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illustration demonstrating the le Fort i osteotomy and distractor placement. a, internal telescopic distraction device is fixed to the zygomatic buttress and the hypoplastic maxilla in a slight downward direction. B, Following the active distraction phase, note the newly formed bone represented by the shaded area.
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Background:. Ectrodactyly ectodermal dysplasia-cleft syndrome is a rare genetic syndrome with an incidence of 1/90,000 live births, characterized by cleft lip and palate, severely hypoplastic maxilla, and hypodontia. Patients diagnosed with ectrodactyly ectodermal dysplasia-cleft syndrome suffer from a severely hypoplastic maxilla that is highly di...
Contexts in source publication
Context 1
... included were operated on according to the well-established protocol of cleft lip and palate reconstruc- tion, including lip and palate repair in infancy and early childhood and bone grafting to the cleft maxilla or alveo- lus between the ages of 8 and 11 years ( Fig. 2). Before the DO procedure, radiographic imaging including panoram- ic x-rays, lateral cephalograms, and cone beam computed tomography were obtained, followed by an evaluation of hard-and soft-tissue parameters (Fig. 3). The presurgi- cal evaluations, based on the aforementioned imaging methods, were carried out to assure adequate alveolar bone continuity, thus allowing a 1-piece maxilla osteotomy and mobilization (Fig. 4). According to the measurements performed on the clinical photographs, cephalograms and dental casts, the length and vector of elongation were planned, and distraction devices were ...
Context 2
... the maxillary distraction surgery was based on a Le-Fort I osteotomy according to Bell 11 and a fixa- tion of 2 bilateral internal distractors (KLS-Martin, Jack- sonville, Fla.). First, distraction appliances were adapted before the osteotomy with special attention to a forward and downward vector of elongation (Fig. 3). Second, due to a significant vertical loss of the maxillary bone, the max- illary osteotomy was confined between the infraorbital nerve superiorly and the dental roots (if existed) inside the atrophied alveolar crest inferiorly. The upper arms were then fixed to the zygomatic buttress, laterally and in- feriorly to the infraorbital nerve and the lower arms to the alveolar crest. Due to the thin demineralized bone of EEC patients, it is highly important to ensure a proper anchor- age of the intraoral devices, thus minimizing the risk of loosening hardware. The maxillary periosteum and mu- cosa were sutured leaving the bilateral activation rods in the maxillary vestibulum ready for activation (Fig. 5). Im- mediate postoperative radiographic images were obtained for all patients (Fig. ...
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Purpose:
Cleft lip and palate (CLP) patients often require orthognathic surgical correction due to maxillary hypoplasia secondary to primary surgeries, through either distraction osteogenesis (DO) or conventional orthognathic surgery (CO). The objective was to evaluate both surgical techniques regarding functional, aesthetics and quality-of-life a...
Citations
... To achieve normal facial proportions and intermaxillary relations, a marked maxillary advancement is required. 27 Due to ectrodactyly, maintaining acceptable oral hygiene is especially difficult for the patient, although by taking the time to correctly educate and motivate him it was possible to achieve a satisfactory condition even 2 years after the insertion. Pettit et al. also draw attention to the importance of oral hygiene education and prevention. ...
Aim:
Ectrodactyly-ectodermal dysplasia-cleft lip/palate (EEC) syndrome is a rare genetic disorder that affects ectodermal derived structures, including teeth, nails, hair, and sweat glands. Prosthetic rehabilitation of patients with EEC syndrome is essential towards improving their overall quality of life.
Methods and results:
In the case shown, a telescopic retained overdenture was made on the lower jaw based on digital impression of a patient with EEC syndrome associated with cleft lip and cleft palate. Due to the congenital anomalies and limited mouth opening, the impression was taken with intraoral scanner, and after designing the telescopes on the digital model, the primary and secondary telescopes were confectioned by selective laser sintering.
Conclusion:
Combining digital dental technology and conventional clinical prosthetic treatment methods, results in a well-functioning overdenture even in such complicated situations. The prosthodontic rehabilitation of patients with ECC helps to restore the masticatory and phonetic functions, increases the patient's self-esteem, and prevents further psychological trauma caused by hypodontia.
... Almost half of the KS patients have CPO, and those without an associated cleft have a high arched palate [115], micrognathia, and/or abnormal tongue movement [116]. Patients also had uvula bifida, lip pits, and nodules [115]. ...
Syndromes affecting craniofacial and dental structures account for one-third of the congenital anomalies. Early diagnosis and treatment guided by an interdisciplinary team are of vital importance not only for an optimal outcome but also because these syndromes are associated often with life-threatening conditions. However, the affected craniofacial structures isolated or as part of a syndrome might not be identified early, especially in patients with unknown family history. Many craniofacial malformations and cognitive phenotypes are apparent only after the first years of life. Moreover, the treatment of these patients is often complicated due to a lack of treatment guidelines and adequate medical knowledge. The role of the team is essential for the prevention, early diagnosis, and guidance for proper function and craniofacial development. Consideration of the patient’s developmental variations, deep phenotyping, and long-term clinical evaluation will assist in unraveling the phenotype-genotype correlations and further in an early diagnosis. Despite knowledge gaps and lack of consensus in terminology and diagnostic criteria, this chapter aims to provide an update on the pathogenesis, general phenotypes, and oral and craniofacial manifestations for early diagnosis and clinical management. Furthermore, its goal is to promote interaction of the interdisciplinary team for a customized, intergraded, long-term treatment planning of these patients.
... Orthodontic support and efficient treatment can form conditions for optimal maxillofacial growth while adapting favorable inter-jaw association [45]. Surgical approaches, which possibly can differ among patients with ED include orthognathic management, midfacial defects correction, cleft lip or/ and palate repair, and supportive augmentation procedure, with optimizing condition for future dental implants placement [46]. ...
... Rachimel et al. proposed time-structured treatment algorithm for ectrodactyly ED-cleft syndrome patients with severe atrophic mandible, which included next stages [46] (Table 2). ...
... Time-structured treatment algorithm for ectrodactyly ectodermal dysplasia-cleft syndrome patients proposed by Rachimel et al.[46] ...
Distraction osteogenesis (DO) is a surgical technique that takes advantage of natural wound healing mechanisms to augment bone and soft tissues. DO is extremely versatile and can be applied to nearly any bone. In the craniofacial skeleton, the cranial vault, midface, maxilla and mandible are the most common sites for DO. This technique allows larger skeletal movements than could be achieved with conventional techniques, decreases operative time and blood loss, eliminates the need for bone grafts and associated donor site morbidity, and may improve postoperative stability. DO can be used in preparation for, in lieu of, or in combination with orthognathic surgery to correct dentofacial deformities. DO requires meticulous planning to achieve optimal results.
