• Title/Summary/Keyword: Craniofacial malformation

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Concurrent presentation of porocarcinoma and basal cell carcinoma arising on a capillary malformation: a case report

  • Sunkyu Park;Jong-Ho Kim
    • Archives of Craniofacial Surgery
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    • v.24 no.5
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    • pp.236-239
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    • 2023
  • Porocarcinoma (PC) and basal cell carcinoma (BCC) are distinct skin cancers. Few studies have documented the occurrence of two concurrent types of skin cancers, and to the best of our knowledge, this represents the inaugural report of such a coexisting lesion arising from a capillary malformation. Herein, we report a case of concurrent PC and BCC presenting with capillary malformation. A 93-year-old woman visited our hospital with a protruding mass in her right nasal ala that appeared as a capillary malformation. A biopsy was performed on the skin lesion, and BCC was diagnosed. A wide excision was performed. Permanent biopsy revealed that the skin lesion was a PC with basal cells and squamous differentiation. The safety margin of the deep tissue margin was < 0.1 cm; however, considering the advanced age of the patient, further excision was deemed to not possess any benefits. This case illustrates the importance of recognizing the possibility of multiple skin cancers, even in patients with benign lesions such as capillary malformations. The rarity of this presentation highlights the importance of thorough investigation and histopathological examination of skin lesions in guiding appropriate surgical excision.

Reconstruction of the Face Using Thoracodorsal Artery Perforator Free Flap after Resection of Arteriovenous Malformation (안면부 동정맥 기형의 수술적 제거 후 흉배동맥 천공지유리피판을 이용한 결손의 재건)

  • Park, Bum Jin;Lim, So Young;Pyon, Jai Kyong;Mun, Goo Hyun;Bang, Sa Ik;Oh, Kap Sung
    • Archives of Craniofacial Surgery
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    • v.10 no.1
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    • pp.44-48
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    • 2009
  • Purpose: The treatment of arteriovenous malformation (AVM) of the face remains a difficult challenge in plastic surgery. Incomplete resection resulting in uncontrolled bleeding, postoperative enlargement of the remaining malformation, and a poor functional and cosmetic result could be the problems confronted by the surgeons. Methods: A 37 year-old male with large arteriovenous malformation in face treated with preoperative superselective transarterial embolization and free flap transfer. The size of the defect was $13{\times}9cm$. Sclerotheraphy without resection were performed several times but the results were unsatisfactory. Resection was performed the next day of embolization. We were able to repair with the thoracodorsal artery perforator free flap. And facial muscle reconstruction performed by simultaneous muscle and nerve transfer. Results: During the follow-up period 8 months the patient regained an acceptable cosmetic appearance. And he has shown no reexpansion of the malformation. Conclusion: The thoracodorsal artery perforator free flap could be a good choice for the reconstruction for massive defects of the face. A huge arteriovenous malformation could be safely removed and successfully reconstructed by the complete embolization, wide excision and coverage with a well vascularized tissue.

Intramuscular epidermal cyst in the masticator space: a case report

  • Sang Woo Han;Jiye Kim;Sug Won Kim;Minseob Eom;Chae Eun Yang
    • Archives of Craniofacial Surgery
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    • v.24 no.4
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    • pp.193-197
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    • 2023
  • An epidermal cyst, also known as an epidermoid cyst or epidermal inclusion cyst, is the most prevalent type of cutaneous cyst. This non-cancerous lesion can appear anywhere on the body, typically presenting as an asymptomatic dermal nodule with a visible central punctum. In the case presented herein, an epidermal cyst with uncommon features was misdiagnosed as a lymphatic malformation based on preoperative magnetic resonance imaging (MRI). A 61-year-old man came to us with a swollen left cheek that had been present for 11 months. The preoperative MRI revealed a 3×3.8×4.6 cm lobulated cystic lesion with thin rim enhancement in the left masticator space. The initial differential diagnosis pointed toward a lymphatic malformation. We proceeded with surgical excision of the lesion via an intraoral approach, and the specimen was sent to the pathology department. The pathological diagnosis revealed a ruptured epidermal cyst, indicating that the initial diagnosis of a lymphatic malformation based on preoperative MRI was incorrect. Epidermal cysts located under the muscle with no visible central punctum are uncommon, but should be considered if a patient presents with facial swelling.

Correction of Unilateral Nostril Hypoplasia with Z-Plasty in a Child

  • Lee, Dong-Han;Oh, Kap Sung
    • Archives of Craniofacial Surgery
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    • v.15 no.2
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    • pp.94-97
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    • 2014
  • Unilateral nostril hypoplasia is an extremely rare congenital malformation of unknown etiology, and only a few cases have been reported in literature. Owing to variability and complexity of the deformity, surgical correction of unilateral nostril hypoplasia represents one of the most significant reconstructive challenges to reconstructive plastic surgeons. We report a 7-year-old Vietnamese child with nasal and periocular deformity resembling a craniofacial cleft. Grossly, the right nostril was patent but with alar rim deformity, and the left nostril was not readily identifiable. A dystopic medial canthus was present on the left side as well. Closer inspection and palpation of the left side of nose revealed a patency through the soft tissue and underlying bony structure, Thus, a new alar rim were reconstructed with an irregularly shaped Z-plasty to create patency on the involved side. Simulatneously, a second Z-plasty was performed to address the medial canthal deformity. Postoperative appearance and function was sastisfactory at one-year follow up visit. In the treatment of patients with nostril hypoplasia, a careful preoperative physical examination is a prerequisite, and Z-plasty can be a valuable option for surgical correction.

