Short Columella Research Articles

Columellar lengthening with V-Y advancement flap in aesthetic rhinoplasty

Achieving a harmonious nasal shape requires balance from the frontal, lateral, and basal views. A short columella can lead to inadequate tip projection and drooping. While various methods have been used to lengthen a short columella in cleft lip and palate cases, adapting these techniques for aesthetic surgery presents challenges. This report outlines the design and surgical considerations for this issue. Ten patients with a short columella at risk of skin resistance or retraction were evaluated. The V-Y flap technique was employed, featuring a V-shaped design starting at the midpoint between the columellar footplate and the lowest point of the nostril sill. Standard techniques included a columellar graft to prevent narrowing. The rhomboid flap was trimmed and sutured in place. Results showed that the V-Y flap provided adequate tip projection, reduced columellar flaring, and improved nostril shape. Scarring was comparable to an inverted-V incision, with improvements in the columellar base and philtrum and high patient satisfaction. The technique effectively addressed tip projection challenges in patients with short columella.

Case report: Two cases of Cleft rhinoplasty

The correction of cleft lip nose deformity remains a great challenge for any cleft surgeon. The nose is a prominent part of the face, and any cleft lip repair directs the beholders’ eyes from the deformed lip to the deformed nose, Controversies exist on the timing of rhinoplasty in cleft lip patients and is categorized as primary rhinoplasty performed at the time of cleft lip repair, intermediate rhinoplasty at the age of 5–11 years and definitive rhinoplasty. This article presents two cases of unilateral nasal cleft in two adults treated by secondary rhinoplasty using an open technique. The primary deformity is an imbalance between abnormal muscle insertion and maxillary skeletal hypoplasia. The general characteristics of this deformity are a very short columella, downward rotation of the tip and diminished nasal projection, and treatment of this depressed and hypoplastic bony scaffolding is the secret to successful secondary rhinoplasty. Proponents of delayed nasal repair suggest that altering the cartilages in early nasal repair at the time of lip repair would complicate future corrective nasal surgeries if the primary repair would prove unsatisfactory. However, we know that repositioning the wing cartilage at the same time as closing the lip respects nasal growth by using a rigorous technique and control of scars The aim of cleft lip rhinoplasty is both aesthetic and functional: to obtain a normal nose with nasal breathing.

Long-Term Nose Evaluation After Treatment of Bilateral Cleft Lip Using the Spina Technique.

Bilateral cleft lip (BCL) repair is a challenge due to anatomic and functional distortions, worse than unilateral cleft. With regard to the nose, BCL can result in certain characteristics, such as a short columella, a flattened and widened tip, and horizontally oriented nostrils. Lip and nose treatment is advocated by some surgeons to achieve a better outcome. One of the techniques used to treat this condition is the one recommended by Victor Spina, in a complex protocol involving more than one surgical procedure and long-term follow-up. The aim of this study is to evaluate the growth of the nose in patients treated using the Spina technique. This was a retrospective and analytical study of the medical records of patients who were treated using the Spina technique at the Assistance Center for Cleft Lip and Palate (CAIF) at Hospital do Trabalhador in Curitiba, Brazil. Anthropometric measurements of interalar distance and nose tip projection were made from frontal and submental photographs taken at three moments: pre-first surgery, pre-definitive surgery, and late follow-up. Furthermore, other analyzed parameters were: sex, date of birth, age at time of surgery, and late follow-up. With these data, a comparison of nose growth was made between patients with BCL and a population without craniofacial anomalies observed in a study by FARKAS et al (1992). The comparison also took sex and age variables into account. Regarding the interalar distance measurements, both sexes showed a widening of the nose before starting treatment. This widening was maintained throughout follow-up. The growth curve, in turn, remained similar among males, with an average growth rate of only 3.2% higher in patients with BCL. Among females, the difference was one of 16.92%. In terms of nasal tip projection, there was an average increase of 1.12cm (86.15%) in males and 0.69cm (57.02%) in females. It was possible to notice a nasal growth rate in those with BCL similar to the normal pattern and a significant improvement in nasal tip projection in male patients. However, with regard to the data of both sexes, but especially females, the low number of patients jeopardized the significance of the results.

Incisor Show Based Selection of Tip Augmentation Rhinoplasty Technique.

