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1.
J Plast Reconstr Aesthet Surg ; 75(12): 4478-4483, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36229313

RESUMO

PURPOSE: Accessory columellas are rare congenital anomalies characterized by skin appendage in the columella of the nostril. Case reports are scattered, but there are few descriptions about the clinical features and surgical course. METHOD: In this study, 3 patients with 4 lesions were identified (2013-2020). They were morphologically classified, and the accompanying nose deformity, surgical procedure, and postoperative course were examined. RESULTS: According to the morphologic classification, 1 lesion was of the sessile-lobed type, 2 lesions were of the sessile-nodular type, and 1 lesion was of the pedunculated-ovoid type. In terms of accompanying nose deformities, 1 lesion had a wide nasal columella, and 1 lesion had an enlarged left nostril due to a depression at the base of the lesion. Simple ablations were performed in 2 of the lesions, and plastic procedures were performed in the 2 lesions with an accompanying nose deformity. CONCLUSION: As in our cases, accessory columellas may have a variety of appearances and accompanying deformities. The surgical procedure must be considered according to the case. In addition, any changes due to growth must be observed and taken into consideration when they are reoperated.


Assuntos
Fenda Labial , Doenças Nasais , Rinoplastia , Humanos , Rinoplastia/métodos , Fenda Labial/cirurgia , Nariz/cirurgia , Nariz/anormalidades , Septo Nasal/cirurgia , Doenças Nasais/cirurgia , /métodos
3.
Appl Immunohistochem Mol Morphol ; 30(9): 635-639, 2022 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-36093893

RESUMO

Trichorhinophalangeal syndrome (TRPS) is an extremely rare autosomal dominant multisystem disorder characterized by craniofacial and skeletal abnormalities. Three subtypes of TRPS have been described: TRPS type I, TRPS type II, and TRPS type III. Mutations in the TRPS1 gene can cause both TRPS type I and TRPS type III. Therefore, the genotype-phenotype correlation is crucial to determine the subtype. The current family study from Cyprus involves affected patients from 4 generations who presented with alopecia, unoperated umbilical hernia, caput quadratum, long philtrum, depressed nasal bridge, frontal bossing, pes planus, beaked nose, and some deformities in hands and feet. Sequence analysis of the TRPS1 gene revealed a novel c.2854_2858del (p.Asn952ArgfsTer2) frameshift variant leading to a premature stop codon. To the best of our knowledge, we report here the first case of a Turkish Cypriot family of 4 generations with a novel frameshift mutation leading to truncated protein in the TRPS1 gene causing TRPS type I clinical phenotype. Overall, as the genotype and phenotype correlation in TRPSI is still uncertain and complex, the present outcome can enhance our knowledge of this complicated, rare, and severe genetic disorder.


Assuntos
Códon sem Sentido , Mutação da Fase de Leitura , Proteínas de Ligação a DNA/genética , Dedos/anormalidades , Doenças do Cabelo , Síndrome de Langer-Giedion , Nariz/anormalidades , Proteínas Repressoras/genética , Fatores de Transcrição/genética
4.
J Craniofac Surg ; 33(6): 1869-1874, 2022 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-36054892

RESUMO

BACKGROUND: Patients with alveolar cleft unrepaired suffer from nasal deformities of different magnitude. Bone and cartilage grafts are harvested through several incisions. In this study, we present a method to simultaneously correct nasal deformities and repair alveolar cleft using grafts from the nasal septum. PATIENTS AND METHODS: All 6 patients with unilateral cleft lip and palate have alveolar cleft unrepaired combined with nasal deformity. Computed tomography scans and 3-dimensional-printed models of vomer and ethmoid bone were used for the purpose of preoperative design and for assessing the magnitude of deformity. Grafts of bone and cartilage from deviated septum were harvested by septoplasty through which dorsum deviation was corrected. Bone grafts from vomer and ethmoid were then fixed to the prepared alveolar cleft to repair the defect and elevate the alar base. Septal cartilage was adjusted into different shapes of grafts and deformities of nasal tip, nostrils, and columella were then corrected by rhinoplasty to restore the symmetry of the nose. RESULTS: Symmetry of nostrils was improved. The height of alar base on the cleft side was elevated to the level close to the noncleft side. Deviation of the septum, nasal dorsum, and columella was corrected. Projection of the nasal tip was adjusted to facial midline. Midface aesthetics was generally improved. CONCLUSION: Application of septal grafts reduce the number of incisions. One-stage repair of alveolar cleft and nasal deformities, with the aid of digital design, improves the postoperative experience and the general outcome of the surgery.


