Cases reported "Cleft Palate"

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1/12. Maxillary distraction for the management of cleft maxillary hypoplasia with a rigid external distraction system.

    Maxillary hypoplasia is a common finding in patients with repaired orofacial clefts. Management of this condition has been a challenge to the reconstructive team. The introduction of distraction osteogenesis to treat craniofacial skeletal dysplasias has opened alternative approaches to manage these severe conditions. In this article, the authors present their technique to distract the hypoplastic cleft maxilla using a rigid external distraction device. The clinical assessment, indications, orthodontic procedure, surgical technique, and distraction protocol are reviewed. A case report shows the use of the technique. This technique allows the reconstructive team to treat patients in all age groups with predictable and stable results.
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2/12. Prosthetic reconstruction in the cleft lip and palate patient with an extracoronal resilient attachment retained removable partial overdenture: case report.

    There are still some difficulties in prosthetic reconstruction of cleft lip and palate patients with conventional prostheses or implant retained prostheses. The most common difficulties are insufficient alveolar bone quality and quantity, inadequate soft tissue, and abutment teeth. The patient we report on was a 23-year-old man with a clinical diagnosis of right incomplete cleft lip and palate combined with midface dysplasia. The maxillary six anterior teeth were reconstructed. The maxillary right central incisor and canine were used as abutments for an extracoronal resilient attachment (ERA) retained removable partial overdenture. The STERN ERA SYSTEM is a hinged resilient attachment with an ideal stress breaking characteristic, a good retentive function, and easy chairside replacement. The 2-year follow-up examination revealed an adequate esthetic appearance with good retention and stability of the prosthesis. A removable partial overdenture using the teeth adjacent to the cleft area as abutments with an adequate attachment design is an alternative method for prosthetic reconstruction of cleft lip and palate deformity.
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3/12. rehabilitation of a patient with cleft lip and palate with an extremely edentulous atrophied posterior maxilla using zygomatic implants: case report.

    OBJECTIVE: This case report describes the clinical and surgical management of a patient with a unilateral alveolar cleft and associated extremely atrophied totally edentulous maxilla. METHOD: Two zygomatic implants and four endosseous oral implants were placed under general anesthesia in a compromised maxilla to rehabilitate a 33-year-old patient with cleft lip and palate. The two specially designed zygomatic implants were utilized to avoid the need for bone grafting in the patient. The final prosthetic rehabilitation was an esthetic and functional maxillary overdenture prosthesis supported by implants. RESULTS: Preliminary results have shown how dental prostheses supported by endosseous implants in grafted alveolar cleft are a reliable possibility in the dental rehabilitation of this malformation. CONCLUSION: The use of zygomatic implants may be considered a reliable alternative to more resource-demanding techniques such as bone grafting in patients with cleft palate.
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4/12. A new case of 2q duplication supports either a locus for orofacial clefting between markers D2S1897 and D2S2023 or a locus for cleft palate only on chromosome 2q13-q21.

    We report on a pure duplication of the proximal chromosome 2q in a 6.5-year-old boy with V-shaped midline cleft palate and bifid uvula, posteriorly located tongue, and micrognathia (Pierre Robin sequence), celiac disease, failure to thrive, and developmental delay. Cytogenetic and FISH analysis indicated a duplication of chromosome 2q13-q22. In general, pure proximal duplication or triplication of 2q is rare. The clinical features and chromosomal breakpoints of the 10 previously reported patients varied, and no common phenotype or proximal duplication/triplication 2q syndrome could be defined to date. However, based on four previous patients with different orofacial clefts and our case, a locus for orofacial clefting may be located at proximal 2q. The duplication/triplication comprised chromosome 2q13 in all five affected individuals including our patient. Our patient and three previous cases (two with cleft palate only (CPO) and one with cleft lip/palate (CL/P)) showed a cytogenetic breakpoint at 2q13, which could support the presence of a critical dominant gene disrupted by a common breakpoint, however, the fifth case with CPO showed different breakpoints, advocating against the disruption of a critical dominant gene and supporting that the overexpression of a gene(s) on chromosome 2q13-q21 may cause cleft palate only (CPO) and Pierre Robin sequence. Hence, our findings support either the presence of one locus for orofacial clefting (CL/P, CPO, and Pierre Robin sequence) between markers D2S1897 (chromosome 2q12.2) and D2S2023 (chromosome 2q14.2), or alternatively the presence of a locus for CPO and Pierre Robin sequence on chromosome 2q13-q21.
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5/12. The use of overdentures in children with cleft lip and palate: a report of two cases.