A girl with sternal malformation/vascular dysplasia association

  • Lee, Na Yong;Cho, Hye Kyung;Kim, Kyung-Hyo;Park, Eun Ae
    • Clinical and Experimental Pediatrics
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    • v.56 no.3
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    • pp.135-138
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    • 2013
  • Sternal malformation/vascular dysplasia association is a rare congenital dysmorphology, which has not yet been reported in Korea. Its typical clinical features include a sternal cleft covered with atrophic skin, a median abdominal raphe extending from the sternal defect to the umbilicus, and cutaneous craniofacial hemangiomata. We report a case of a full-term newborn who presented with no anomalies at birth, except for a skin defect over the sternum and a supraumbilical raphe. Multiple hemangiomas appeared subsequently on her chin and upper chest wall, and respiratory distress due to subglottic hemangioma developed during the first 2 months of life. Her symptoms were controlled with oral prednisolone administration. No respiratory distress have recurred during the 3-year follow-up period.

Ectopic Preauricular Sinus in a Facial Cleft and Microtia Patient

  • Park, Jae Yeon;Lee, Seil;Kim, Hyo Joong;Jung, Sung Gyun
    • Archives of Craniofacial Surgery
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    • v.18 no.4
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    • pp.287-291
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    • 2017
  • Preauricular sinus is a congenital malformation that is very commonly encountered among the general population and it has especially high prevalence among Asians when compared to other ethnic groups. It can often go unnoticed or easily overlooked by the patient or even by doctors because most of them are asymptomatic and is most of the time only a tiny pit that can be trivial in terms of aesthetics. We report a very rare and unique case that has no precedence what so ever; hence no reported case in the literature: an ectopic preauricular sinus in a facial cleft and microsomia patient.

Clinical assessment and management of auricular arteriovenous malformation: retrospective study

  • Kim, Do Gon;Cho, Hyun Geun;Ryu, Jeong Yeop;Lee, Joon Seok;Lee, Seok Jong;Lee, Jong Min;Lee, Sang Yub;Huh, Seung;Kim, Ji Yoon;Chung, Ho Yun
    • Archives of Craniofacial Surgery
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    • v.22 no.3
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    • pp.141-147
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    • 2021
  • Background: Arteriovenous malformation (AVM) which is a high-blood-flow lesion with connections between arteries and veins without an intervening capillary bed, is difficult to manage. The ear is the second most common site of extracranial AVM. However, studies regarding the management of this condition remain lacking. The purpose of this study was to share managing experiences in our center and to investigate the treatment effect through a retrospective analysis of cases. Methods: Among 265 patients with AVM treated in our vascular anomalies center between January 2008 and January 2021, 10 patients with auricular AVM were included in the study to investigate the lesion distribution, clinical stage, and treatment methods by performing a retrospective evaluation. Results: Among 10 patients, five patients had AVMs distributed in the upper half of the ear, one patient in the lower half of the ear, and four patients in whole ear, respectively. Seven patients had Schobinger stage II, and three had stage III. One patient received surgical treatment only, four patients received sclerotherapy only, and five patients received both surgical treatment and sclerotherapy. The posttreatment status was checked as controlled in two patients, improved in seven patients, persistent in one patient. There were no worsening patients. Conclusion: Auricular AVM is a disease that is difficult to manage by one specific department, thus requiring a collaborative management effort from multidisciplinary team.

In Vivo Expression of EphrinA5-Fc in Mice Results in Cephalic Neural Crest Agenesis and Craniofacial Abnormalities

  • Noh, Hyuna;Park, Eunjeong;Park, Soochul
    • Molecules and Cells
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    • v.37 no.1
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    • pp.59-65
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    • 2014
  • Eph receptors and their ligands ephrins have been implicated in guiding the directed migration of neural crest cells (NCCs). In this study, we found that Wnt1-Cre-mediated expression of ephrinA5-Fc along the dorsal midline of the dien- and mesencephalon resulted in severe craniofacial malformation of mouse embryo. Interestingly, expression of cephalic NCC markers decreased significantly in the frontonasal process and branchial arches 1 and 2, which are target areas for the migratory cephalic NCCs originating in the dien- and mesencephalon. In addition, these craniofacial tissues were much smaller in mutant embryos expressing ephrinA5-Fc. Importantly, EphA7-positive cephalic NCCs were absent along the dorsal dien- and mesencephalon of mutant embryos expressing ephrinA5-Fc, suggesting that the generation of cephalic NCCs is disrupted due to ephrinA5-Fc expression. NCC explant experiments suggested that ephrinA5-Fc perturbed survival of cephalic NCC precursors in the dorsal midline tissue rather than affecting their migratory capacity, which was consistent with our previous report that expression of ephrinA5-Fc in the dorsal midline is responsible for severe neuroepithelial cell apoptotic death. Taken together, our findings strongly suggest that expression of ephrinA5-Fc decreases a population of cephalic NCC precursors in the dorsal midline of the dien- and mesencephalon, thereby disrupting craniofacial development in the mouse embryos.

Surgical Treatment of Polyotia

  • Rha, Eun Young;Kim, Dong Hwi;Byeon, Jun Hee
    • Archives of Craniofacial Surgery
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    • v.16 no.2
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    • pp.84-87
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    • 2015
  • Polyotia is an extremely rare type of the auricular malformation that is characterized by a large accessory ear. A 3-year-old girl presented to us with bilateral auricular abnormalities and underwent two-stage corrective operation for polyotia. In this report, we present the surgical details and postoperative outcomes of polyotia correction in the patient. Relevant literature is reviewed.