Assuntos
Fenda Labial , Fissura Palatina , Doenças Nasais , Rinoplastia , Cartilagem/transplante , Fenda Labial/diagnóstico por imagem , Fenda Labial/cirurgia , Fissura Palatina/diagnóstico por imagem , Fissura Palatina/cirurgia , Estética Dentária , Osso Etmoide/cirurgia , Humanos , Septo Nasal/cirurgia , Septo Nasal/transplante , Nariz/anormalidades , Nariz/cirurgia , Doenças Nasais/cirurgia , Rinoplastia/métodos , Resultado do Tratamento , Vômer/cirurgia
5.
J Hand Surg Asian Pac Vol ; 27(4): 742-746, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-35965362

RESUMO

Laurin-Sandrow syndrome (LSS) is an extremely rare syndrome of mirror hand and leg with less than 20 cases reported in literature. The syndrome has been attributed to a mutation in the MIPOL-1 (mirror-image polydactyly) gene located on locus 14q13.3-q21 coding for CCDC193 (coiled-coli domain containing 193) protein. It is characterised by limb, facial and central nervous system anomalies with the most constant being fibular dimelia with fibular ray duplication, polydactyly with secondary deformities of fixed equinus, knee joint instability and flexion deformity. It is associated less frequently with ulnar dimelia, thumb aplasia/hypoplasia, ulnar ray duplication, symbrachypolydactyly, 'rosette' hands, facial dysmorphism like hypertelorism, broad columella and flat nose, CNS anomalies like aplasia/hypoplasia of corpus callosum, hydrocephalus and muscular dystonia. We report a 2-year-old male child with LSS and perform a literature review to expound on this rare syndrome. Level of Evidence: Level V (Therapeutic).


Assuntos
Anormalidades Múltiplas , Ectromelia , Deformidades Congênitas do Pé , Deformidades Congênitas da Mão , Anormalidades Múltiplas/genética , Criança , Pré-Escolar , Deformidades Congênitas do Pé/genética , Humanos , Masculino , Nariz/anormalidades , Síndrome
6.
J Craniofac Surg ; 33(8): 2513-2521, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35901453

RESUMO

BACKGROUND: Cleft rhinoplasty is a challenging form of nasal correction of both esthetic and functional deformities. The septal cartilage in many Asian patients are not sufficient and weak. Does a combination of the septal cartilage and the bony septum have both esthetic and functional benefits to secondary unilateral cleft rhinoplasty? PATIENTS AND METHODS: Thirty patients with a unilateral cleft lip palate underwent open rhinoplasty from October 2018 to January 2021. After preserving a 10 mm L-strut, the posterior cartilaginous and bony septum were harvested as an integrative unit. The osteocartilaginous graft was used as a caudal septal extension graft and an extended spreader graft. Correcting the asymmetry of the tip and tip projection followed. The intraoperative harvested composite graft was analyzed. Acoustic rhinometry and the 3-dimensional anthropometric measurements of the external nose were assessed before and after surgery. RESULTS: The osteocartilaginous unit was much larger than the cartilaginous part of this unit. The mean nasal tip height and the nasolabial angle increased significantly after surgery. The measurement of cross-sectional areas and volumes by acoustic rhinometry revealed that septorhinoplasty provided a significant increase in the function of both nasal cavities. CONCLUSIONS: This septal bony cartilaginous graft is effective for cleft lip nasal deformity when correcting the deviated septum, creating a supporting frame to correct the nasal tip asymmetry, improving function.