    The treatment of children with cleft lip and palate is complex. Advances in primary surgery and the advent of alveolar bone grafting have resulted in the reduction of prosthetic intervention. However, in patients where surgery is contraindicated or has been unsuccessful, an alternative treatment is required. Overdentures are a simple, conservative and reversible non-surgical alternative for children with cleft lip and palate. We present two cases with severe complete bilateral cleft lip and palate that were managed in this way.
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6/12. Vector alignment in maxillary distraction osteogenesis.

    Maxillary distraction osteogenesis is an alternative treatment of cleft patients with severe maxillary hypoplasia. The aim of this paper is to present the combined surgical/orthodontic treatment of a cleft lip and palate patient and to evaluate the maxillary distraction procedure and the distraction vector in high Le Fort I osteotomy.
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7/12. Provisional restoration for a patient with cleft lip and palate: a clinical report.

    A method for designing and fitting a provisional restoration for a patient with a cleft lip and palate was described. The provisional prosthesis provides an alternative treatment option that allows the dentist to plan the definitive restoration while providing the patient with a temporary but esthetic and functional restoration.
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8/12. Oblique facial clefts: pathology, etiology, and reconstruction.

    Modern views on embryology have increased our understanding of the nature of oblique facial clefts. The anomalies that have their origin at the junction of facial processes, such as the nasomaxillary dysplasias, may be named primary clefts or transformation. The maxillary clefts that are due to a developmental arrest of the skeleton are in fact secondary defects of differentiation defects. The teratology of these malformations is discussed, and attention is drawn to the amniotic rupture syndrome as a possible cause. All these clefts are rare, their incidence ranging from 0.75 to 5.4 per 1000 common clefts. This author has been involved in the treatment of nine of these patients. Four had their malformation reconstructed with one of the conventional procedures described in the literature, but the results, although initially acceptable, soon deteriorated. A more aggressive approach was therefore chosen. rotation and advancement of the cheek proved to be extremely effective and is now advocated as the procedure of choice. The transposition of a median forehead flap is considered an excellent alternative. Use of these procedures in five patients is reported. There were no complications.
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9/12. Alternative technique of constructing bilateral cleft palate in late childhood: a case report and literature review.

    Although great advances in treatment of oro-facial clefts have been made over the past 50 years; in developing countries, due to social stigmas and unavailability of specialised medical facilities and personnel to the majority of the population, there is an increasing incidence of patients coming late for repair of oro-facial clefts. This presents a challenge to both plastic, oral surgeons and associated specialists. The aim of the present article is to demonstrate, in the form of a case report, an alternative surgical technique that has be successful in 8 cases for the late repair of severe bilateral cleft palate utilizing locally available and affordable materials. The objective of managing patients with cleft palate in developing countries is to provide the best repair and rehabilitation with as few operations and in-patient care as possible due to limited financial resources experienced by the majority of in-patients. A multidisciplinary approach to the management of oro-facial clefts is emphasised.
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10/12. EC syndrome in a girl with paracentric inversion (7)(q22.1;q36.3).

    Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome associated with a paracentric inversion of chromosome 7 in a 2-year-old Japanese girl is reported. She had sparse and light-brown hair, bilateral cleft lip and palate, fused lower incisors, a pigmented skin lesion at the neck, accessory nipples, limited extension of elbow joints and bilateral ectrodactyly of hands and feet. Cytogenetic studies demonstrated a balanced inv(7)(q22.1;q36.3) in the patient and her father. The association of EEC syndrome and inv(7) in the patient suggested a putative locus of the EEC syndrome gene either at 7q22.1 or 7q36.3, although a coincidental occurrence of the two conditions is an alternative explanation. A comparison with reported karyotypes in patients with EEC or isolated ectrodactyly favoured 7q22.1 as the locus. A normal phenotype of the father in our family might reflect reduced penetrance of the EEC syndrome or, possibly, reduced expression of a maternally-derived allele of the EEC syndrome gene through a genomic imprinting mechanism.
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