• Archives of Plastic Surgery
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• v.43 no.3
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• pp.242-247
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• 2016

Background Median cleft lip is a rare anomaly consisting of a midline vertical cleft through the upper lip. It can also involve the premaxillary bone, the nasal septum, and the central nervous system. In our current report, we present the clinical features of 6 patients with a median cleft lip and their surgical management according to the accompanying anomalies. Methods From December 2010 to January 2014, 6 patients with a median cleft lip were reviewed. Five of these cases underwent surgical correction; alveolar bone grafting was performed in a patient with a median alveolar cleft. The surgical technique included inverted-U excision of the upper lip and repair of the orbicularis oris muscle. The mean follow-up period was 20.4 months (range, 7.4-44.0 months). Results The study patients presented various anomalous features. Five patients received surgical correction, 4 with repair of the median cleft lip, and one with iliac bone grafting for median alveolar cleft. A patient with basal sphenoethmoidal meningocele was managed with transoral endoscopic surgery for repair of the meningocele. Successful surgical repair was achieved in all cases with no postoperative complications. Conclusions Relatively mild forms of median cleft lip can be corrected with inverted-U excision with good aesthetic outcomes. In addition, there is a broad spectrum of clinical features and various anomalies, such as nasal deformity, alveolar cleft, and short upper frenulum, which require close evaluation. The timing of the operation should be decided considering the presence of other anomalies that can threaten patient survival.

• Archives of Craniofacial Surgery
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• v.20 no.6
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• pp.397-400
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• 2019

Frontonasal dysplasia is an uncommon congenital anomaly with diverse clinical phenotypes and highly variable clinical characteristics, including hypertelorism, a broad nasal root, median facial cleft, a missing or underdeveloped nasal tip, and a widow's peak hairline. Frontonasal dysplasia is mostly inherited and caused by the ALX genes (ALX1, ALX3, and ALX4). We report a rare case of a frontonasal dysplasia patient with mild hypertelorism, a broad nasal root, an underdeveloped nasal tip, an accessory nasal tag, and a widow's peak. We used soft tissue re-draping to achieve aesthetic improvements.

• Archives of Plastic Surgery
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• v.46 no.6
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• pp.518-524
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• 2019

Background Anterior palatal repair performed during cleft lip repair using a vomerine flap may assist in recruiting additional soft tissue for subsequent completion of palatoplasty, especially in patients with a wide cleft. We present our early results in the hope of triggering a re-evaluation of this technique regarding its advantages for maxillary growth through further studies of patients with a wide cleft. Methods A retrospective analysis of patients with complete unilateral and bilateral cleft lip and palate was performed, including cleft and palatal measurements taken during initial surgery (lip repair together with anterior palate repair) and upon completion of palatoplasty. Results In total, 14 patients were included in this study, of whom nine (63.3%) had unilateral cleft lip and palate and five (37.5%) had bilateral cleft. All patients had a wide cleft palate. Lip and anterior palate repair was done at a median age of 3 months, while completion of palatoplasty was done at a median age of 10.5 months. Measurements taken upon completion of palatoplasty showed significant cleft width reduction in the mid-palate and intertubercle regions; however, the palatal arch distances at nearby landmarks showed non-significant marginal changes. Conclusions Anterior palate repair using a vomerine flap significantly reduced the remaining cleft width, while the palatal width remained. Further research is warranted to explore the long-term effects of this technique in wide cleft patients in terms of facial growth.