Assuntos
Fenda Labial , Fissura Palatina , Rinoplastia , Humanos , Rinoplastia/métodos , Fenda Labial/cirurgia , Septo Nasal/cirurgia , Estética Dentária , Nariz/cirurgia , Nariz/anormalidades , Fissura Palatina/cirurgia , Cartilagem/transplante , Transplante Ósseo , Resultado do Tratamento
7.
BMC Surg ; 22(1): 227, 2022 Jun 13.
Artigo em Inglês | MEDLINE | ID: mdl-35698063

RESUMO

BACKGROUND: Supernumerary Nostril, also called triple nostrils or accessory nostril, is a rare congenital nasal malformation. CASE PRESENTATION: We report one conceal case of supernumerary nostril in a 19-years-old men which is misdiagnosed to a simple small nasal skin pit. Ordinary surgical excision led to recurrent infection of the lesion postoperatively, and was eventually required secondary surgery and the lesion was finally confirmed by pathological biopsy as a trinasal nostrils. CONCLUSIONS: Through this case, we stress the essential role in differential diagnosis, confirming the diagnosis and seeking for better solutions. Level of Evidence V.


Assuntos
Nariz , Rinoplastia , Adulto , Biópsia , Erros de Diagnóstico , Humanos , Masculino , Nariz/anormalidades , Nariz/cirurgia , Pele , Adulto Jovem
8.
J Plast Reconstr Aesthet Surg ; 75(7): 2387-2440, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-35525781

RESUMO

BACKGROUND: Isolated ala nasi cleft is a very rare anomaly that causes significant esthetic deformity. This anomaly is usually managed in pediatric patients, and the residual deformity is corrected as much as possible in additional stages. Adult patients typically seek management of concomitant nasal deformities that require standard septorhinoplasty. METHOD: In this article, we have reported four adult patients with isolated ala nasi cleft. They were treated with a novel technique for the management of alar deformity and simultaneous nasal dysmorphia. RESULTS: Patients treated had uneventful primary healing with no significant complaints regarding the shape of the nose and the scar. CONCLUSION: Providing an esthetic and anatomic reconstruction with this technique seems to be a useful option for reconstruction of the isolated ala nasi cleft.


Assuntos
Fenda Labial , Doenças Nasais , Rinoplastia , Adulto , Criança , Fenda Labial/complicações , Fenda Labial/cirurgia , Humanos , Cartilagens Nasais/cirurgia , Nariz/anormalidades , Nariz/cirurgia , Doenças Nasais/cirurgia , Rinoplastia/métodos
9.
J Plast Reconstr Aesthet Surg ; 75(8): 2796-2801, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35562289

RESUMO

BACKGROUND: Nasal tip management is considered to be one of the most complicated and challenging parts of rhinoplasty in East Asian patients. Within current rhinoplasty surgical practice, costal cartilage serves as an ideal source for grafting due to its ability to provide strong support to the nasal tip. METHODS: From March 2013 to December 2021, integrated clip-shaped costal cartilage grafts were applied to patients with primary (n = 12) or secondary (n = 3) short nose deformities. The costal cartilage was carved into an monobloc clip-shaped cartilage graft and then placed on the nasal septum and fixed with sutures. The nasal length index, projection index, and nasolabial angles were measured preoperatively and postoperatively. Postoperative evaluation at 8 months was performed using a grading scale. RESULTS: A total of 15 patients were included in this study with an average age of 23.7 ± 3.6 years. The mean follow-up period was 14.3 ± 5.3 months and ranged from 8 to 26 months. There were statistically significant differences between the preoperative and postoperative values in nose length index and projection index. Also, the nasolabial angle was significantly reduced. Postoperative evaluation, conducted at a minimum of 8 months following surgery, showed that 86.6% (13/15) of patients felt that their surgical results were good or excellent. No patients rated the results as "poor". CONCLUSION: The integrated clip-shaped costal cartilage carving approach has been shown to be a practical method of obtaining satisfactory esthetic outcomes in patients with nasal deformities.