Our recent studies show that the majority of patients seeking rhinoplasty have inadequate incisor show. Furthermore, that some rhinoplasty maneuvers, such as a columella strut can alter the lip position and consequently the incisor show. We report the senior author's algorithm for tip augmentation technique based on our prior studies and 44 years of rhinoplasty keen observations. The five most common scenarios are described. When there is a short columella with inadequate incisor show, bilateral extended spreader grafts and columella strut with a tapered posterior portion are recommended, serving as a reliable midline septal extension graft supporting the medial crura in a more anterior position with sutures and a positive effect on incisor show. When there is optimal lip/incisor relationship, a columella strut with a tapered posterior end fixed to the septum, with or without extended spreader grafts is a better choice to prevent a change in smile. If there is excessive gum show, the optimal tip augmentation technique is a columella strut without tapering or even wider posterior end, with fixation of the columella strut to the septum, with or without extended spreader grafts which will push the upper lip caudally. The remaining scenarios are described in detail. Tip augmentation maneuvers in rhinoplasty can have a significant influence on upper lip position, which is often detrimental to the smile. The pre-operative position of the upper lip during smile should play an integral role in the selection of tip augmentation technique.

Binder Phenotype—Prenatal Diagnosis, Management, and Postnatal Outcome: Insights from a Case Series and Updated Review of the Literature

AbstractBinder phenotype (BP) or maxillonasal dysplasia is a developmental disorder of the anterior part of the maxilla and nasal complex and is characterized by a short nose with a flat nasal bridge, short columella, acute nasolabial angle, perialar flatness, convex upper lip, and tendency to a class III malocclusion. The etiology of BP is heterogeneous with diverse features and outcomes. The ultrasound features of BP are midfacial hypoplasia with verticalized nasal bones, short columella with flattened tip and alar wings, and the nasofrontal angle measuring >140 degrees. In this case series, we present seven cases of BP detected antenatally, their varied etiology, management, and outcomes with a 2-year follow-up. We conclude that the diagnosis of facial dysmorphisms, such as BP, brings a lot of apprehension and agony in the parents amounting to multiple tests and counseling sessions. Physiognomy which is the normal familial appearance should be considered before concluding whether the observed feature is normal or pathological. Accurate diagnosis, adequate testing, and personalized counseling will help in the prevention of needless termination of pregnancies and ensure an optimal perinatal outcome.

Paradigm Shift in Rhinoplasty with Virtual 3D Surgery Software and 3D Printing Technology.

Most Asians have a nose with a short columella and a low dorsum; augmentation rhinoplasty using implants is commonly performed in Asian countries to achieve a taller and more well-defined nasal dorsum. However, the current knowledge is insufficient to fully understand the various subjective desires of patients, reflect on them during surgery, or to objectively analyze the results after surgery. Advances in digital imaging technologies, such as 3D printing and 3D scanning, have transformed the medical system from hospital-centric to patient-centric throughout the medical field. In this study, we applied these techniques to rhinoplasty. First, we used virtual 3D plastic surgery software to enable surgical planning through objectified numerical calculations based on the visualized data of the patient's medical images rather than simple virtual plastic surgery. Second, the customized nasal implant was manufactured by reflecting the patient's anatomical shape and virtual 3D plastic surgery data. Taken together, we describe the surgical results of applying these rhinoplasty solutions in four patients. Our experience indicates that high fidelity and patient satisfaction can be achieved by applying these techniques.

Secondary Bilateral Cleft Rhinoplasty: Achieving an Aesthetic Result.

Secondary rhinoplasty in patients with bilateral cleft lip poses ongoing challenges and requires a reliable method for achieving optimal outcomes. The purpose of this study was to establish a safe and effective method for secondary bilateral cleft rhinoplasty. A consecutive series of 92 skeletally matured patients with bilateral cleft lip and nasal deformity were included. All had undergone secondary open rhinoplasty, performed by a single surgeon with a bilateral reverse-U flap and septal extension graft, between 2013 and 2021. Medical records of these 92 patients were reviewed to assess the clinical course. A 3-dimensional (3D) anthropometric analysis and panel assessment of 32 patients were performed to evaluate the aesthetic improvement, with an age-, sex-, and ethnicity-matched normal control group for comparisons. The methods showed statistically significant improvement in addressing a short columella (columellar height), short nasal bridge (nasal bridge length), de-projected nasal tip (nasal tip projection, nasal dorsum angle), poorly defined nasal tip (nasal tip angle, dome height, and panel assessment), and transversely oriented nostrils (columellar height, alar width, nostril type). Importantly, these improvements were accompanied by a low complication rate of 4%. However, upper lip deficiency over the upper lip angle and labial-columellar angle remained without significant improvement. In this study we described effective secondary rhinoplasty, which was composed of a bilateral reverse-U flap and septal extension graft, with acceptable outcome. The 3D anthropometric analysis and panel assessment clarified that our rhinoplasty procedure could bring the nasal morphology in these patients closer to the normal data.

