Siamese Twins With Craniofacial Duplication and Bilateral Cleft Lip/Palate in a Ceramic Representation of the Chimú Culture (Peru): A Comparative Analysis With a Current Case

2014 ◽  
Vol 17 (3) ◽  
pp. 211-214 ◽  
Author(s):  
Harry Pachajoa ◽  
Maria F. Hernandez-Amaris ◽  
Gloria Liliana Porras-Hurtado ◽  
Carlos A. Rodriguez

Craniofacial duplication or diprosopus is a very rare malformation that is present in approximately 0.4% of conjoined twins. Here is presented a case of craniofacial duplication in association with bilateral cleft lip/palate in both heads found in a ceramic representation from the early Chimú culture from Peru. A comparative analysis is made with a current case of a 28-week-old fetus with similar characteristics. After reviewing the medical literature on conjoined twins, very few reports of facial cleft in both twins were found, with no reports at all of bilateral cleft lip/palate. This ceramic crock is considered one of the first representations suggestive of craniofacial duplication, and probably the first reporting it in association with facial cleft.

2022 ◽  
pp. 1-8
Author(s):  
Liliana Fernández Hernández ◽  
Miguel A. Alcántara Ortigoza ◽  
Sandra E. Ramos Angeles ◽  
Ariadna González-del Angel

5q14.3 deletion syndrome (MIM#613443) is an uncommon but well-known syndrome characterized by intellectual disability, epilepsy, hypotonia, brain malformations, and facial dysmorphism. Most patients with this syndrome have lost one copy of the <i>MEF2C</i> gene (MIM*600662), whose haploinsufficiency is considered to be responsible for the distinctive phenotype. To date, nearly 40 cases have been reported; the deletion size and clinical spectrum are variable, and at least 6 cases without <i>MEF2C</i> involvement have been documented. We herein report the clinical and cytogenomic findings of an 11-year-old girl who has a 5q14.3q21.1 de novo deletion that does not involve <i>MEF2C</i> but shares the clinical features described in other reported patients. Moreover, she additionally presents with bilateral cleft-lip palate (CLP), which has not been previously reported as a feature of the syndrome. The most frequent syndromic forms of CLP were ruled out in our patient mainly by clinical examination, and Sanger sequencing was performed to discard the presence of a <i>TBX22</i> gene (MIM*300307) defect. Our report suggests CLP as a possible unreported feature and redefines the critical phenotypic regions of 5q14.3 deletion syndrome.


1999 ◽  
Vol 36 (5) ◽  
pp. 407-412 ◽  
Author(s):  
Alexander Gaggl ◽  
Günter Schultes ◽  
Hans Kärcher

Objective: To assess the aesthetic and functional long-term results of surgical and orthodontic treatment of patients with bilateral cleft lip, palate, and alveolus. Design: Long-term follow-up study. Setting: Teaching hospital in Austria. Patients: Twenty adult patients who had been operated on as children for bilateral cleft lip, palate, and alveolus. Interventions: Lateral cephalometric and model analysis. The sum of all mesiodistal tooth diameters in the maxilla and mandible were compared with standard Bolton tracings. Main outcome measures: Aesthetic and functional results. Results (model analysis): The upper arch was too wide in 12 patients and the mandibular arch was too wide in 4 patients. In 11 patients, the lateral teeth were crowded, and all had a persistent transverse space deficit and a reduction in sagittal measurements. Fifteen patients had alveolar midline displacement of the maxilla as well as of the mandible. Results (lateral cephalometric measurements): The lateral cephalograms showed a mean sella-nasion-A point angle of 77° and a maxillary baseline-nasion-sella line angle of 9°, indicating a tendency toward maxillary retrognathia. An anterior facial height index of 42% (compared with the standard 58%) indicated a slight reduction in midface height with consequent increase in the height of the lower face. Conclusion: There is specific growth impairment of the midface in adults who were treated as children for bilateral clefts of lip, palate, and alveolus. An optimal result can be achieved only by additional orthognathic surgery (Le Fort II osteotomy).


2016 ◽  
Vol 39 (2) ◽  
pp. 90-95 ◽  
Author(s):  
Amin Rahpeyma ◽  
Saeedeh Khajehahmadi ◽  
Ali Ghasemi

2013 ◽  
Vol 42 (10) ◽  
pp. 1197
Author(s):  
A. Ferrer Fuertes ◽  
E. García Díez ◽  
A. Rivera Baró ◽  
R. Sieira Gil ◽  
G.Y. Cho-Lee ◽  
...  

2017 ◽  
Vol 23 (2) ◽  
Author(s):  
Otto Madrid ◽  
Isabel-Cristina Jaramillo ◽  
Habib Barhoum

Clinical case of a 28 year old patient with bilateral cleft lip palate, who was treated during a period of 5 years and 4 months, at the University of Valle postgraduate orthodontic’s clinic of lip and palate, is presented. The patient had undershot jaw, severe mandibular macrognatismo, molar malocclusionclass I and class II right and left, accompanied by severe recumbent and mild retrusión of upper incisors and lower and, multiple malposition and impaction tooth 12. A non - surgical orthodontic plan compensation was developed and accompanied by an initial mandibular treatment, orthopedic expansion and corrective orthodontic treatment, using fixed appliances such as standard braces slot 0.22x0.028 inches, extractions of lower first premolars, typing canines as lateral incisors as well as rehabilitation of the upper front teeth. Satisfactory results were obtained in terms of aesthetic and functional occlusion. Key words: Cleft lip, cleft palate, orthodontic treatment of compensation, bilateral cleft lip and palate.


2014 ◽  
Vol 1 (1) ◽  
pp. 48
Author(s):  
DivyaNarain Upadhyaya ◽  
Vijay Kumar ◽  
Veerendra Kumar ◽  
Brijesh Mishra ◽  
ArunK Singh

2002 ◽  
Vol 39 (3) ◽  
pp. 376-379 ◽  
Author(s):  
Chung-Chih Yu ◽  
Fen-Hwa Wong ◽  
Lun-Jou Lo ◽  
Yu-Ray Chen

Objective: The association of cleft lip/palate (CLP) with other anomalies is not uncommon, but its association with Wilms tumor (WT) is very rare, especially in a familial pattern. In this report, we present a family in which six members in two generations were affected with CLP, WT, or both. Patients and Results: A male patient presented with right complete CLP. He had a family history of facial cleft and abdominal tumor. Lip repair was performed at 3 months of age. An abdominal mass was noticed at 12 months of age, which proved to be WT. Surgical excision of the tumor and chemotherapy were conducted. He subsequently underwent palate repair. His father had an unrepaired microform cleft lip. Three of his aunts were known to have similar problems: one had both facial cleft and WT, one had WT only, and the other had facial cleft only. One of his cousins also was affected with WT. Conclusions: This is a unique family affected with a rare association of CLP and WT. Pedigree study revealed an autosomal dominant hereditary pattern.


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