Assuntos
Cartilagem Costal , Deformidades Adquiridas Nasais , Doenças Nasais , Rinoplastia , Adulto , Cartilagem Costal/transplante , Humanos , Septo Nasal/cirurgia , Nariz/anormalidades , Nariz/cirurgia , Deformidades Adquiridas Nasais/cirurgia , Doenças Nasais/cirurgia , Estudos Retrospectivos , Rinoplastia/métodos , Instrumentos Cirúrgicos , Adulto Jovem
10.
Aesthetic Plast Surg ; 46(4): 1731-1737, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35451608

RESUMO

BACKGROUND: Dento-maxillofacial deformities are often associated with nasal deviation, and patients often complain of nasal deviation after orthognathic surgery. This study aimed to quantitatively evaluate the facial visual attention given to dento-maxillofacial deformities accompanying nasal deviation from the perspective of patients and determine whether orthognathic surgery could alter this outcome. METHODS: The scanning paths of 137 patients were recorded using an eye-tracking device; recordings were made while the patients viewed images of dento-maxillofacial deformities associated with various degrees of nasal deviation before or after orthognathic surgery. Visual attention focused on the lower face and nose was analyzed. RESULTS: When viewing postoperative faces, the participants focused more visual attention on noses and less on the lower face than they did on preoperative faces. Interestingly, for preoperative faces, nasal deviation could significantly increase participants' visual attention to the lower face, and visual attention to noses was significantly increased when noses were deviated 12°, while for postoperative faces, a nasal deviation of 4° or more was associated with a significant increase in participants' visual attention to the nose. CONCLUSIONS: Patients tended to focus their visual attention on the lower region of preoperative faces and ignored nose irregularities. Orthognathic surgery can alter visual attention, shifting it from the lower face to the nose, and a deviation of 4° or more could be a potential concern for patients. Clinicians must inform patients preoperatively about preexisting nasal deviations, which can guide surgical planning and help manage patient expectations. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .


Assuntos
Cirurgia Ortognática , Rinoplastia , Tecnologia de Rastreamento Ocular , Humanos , Septo Nasal/cirurgia , Nariz/anormalidades , Nariz/cirurgia , Percepção , Rinoplastia/métodos , Resultado do Tratamento
11.
J Craniofac Surg ; 33(2): 506-511, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35385909

RESUMO

BACKGROUND: Correcting a secondary bilateral cleft lip nasal deformity is very complex with many challenges due to its complexity. This study presents the surgical results after correcting a secondary bilateral cleft lip nasal deformity using isolation and repositioning of the lower lateral cartilages, the columellar strut, and an onlay graft on the nasal tip. METHOD: Eighteen patients who had bilateral cleft lip nasal deformities and who had surgery performed between September 2013 and December 2019 were included in this study. The age of the patients ranged between 19 and 52 years. Open rhinoplasty with bilateral reverse-U incision was performed. The lower lateral cartilages were isolated and repositioned. Nasal tip support is provided using a columellar strut with a septal cartilage or Medpor strut. In the vertical component of the nasal tip, a conchal cartilage graft was used. The mean follow-up period was 2 years (ranging from 1 to 6 years). RESULTS: Fourteen of the 18 patients underwent photogrammetric evaluation. After surgery, the projection of the nasal tip and angle increased, but nasal bridge length decreased. The columella-labial angle significantly increased by 10.1% after surgery which was statistically significant (P < 0.05). The angle between the nostril axes significantly decreased by 46.2% postoperatively and also reached statistical significance (P < 0.05), indicating that the displaced alar base and lower nostril sill improved and the major axis of the nostril changed more vertically oblique. All patients were pleased with their surgical results. CONCLUSION: The proposed technique was highly effective in correcting secondary bilateral cleft lip nasal deformities in adults.