Prospective study of nasal growth in bilateral cleft lip and palate patients after naso-alveolar moulding and primary columella lengthening compared to controls at 5, 10 and 20 years.

A short columella, wide nostrils and a flat nasal tip are common features in patients with bilateral complete cleft lip and palate (BCLP). The objective of this study was to evaluate nasal morphology during growth in patients treated with naso-alveolar moulding (NAM) and primary surgical columella lengthening (PCL) compared with matched non-cleft individuals. Prospective longitudinal case-control study. Thirty-four consecutively treated BCLP patients at 5 and 10 years and at the end of growth (19.7 ± 2.0 years) were compared through normalized photogrammetry to a control of 34 age and sex-matched non-cleft individuals. Regression Models for Panel Data assessed how nasal measurements were influenced by surgery, age and gender. Nasal protrusion was equal to non-cleft controls at all ages. Length of the columella was also comparable to controls at 5 and 10 years, but significantly shorter at the end of growth. Inter-alar and nasal tip width and nasolabial angle were significantly wider than controls at all ages: More than 60% of the patients have asked for correction of the nasal width, but no early surgery for columella lengthening was needed. NAM and PCL have provided a nasal projection close to that of non-cleft individuals until adulthood, while length of the columella was physiological at 5 and 10, but shorter than controls at age 20. Width of the nasal tip and width of the alar bases were significantly wider than the controls and eventually required secondary nasal width correction in over two thirds of the sample.

Primary rhinoplasty in patients with bilateral cleft lip: Longitudinal results and comparison between closed and semi-open approaches

Although several studies have shown that primary rhinoplasty in patients with cleft lip provides good outcomes with limited effect on nasal growth, the surgical procedure remains to be standardized. The purpose of this study was to evaluate the long-term outcome of primary semi-open rhinoplasty with Tajima reverse-U incision, compared with that of closed rhinoplasty. Consecutive nonsyndromic patients with complete bilateral cleft lip and palate (n=52) who underwent primary semi-open rhinoplasty between 2001 and 2016 were reviewed. Patients who underwent primary closed rhinoplasty (n=61) and control group individuals were recruited for comparison. Computer-based standardized measurements of 2D photographs and panel assessments by laypersons were collected and statistically analyzed. In the comparative analysis at preschool age, semi-open rhinoplasty significantly improved the typical nasal deformities, including transversely oriented wide nostrils, short columella, and de-projected nasal tip, more effectively than closed rhinoplasty. Without major drawbacks, these parameters in the semi-open group were well maintained closer to those in the control group till skeletal maturity. After primary rhinoplasty, 54% of patients in the closed group and 4% in the semi-open group underwent intermediate rhinoplasty at preschool age. This study showed that the patients who underwent primary semi-open rhinoplasty achieved long-term and persistent outcomes that were closer to the normal nasal morphology compared with the patients treated with closed rhinoplasty, while avoiding intermediate rhinoplasty during the preschool to adolescent periods.

Nasal columella reconstruction using philtrum advancement flap: a case report of treatment of nasal stenosis

BackgroundNasal columella reconstruction is a challenging procedure, and the main goal is to maintain function as well as a satisfactory aesthetic outcome. Several surgical techniques have been described for the reconstruction of nasal columella. However, there is a paucity of literature on these techniques in our environment, and the use of superiorly based philtrum advancement flaps is extremely rare in African literature.Case presentationA 60-year-old man presented with nasal obstruction and nasal deformity following facial trauma 6 months prior to presentation. Examination revealed bilateral stenosed nasal cavities and a very short columella with a healed nasal scar. The diagnosis of nasal stenosis was made. Intra-operatively, a superiorly based philtrum advancement flap was designed and elevated, granulation tissues in the nasal cavities were excised, the septum was repositioned, the base of the flap was sutured at the midline of the floor of the nose, and the donor site was approximated. A bilateral nasal stent was applied for 4 weeks. Postoperatively, the recipient and donor sites healed completely with an aesthetically acceptable appearance, and the patient was satisfied with the outcome.ConclusionNasal columellar reconstruction is a complex procedure. The superiorly based philtrum advancement flap is a simple and aesthetically acceptable method of treating nasal stenosis. This method demonstrated an adequate nasal airway and a good cosmetic appearance.