• Archives of Plastic Surgery
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• v.34 no.4
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• pp.528-530
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• 2007

Purpose: Holoprosencephaly(HPE) is a rare developmental defect due to incomplete cleavages of the prosencephalon during the third week of fetal development. Chromosomal anomalies, genetic syndrome, teratogen, or genetic disorder of non-syndromic HPE are usually accepted as etiology. The consequences of prechordal mesoderm defect are varying degrees of deficit of midline facial development, especially the median nasal process(premaxilla), and incomplete morphogenesis of the forebrain. We experienced a case of lobar HPE with complete cleft lip and palate. Methods: A female newborn infant was born at $38^{+6}$ weeks' gestational age via NSVD. The infant's birth weight was 3.6 kg, height 52 cm, and head circumference 32.5 cm, showing microcephaly, flat nose, median complete cleft lip & palate, and hypotelorism, along with defects of midfacial development including losses of premaxilla, philtrum, nasal septum, and columella. Results: There were no specific findings noted from the head and neck X-ray and tests for endocrine and metabolic disorders, but clinical characteristics of midface and dysgenesis corpus callosum on brain MRI were seen, so that this case was diagnosed with HPE. Conclusion: HPE is divided into three categories of alobar, semilobar, and lobar prosencephaly according to the degree of cerebral hemisphere separation. Assesment of patient's brain abnormality and malformation is essential in determining the extent and benefit of surgical intervention. This case was included in the lobar type HPE which shows relatively good prognosis compared with other types and reconstruction of median complete cleft lip & palate and midfacial defects will be performed.

• Korean Journal of Cleft Lip And Palate
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• v.10 no.2
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• pp.67-74
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• 2007

After Tennison introduced a triangular flap method which, for the first time, preserved the Cupid's bow, Randall gave this method a sound mathematical basis. This method is also called as an inferior triangular cheiloplasty which is characterized by making a small triangular flap from the lateral border of cleft destined to be fitted into an incision on the medial side of cleft. He postulated that the height obtained was equal to the sum of the median of the two triangles used in the cheiloplasty. Using this technique, a 22 month-old male patient with incomplete unilateral cleft lip was corrected primarily. The deviation of the columella and flattening of the nostril on the cleft side were minimal. The operation was done under general anesthesia and patient was healed uneventfully. We tried to improve the symmetry and esthetic feature of philtrum, nostril sill, alar-facial groove, preventing the notch formation on the nostril floor, and to reconstruct the muscle sling in the upper part of lip. The shape of Cupid's bow was restored, and the symmetry of columella was regained as a result. In summary, the inferior triangular cheiloplasty is effective to correct the primary unilateral cleft lip, results in the restoration of favorable anatomy and function.

• Archives of Craniofacial Surgery
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• v.24 no.2
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• pp.59-65
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• 2023

Background: Fibrous dysplasia (FD) is a localized bone disorder in which fibro-osseous tissue replaces the normal bone structure. Patients with craniofacial FD often present with gradual swelling, deformity, and compromised vision or hearing. We previously introduced "the core extirpation method," a novel surgical technique that is minimally invasive like traditional bone shaving but has longer-lasting effects. This study presents the long-term outcomes of our core extirpation method. Methods: We conducted a retrospective analysis of patients who underwent core extirpation for FD of the zygomaticomaxillary region from 2012 through 2021. Computed tomography (CT) scans were performed 6 to 12 months before the operation, immediately before and after the operation, and during follow-up visits. We performed all operations using the upper gingivobuccal approach, and we extirpated the core of the lesion while preserving the cortical structures of the zygoma and the maxilla to maintain symmetrical facial contour. Results: In 12 patients with lesions in the growth phase, anteroposterior/mediolateral (AP/ML) length discrepancies and the volume increased between preoperative and immediate postoperative CT scans. All patients' immediate postoperative AP/ML discrepancies were stable up to 12-17 months postoperatively. Postoperative volume showed continuous lesion growth; the median volume growth rate was 0.61 cc per month. Conclusion: In this article, we present our experiences managing FD using the minimally invasive core extirpation technique, which entails small expected blood loss and can be performed as day surgery. It provides similar cosmetic outcomes as traditional bone shaving but with longer-lasting results. Although there are some limitations with the study's retrospective nature and small sample size, our 4-year follow-up results show promising results of the core extirpation method in well-indicated patients.