Assuntos
Fenda Labial , Rinoplastia , Ferida Cirúrgica , Adulto , Cartilagem/transplante , Fenda Labial/complicações , Fenda Labial/cirurgia , Reposicionamento de Medicamentos , Humanos , Pessoa de Meia-Idade , Cartilagens Nasais/cirurgia , Septo Nasal/cirurgia , Nariz/anormalidades , Nariz/cirurgia , Rinoplastia/métodos , Ferida Cirúrgica/cirurgia , Resultado do Tratamento , Adulto Jovem
12.
Clin Dysmorphol ; 31(3): 109-112, 2022 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-35256564

RESUMO

INTRODUCTION: Laurin-Sandrow syndrome also known as tetramelic mirror-image polydactyly is a rare congenital disorder characterized classically by polysyndactyly of the hands, mirror feet and nose anomalies (hypoplasia of the nasal alae and short columella) often associated with ulnar and/or fibular duplication. As a pathologic entity, it is heterogeneous, the patients displaying a variety of symptoms. This review aims to analyze the different aspects of the condition, such as clinical findings and methods of treatment to summarize the principal features of Laurin-Sandrow syndrome. MATERIALS AND METHODS: The review is based on searches on PubMed, Web of Science and Researchgate of the following terms: "Laurin-Sandrow syndrome", "mirror hands", "mirror feet", "tetramelic mirror-image polydactyly", "fibular dimelia" and "ulnar dimelia". Clinical cases, reviews and original articles were included. RESULTS: As a consequence of our findings, we suggest a modification of the Al-Qattan classification system for Mirror Hand-Multiple Hand Spectrum. CONCLUSION: Even though it has an extremely low incidence, a thorough understanding of the syndrome enables the surgeon to choose the appropriate treatment with the ultimate goal to improve the patient's life quality.


Assuntos
Anormalidades Múltiplas , Ectromelia , Deformidades Congênitas do Pé , Deformidades Congênitas da Mão , Polidactilia , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/patologia , Ectromelia/patologia , Deformidades Congênitas do Pé/diagnóstico , Deformidades Congênitas do Pé/genética , Deformidades Congênitas da Mão/diagnóstico , Humanos , Nariz/anormalidades , Polidactilia/diagnóstico , Polidactilia/genética
13.
J Healthc Eng ; 2022: 7414165, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35222891

RESUMO

In this paper, we have carefully investigated the clinical phenotype and genotype of patients with Johanson-Blizzard syndrome (JBS) with diabetes mellitus as the main manifestation. Retinal vessel segmentation is an important tool for the detection of many eye diseases and plays an important role in the automated screening system for retinal diseases. A segmentation algorithm based on a multiscale attentional resolution network is proposed to address the problem of insufficient segmentation of small vessels and pathological missegmentation in existing methods. The network is based on the encoder-decoder architecture, and the attention residual block is introduced in the submodule to enhance the feature propagation ability and reduce the impact of uneven illumination and low contrast on the model. The jump connection is added between the encoder and decoder, and the traditional pooling layer is removed to retain sufficient vascular detail information. Two multiscale feature fusion methods, parallel multibranch structure, and spatial pyramid pooling are used to achieve feature extraction under different sensory fields. We collected the clinical data, laboratory tests, and imaging examinations of JBS patients, extracted the genomic DNA of relevant family members, and validated them by whole-exome sequencing and Sanger sequencing. The patient had diabetes mellitus as the main manifestation, with widened eye spacing, low flat nasal root, hypoplastic nasal wing, and low hairline deformities. Genetic testing confirmed the presence of a c.4463 T > C (p.Ile1488Thr) pure missense mutation in the UBR1 gene, which was a novel mutation locus, and pathogenicity analysis indicated that the locus was pathogenic. This patient carries a new UBR1 gene c.4463 T > C pure mutation, which improves the clinical understanding of the clinical phenotypic spectrum of JBS and broadens the genetic spectrum of the UBR1 gene. The experimental results showed that the method achieved 83.26% and 82.56% F1 values on CHASEDB1 and STARE standard sets, respectively, and 83.51% and 81.20% sensitivity, respectively, and its performance was better than the current mainstream methods.