A case of combined oxidative phosphorylation deficiency 32 caused by MRPS34 gene variation and literature review

Objective: To investigate the clinical features and genetic features of combined oxidative phosphorylation deficiency 32 (COXPD32) caused by MRPS34 gene variation. Methods: The clinical data and genetic test of a child with COXPD32 hospitalized in the Department of Neurology, Children's Hospital, Capital Institute of Pediatrics in March 2021 were extracted and analyzed. A literature search was implemented using Wanfang, China biology medicine disc, China national knowledge infrastructure, ClinVar, human gene mutation database (HGMD) and Pubmed databases with the key words "MRPS34" "MRPS34 gene" and "combined oxidative phosphorylation deficiency 32" (up to February 2023). Clinical and genetic features of COXPD32 were summarized. Results: A boy aged 1 year and 9 months was admitted due to developmental delay. He showed mental and motor retardation, and was below the 3rd percentile for height, weight, and head circumference of children of the same age and gender. He had poor eye contact, esotropia, flat nasal bridge, limbs hypotonia, holding instability and tremors. In addition, Grade Ⅲ/6 systolic murmur were heard at left sternal border. Arterial blood gases suggested that severe metabolic acidosis with lactic acidosis. Brain magnetic resonance imaging (MRI) showed multiple symmetrical abnormal signals in the bilateral thalamus, midbrain, pons and medulla oblongata. Echocardiography showed atrial septal defect. Genetic testing identified the patient as a compound heterozygous variation of MRPS34 gene, c.580C>T (p.Gln194Ter) and c.94C>T (p.Gln32Ter), with c.580C>T being the first report and a diagnosis of COXPD32. His parents carried a heterozygous variant, respectively. The child improved after treatment with energy support, acidosis correction, and "cocktail" therapy (vitaminB1, vitaminB2, vitaminB6, vitaminC and coenzyme Q10). A total of 8 cases with COXPD32 were collected through 2 English literature reviews and this study. Among the 8 patients, 7 cases had onset during infancy and 1 was unknown, all had developmental delay or regression, 7 cases had feeding difficulty or dysphagia, followed by dystonia, lactic acidosis, ocular symptoms, microcephaly, constipation and dysmorphic facies(mild coarsening of facial features, small forehead, anterior hairline extending onto forehead,high and narrow palate, thick gums, short columella, and synophrys), 2 cases died of respiratory and circulatory failure, and 6 were still alive at the time of reporting, with an age range of 2 to 34 years. Blood and (or) cerebrospinal fluid lactate were elevated in all 8 patients. MRI in 7 cases manifested symmetrical abnormal signals in the brainstem, thalamus, and (or) basal ganglia. Urine organic acid test were all normal but 1 patient had alanine elevation. Five patients underwent respiratory chain enzyme activity testing, and all had varying degrees of enzyme activity reduction. Six variants were identified, 6 patients were homozygous variants, with c.322-10G>A was present in 4 patients from 2 families and 2 compound heterozygous variants. Conclusions: The clinical phenotype of COXPD32 is highly heterogenous and the severity of the disease varies from development delay, feeding difficulty, dystonia, high lactic acid, ocular symptoms and reduced mitochondrial respiratory chain enzyme activity in mild cases, which may survive into adulthood, to rapid death due to respiratory and circulatory failure in severe cases. COXPD32 needs to be considered in cases of unexplained acidosis, hyperlactatemia, feeding difficulties, development delay or regression, ocular symptoms, respiratory and circulatory failure, and symmetrical abnormal signals in the brainstem, thalamus, and (or) basal ganglia, and genetic testing can clarify the diagnosis.

Columellar Lengthening in Primary and Secondary Rhinoplasty for Binder's Syndrome: A Fresh Perspective.

Binder's syndrome is a developmental disorder with a short columella and nasal base flaring among the many features that have been described. Since the nose occupies the central position over the face, these features appear as a major cosmetic deformity for which patients seek correction. Traditionally various designs of V-Y advancement flap from the upper lip have been described, but they are not without problems. In this article, the authors have discussed a novel design that can mitigate those problems and also described a method of providing additional vascular safety in case of secondary rhinoplasty.

