[Show abstract][Hide abstract] ABSTRACT: 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.
Twin Research and Human Genetics 04/2014; 17(3):1-4. DOI:10.1017/thg.2014.20 · 1.92 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Objectives: Conjoined twins are rare. High-quality imaging techniques are essential for proper first-trimester diagnosis. Technological development leads to new imaging techniques such as 3-dimensional virtual embryoscopy. The aim of this review was to explore imaging techniques used in the first-trimester diagnosis of conjoined twins and provide a systematic diagnostic table for making this diagnosis. Design: A PubMed literature search was performed using the terms ultrasound, Doppler, MRI, and CT combined with first-trimester and conjoined twins. Three recent cases at our department are reviewed and examined additionally using 3-dimensional virtual embryoscopy. Results: The different types of conjoined twins are summarized in a table for practical use during ultrasound examination. In evaluating conjoined twins, 2-dimensional ultrasound is the criterion standard. Three-dimensional and Doppler ultrasounds add anatomical and prognostic information. Virtual embryoscopy imaging reveals additional findings in our 3 cases not seen with routine 2-dimensionalultrasound examination. Conclusions: Each case of conjoined twins is unique and should be evaluated with the best possible imaging techniques. Three-dimensional and Doppler ultrasound should be added to the systematic diagnostic evaluation of conjoined twins. Virtual embryoscopy imaging may contribute to earlier, more appropriate counseling and management of these pregnancies.
[Show abstract][Hide abstract] ABSTRACT: Diprosopus (Greek; di-, "two" + prosopon, "face"), or craniofacial duplication, is a rare craniofacial anomaly referring to the complete duplication of facial structures. Partial craniofacial duplication describes a broad spectrum of congenital anomalies, including duplications of the oral cavity. This paper describes a 15 month-old female with a duplicated oral cavity, mandible, and maxilla. A Tessier type 7 cleft, midline meningocele, and duplicated hypophysis were also present. The preoperative evaluation, surgical approach, postoperative results, and a review of the literature are presented. The surgical approach was designed to preserve facial nerve innervation to the reconstructed cheek and mouth. The duplicated mandible and maxilla were excised and the remaining left maxilla was bone grafted. Soft tissue repair included closure of the Tessier type VII cleft. Craniofacial duplication remains a rare entity that is more common in females. The pathophysiology remains incompletely characterized, but is postulated to be due to duplication of the notochord, as well as duplication of mandibular growth centres. While diprosopus is a severe deformity often associated with anencephaly, patients with partial duplication typically benefit from surgical treatment. Managing craniofacial duplication requires a detailed preoperative evaluation as well as a comprehensive, staged treatment plan. Long-term follow up is needed appropriately to address ongoing craniofacial deformity.
Journal of cranio-maxillo-facial surgery: official publication of the European Association for Cranio-Maxillo-Facial Surgery 08/2013; DOI:10.1016/j.jcms.2013.05.016 · 1.25 Impact Factor
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