Assuntos
Diabetes Mellitus , Retinopatia Diabética , Algoritmos , Anus Imperfurado , Mineração de Dados , Displasia Ectodérmica , Fundo de Olho , Transtornos do Crescimento , Perda Auditiva Neurossensorial , Humanos , Hipotireoidismo , Processamento de Imagem Assistida por Computador , Deficiência Intelectual , Nariz/anormalidades , Pancreatopatias , Perfusão
14.
Ann Otol Rhinol Laryngol ; 131(12): 1409-1412, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35043659

RESUMO

OBJECTIVES: Congenital frontonasal dysplasia (CFND) is a rare heterogeneous collection of facial deformities. Due to the range of complexity, surgical management is not standardized. METHODS: We present a severe case of CFND and approach to managing multiple defects with a focus on rhinoplasty. RESULTS: This infant was born full term with a large mass instead of a nose, a bilateral cleft lip and palate, and hypertelorbitism. Our primary concerns initially were to address communication with the intracranial cavity, preserve a nasal lining, and improve nasal appearance and airway function in the short term without interfering with subsequent rhinoplasty and adult nasal appearance. CONCLUSIONS: This complex case of CFND is more severe than anything we encountered in our literature review and demonstrates the necessity for multidisciplinary approach to multiple craniofacial defects. Future plans for this patient include rhinoplasty with auricular graft, scar revision, and addressing tip support.


Assuntos
Fenda Labial , Fissura Palatina , Rinoplastia , Adulto , Fenda Labial/cirurgia , Fissura Palatina/cirurgia , Anormalidades Craniofaciais , Face/anormalidades , Humanos , Lactente , Nariz/anormalidades , Nariz/cirurgia
15.
Hum Pathol ; 121: 73-80, 2022 03.
Artigo em Inglês | MEDLINE | ID: mdl-35063444

RESUMO

When a sarcomatous neoplasm is identified in the breast, distinguishing metaplastic carcinoma, malignant phyllodes tumor (MPT), and primary sarcoma is a diagnostic challenge, especially on small biopsies, as all these tumors may have overlapping morphological features, thoroughly grossing with histological examination and immunohistochemical staining being the standard approach to aid in classifying these lesions. Recently, we identified a highly sensitive and specific breast carcinoma marker TRPS1 with high expression in metaplastic breast carcinoma. In the current study, we tested TRPS1 in MPTs and primary sarcoma of the breast. We found TRPS1 was highly expressed (95%) within spindle cell, chondro-osseous, and/or liposarcomatous components of MPTs, in all breast primary chondrosarcomas and extraskeletal osteosarcomas, but not in other sarcomas of the breast. In extramammary sarcomas, TRPS1 was expressed in 28% of conventional chondrosarcomas and 56% of osteosarcomas of bone, but rarely in undifferentiated pleomorphic sarcomas (UPSs), liposarcomas, and angiosarcomas. In summary, MPTs may share similar genetic background with metaplastic carcinoma exhibiting TRPS1 expression, and TRPS1 may play a role in chondro-osseous differentiation because of its expression in chondro-osseous sarcomas from both breast and extramammary sites. Our findings suggest TRPS1 may be clinically useful in distinguishing MPT and metaplastic carcinoma from primary breast sarcoma except for tumors with chondro-osseous differentiation.


Assuntos
Neoplasias Ósseas , Neoplasias da Mama , Carcinoma , Condrossarcoma , Osteossarcoma , Tumor Filoide , Sarcoma , Neoplasias de Tecidos Moles , Neoplasias Ósseas/genética , Neoplasias da Mama/patologia , Carcinoma/patologia , Condrossarcoma/genética , Feminino , Dedos/anormalidades , Doenças do Cabelo , Humanos , Síndrome de Langer-Giedion , Nariz/anormalidades , Tumor Filoide/patologia , Proteínas Repressoras , Sarcoma/patologia
16.
Plast Reconstr Surg ; 149(2): 254e-260e, 2022 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-35077424