Which Is the Optimal Anthropometric Method for Asian Noses: Conventional Rulers, Two-Dimensional Photographs, Three-Dimensional Imaging, or Computed Tomography?

Most Asian noses have a bulbous tip, low radix, low dorsum, short columella, and thick skin envelope, which make rhinoplasty one of the most popular plastic surgery procedures among Asians. Nasal anthropomorphic measurements are required for planning and obtaining a pleasing aesthetic result in rhinoplasty.1 In addition, understanding the relative effect of individual treatments or techniques depends on accurate and objective nasal measurements. Several anthropometric methods have been applied, including conventional rulers, two-dimensional photographs, three-dimensional imaging, or computed tomography (Table 1). Table 1. - Advantages and Disadvantages of Nasal Measurement Methods Method Advantages Disadvantages Conventional rulers Accessible Imprecise, examiner-biased, unfavorable angular measurements Two-dimensional photographs Accessible, appearance record, favorable angular measurements Need to set scale manually, not applicable to the noses with low radix and alar hypertrophy Three-dimensional imaging High reliability, accuracy, time saving, low cost, no radioactivity, repeatability No tissue information, expensive imaging machine CT High reliability, accuracy, visible bone and soft tissue High cost, radioactivity CT, computed tomography. Anthropometric measurements are referenced to the standards established by Farkas et al.2 Conventional rulers, such as calipers and protractors, have proven to be time-consuming, examiner-biased, and extremely relying on patient cooperation. This is probably because of the difficulty in identifying anatomical landmarks with sufficient precision. In addition, the identification of some landmarks by individuals is somewhat subjective. As the nose is three-dimensional, it is also difficult to simulate some nasal angles, such as nasal tip angle and nasofacial angle, and measure them accurately with protractors. Two-dimensional photographs are routinely used to record changes before and after rhinoplasty. Linear measurements can be obtained using image processing software if the photographs contain a scale; otherwise, only angles can be measured. The accuracy of the measurements can be affected by many factors, such as lighting, camera angle, and distance relative to the patient. A large percentage of Asians have a low radix, and the landmarks of nasion points are not visible from lateral photographs, resulting in some nasal measurements—including nasal height, nasal length, nasofrontal angle, and nasofacial angle—not being able to be measured. Likewise, alar hypertrophy causes the subnasal points to be unrecognizable on lateral photographs and, consequently, the nasal depth and nasolabial angle cannot be measured. Three-dimensional imaging can produce high-resolution three-dimensional images by capturing and constructing topographic surface maps. Several studies have been performed using three-dimensional imaging for facial anthropometric measurements, and the method has proven to be highly accurate and reliable.3,4 Computed tomography has become an important tool for rhinoplasty because of its high efficiency to analyze and simulate nasal bone and soft tissue independently. The midsagittal images and axial images of computed tomography were used for nasal measurements previously, whereas the position of the head during the examination may affect the images. Besides, computed tomographic data can be used to generate three-dimensional images of the face to facilitate nasal measurements.5 The three-dimensional images can be moved and rotated to any view, and the nasal details can be magnified to identify the anthropometric landmarks. The scale of the three-dimensional image is defaulted by the machine with high precision, without the need to determine the scale manually as for two-dimensional photographs. Computed tomography allows precise acquisition of head tissue information, but the disadvantages of high cost and radioactivity prevent its routine use for nasal measurements. Although three-dimensional imaging can obtain only facial surface images, it is sufficient for nasal measurements. Compared to computed tomography, three-dimensional imaging has the advantages of time saving, low cost, no radioactivity, and repeatability. Overall, three-dimensional imaging is recommended for nasal measurements, especially for Asian noses. One day, three-dimensional scanning will replace direct measurements and two-dimensional photographs, because of its reliability, accuracy, repeatability, time saving, patient convenience, and low cost. DISCLOSURE The authors have no financial interest in any of the products or devices mentioned in this article.

