblepharon-ED-clefting (AEC) syndrome. We report on a 16-year-old patient with ectrodactyly-ED-clefting (EEC) syndrome, who exhibited a scarring alopecia due to deep folliculitis. On scanning electron microscopy, irregular torsion and longitudinal grooving of the hair shaft (pili torti et canaliculi) were observed. Quantitative determinations of the elastic and viscous parameters of hair demonstrated a normal viscosity but a significantly reduced hair elasticity, indicating either an abnormal composition or a disordered arrangement of microfibrils within the apparently normal keratin matrix. In contrast to the erosive scalp dermatitis of early onset in the AEC syndrome, alopecia in this case of EEC syndrome demonstrated follicular scarring with onset during puberty. We question a possible role of the anatomical hair abnormality in the pathogenesis of chronic deep folliculitis in this and clinically related syndromes.
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ranking = 4
keywords = physical
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9/9. A model for the cleft lip nasal deformity.

    The underlying pathology of the cleft lip nasal deformity has yet to be fully realized, and cleft lip rhinoplasty continues to challenge the reconstructive surgeon. A new model is proposed, which is composed of elements that represent known anatomical structures of the nose. These structures are considered elemental to the mechanism of the primary cleft lip nasal deformity. The lobule is reduced to four arches. Five points on the skull provide foundations for these arches, which react interdependently to extrinsic forces and positional change. When certain changes are imposed on the model, predictable alterations in the configuration of the model imitate the observed deformities in the spectrum of the cleft lip nasal deformity, unilateral and bilateral, mild through severe. The model is described with illustrations, anatomic dissection, physical models, and selected clinical cases. A better understanding of the mechanisms of the cleft nasal deformities can be obtained through analysis of the model.
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ranking = 1
keywords = physical
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