RESUMO

BACKGROUND: In 1968, Ralph Millard published his "Millard II" method for repair of wide, complete unilateral cleft lip and nose deformity. In 1979, Murawski published a major modification of the Millard II procedure in Polish. This motif was taken up 8 years later by Mohler and 22 years later by Cutting. The Murawski variation on the Millard II procedure has become a dominant motif in unilateral cleft lip repair worldwide. This brief report intends to introduce the method to the English language literature and present long-term results. METHODS: The Murawski method alters the Millard II procedure by changing the upper medial curve into a point in the columellar base. This creates a broad C flap used to fill the entire defect produced by downward rotation of the medial lip. Millard's lateral advancement flap becomes unnecessary. A lateral approach to primary nasal reconstruction allows the lateral C flap to be used to construct the nasal floor and sill. The method is described using a physics-based surgical simulator. RESULTS: Long-term results of the method are demonstrated with four patients with 15 to 25-year follow-up. None of these patients had any revisions to the lip or nose. CONCLUSIONS: The Murawski repair was the first to modify the Millard II repair by sharpening the medial columellar incision, eliminating the need for a lateral advancement flap. This motif was put forth in the years to follow by Mohler and Cutting. Long-term results of the method are presented.


Assuntos
Anormalidades Múltiplas/cirurgia , Fenda Labial/cirurgia , Nariz/anormalidades , Nariz/cirurgia , /métodos , Seguimentos , Humanos , Fatores de Tempo , Resultado do Tratamento
17.
Cleft Palate Craniofac J ; 59(6): 693-700, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-34132122

RESUMO

OBJECTIVE: We aimed to assess significant ethnic variabilities in infants' nasolabial anthropometry to motivate variations in surgical correction of a synchronous bilateral cleft lip/nasal anomaly, specifically whether a long columella is a European feature, therefore accepting a short columella and/or delayed columellar lengthening suitable for reconstruction in ethnic patients. METHODS: Thirty-three infants without craniofacial pathology (10 African American [AA], 7 Hispanic [H], and 16 of European descent [C]), ages 3 to 8 months, presenting to the Johns Hopkins All Children's general pediatric clinic were recruited. Four separate 3D photographs (2 submental and frontal views each) were taken using the Vectra H1 handheld camera (Canfield Imaging). Eighteen linear facial distances were measured using Mirror 3D analysis (Canfield Imaging Systems). Difference between ethnicities was measured using analysis of variance with the Bonferroni/Dunn post hoc comparisons. Pearson correlation was employed for interrater reliability. All statistical analyses were carried out using SPSS version 21.0 (IBM Corp), with statistical significance set at P < .05. RESULTS: Nasal projection (sn-prn) and columella length (sn-c) did not differ significantly between groups (P = .9). Significant differences were seen between ethnic groups in nasal width (sbal-sbal [C-AA; P = .02]; ac-ac [C-AA; P = .00; H-AA; P = .04]; al-al [C-AA; P = .00; H-AA; P = .001]) and labial length (sn-ls [C-AA; P = .041]; sn-sto [C-AA; P = .005]; Cphs-Cphi L [C-AA; P = .013]; Cphs-Cphi R [C-AA; P = .015]). Interrater reliability was good to excellent and significantly correlated for all measures. CONCLUSIONS: African American infants exhibited wider noses and longer lips. No difference was noted in nasal projection or columella length, indicating that these structures should be corrected during the primary cleft lip and nasal repair for all patients and should not be deferred to secondary correction.


Assuntos
Fenda Labial , Doenças Nasais , Antropometria , Criança , Fenda Labial/diagnóstico por imagem , Fenda Labial/cirurgia , Etnicidade , Humanos , Lactente , Septo Nasal , Nariz/anormalidades , Reprodutibilidade dos Testes
18.
Cleft Palate Craniofac J ; 59(4): 543-547, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-33887981

RESUMO

Although primary surgery for cleft lip has improved over time, the degree of secondary cleft or nasal deformity reportedly varies from a minimum degree to a remarkable degree. Patients with cleft often worry about residual nose deformity, such as a displaced columella, a broad nasal floor, and a deviation of the alar base on the cleft side. Some of the factors that occur in association with secondary cleft or nasal deformity include a deviation of the anterior nasal spine, a deflected septum, a deficiency of the orbicularis muscle, and a lack of bone underlying the nose. Secondary cleft and nasal deformity can result from incomplete muscle repair at the primary cleft operation. Therefore, surgeons should manage patients individually and deal with various deformities by performing appropriate surgery on a case-by-case basis. In this report, we applied the simple method of single VY-plasty on the nasal floor to a patient with unilateral cleft to revise the alar base on the cleft side. We adopted this approach to achieve overcorrection on the cleft side during surgery, which helped maintain the appropriate position of the alar base and ultimately balanced the nose foramen at 13 months after the operation. It was also possible to complement the height of the nasal floor without a bone graft. We believe that this approach will prove useful for managing cases with a broad and low nasal floor, thereby enabling the reconstruction of a well-balanced nose.


Assuntos
Fenda Labial , Doenças Nasais , Rinoplastia , Fenda Labial/complicações , Fenda Labial/cirurgia , Humanos , Septo Nasal/cirurgia , Nariz/anormalidades , Nariz/cirurgia , Doenças Nasais/cirurgia , Rinoplastia/métodos , Resultado do Tratamento
19.
Cleft Palate Craniofac J ; 59(8): 1079-1085, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-34549628

RESUMO

PURPOSE: Optimal correction of the cleft nasal deformity remains challenging. The purpose of this study was to examine the practice patterns and postoperative course of patients undergoing cleft lip repair with rhinoplasty compared to those who have primary lip repair without rhinoplasty. METHODS AND MATERIALS: A retrospective cohort study was conducted based on the Kids' Inpatient Database. Data were collected from January 2000 to December 2011 and included infants aged 12 months and younger who underwent cleft lip repair. The predictor variable was the addition of rhinoplasty at primary cleft lip repair. Primary outcome variables included hospital setting, year, and admission cost, while secondary outcome variables included length of stay and postoperative complication rate. Independent t-tests and chi-squared tests were performed. Continuous variables were analyzed by multiple linear regression models. RESULTS: The study sample included 4559 infants with 1422 (31.2%) who underwent primary cleft rhinoplasty. Over time, there was a significant increase in the proportion of cleft lip repairs accompanied by a rhinoplasty (p < .01). A greater proportion of patients with unilateral cleft lips received simultaneous rhinoplasty with their lip repairs (33.8 vs 26.0%, p < .01). This cohort had a significantly shorter length of stay (1.6 vs 2.8 days, p < .01) when compared to children that underwent cleft lip repair alone. CONCLUSIONS: Performing primary cleft rhinoplasty is becoming more common among cleft surgeons. Considering comparable costs and complication rates, a rhinoplasty should be considered during the surgical treatment planning of patients with cleft nasal deformities.


Assuntos
Fenda Labial , Doenças Nasais , Rinoplastia , Criança , Fenda Labial/cirurgia , Humanos , Lactente , Nariz/anormalidades , Doenças Nasais/cirurgia , Estudos Retrospectivos , Rinoplastia/métodos , Resultado do Tratamento
20.
Cleft Palate Craniofac J ; 59(4): 530-537, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-34291675

RESUMO

Complete congenital arhinia is a rare defect of embryogenesis leading to the absence of the external nose and airway. We report our novel multistaged reconstructive approach and literature review. Nasal methyl methacrylate prosthesis was created from a stereolithographic model for use as a temporary prosthesis and tissue expander. Lefort 1 with cannulization was utilized for midface advancement and airway formation. External framework was reconstructed with bilateral conchal bowl cartilage and rib osteocartilagenous grafts. Patient was pleased with the aesthetics and had safe decannulation with the ability to breathe through the nose and airway.


Assuntos
Implantes Dentários , Rinoplastia , Anormalidades Congênitas , Estética Dentária , Humanos , Nariz/anormalidades , Nariz/diagnóstico por imagem , Nariz/cirurgia
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