Menke-Hennekam syndrome 1: A Case Report

IntroductionMenke-Hennekam syndrome (MHS) is a relatively new genetic condition characterized by intellectual disabilities, autistic behavior, auditory defects, recurrent upper respiratory tract infections, microcephaly and short stature. Facial characteristics include short palpebral fissures, telecanthus, depressed nasal bridge, short nose, anteverted nares, short columella, and long philtrum. The genetic defect is represented by missense variants of CREBBP gene, located on exons 30 or 31. There are only around 30 cases reported by now.ObjectivesThe aim of the paper is to report a new case of MHS.MethodsThe case is a 3-year-old boy admitted in our department for developmental delay. The clinical examination revealed dysmorphic features; severe speech delay, mild intellectual disability, autistic behaviour.The patient had a personal history of recurrent respiratory infections, visual defect and bilateral sensorineural hearing loss. Other investigations included EEG, abdominal echography, and cerebral MRI all were normal. The genetic studies included array CGH and WES.ResultsThe array CGH was normal. WES identified a pathogenic heterozygote variant c.5600G>A in the exon 31 of CREBBP gene, confirming MHS.ConclusionsOverall, the features of our patient are consistent with those reported in the previous reports, including developmental and speech delay, autistic behavior, dysmorphic features, recurrent upper way infections, sensorineural hearing loss, and visual defects. Other common features, such as growth delay and microcephaly were not present in our patient. Our case contributes to the clinical characterisation of the new syndrome. Funding: The research leading to these results has received funding from the EEA Grant 2014-2021, under the project contract No 6/2019.DisclosureNo significant relationships.

"Laurin-Sandrow Syndrome - a review of the literature and classification system".

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. 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. As a consequence of our findings, we suggest a modification of the Al-Qattan classification system for Mirror Hand-Multiple Hand Spectrum. 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.

Photometric Evaluation of Adult Patients With Bilateral Cleft Lip and Palate Treated With Nasoalveolar Molding and Primary Columella Lengthening.

The aim of this study was to assess the nasal shape of young adults with BCLP treated with primary surgical columella lengthening and nasoalveolar molding (NAM). A group of 28 young adult patients with BCLP (mean age: 19.1±1.4 years) was compared through normalized photogrammetry to a control of 28 age- and sex-matched noncleft young adults. Nasal protrusion and length of the columella were not different from noncleft young adults. On the other hand, nasolabial angle, columellar width, interalar, and nasal tip width were significantly wider than the noncleft controls. Thus, 27% of the patients have requested at this time secondary correction of the excessive nasal width. Both NAM and primary rhinoplasty in patients with BCLP resulted in a near normal length of the columella and nasal projection until young adulthood. Nevertheless, width of all nasal features was significantly wider than the noncleft population and required secondary nasal correction in one-third of the sample.

Determination of Ethnic Variation in Infant Nasolabial Anthropometry Using 3D Photographs: Implications for Bilateral Cleft Lip Nasal Correction.

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. 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. 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. 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.

CONGENITAL CUTIS LAXA: A RARE GENODERMATOSIS

Cutis Laxa (CL) / generalized elastolysis / dermatomegaly is a heterogeneous group of disorders which are related to elastic tissue abnormalities. Depending on extent of abnormal elastic tissue, it may be mild or severe. Severe form presents with loose, inelastic, wrinkled skin resembling ill tted suit. Infant has characteristic facial features like old man appearance, a hooked nose, a short columella, a long upper lip with long philtrum, and everted lower eyelids. CL is categorised as congenital or acquired and the inheritance can be autosomal dominant or recessive, or X linked. Occasionally a few metabolic disorders like Menkes disease, disorders of glycosylation are associated with Congenital CL. Acquired cutis laxa has developed after a febrile illness and various inammatory skin diseases. Here we present a case of a full-term SGA (small for gestational age) female child born with features of CL.

The Harvest of a Lateral Segment of Costal Cartilage for Treating Nasal Deformities.

Although costal cartilage has many uses and is a reliable source of cartilage for rhinoplasty procedures, donor-site complications may arise with conventional harvesting techniques. The present report reports a novel technique of harvesting costal cartilage using a specially designed scalpel and studies the use of the harvested cartilage in the reconstruction of secondary nasal deformities in patients with cleft lips. Ten patients (7 females and 3 males) with nasal deformities secondary to cleft lip underwent rhinoplasty using this new technique at the Department of Oral and Maxillofacial Surgery, Second Hospital of Hebei Medical University, China, between May 2011 and December 2013. Clinical outcomes were evaluated with a follow-up period of 6 to 30 months. The new technique successfully corrected primary nasal deformities, including flat nasal tip, short columella, flaring alae, and asymmetrical nostrils. Surgeons and patients assessed the outcome to be either good or satisfactory. Patients experienced transient discomfort at the wound site but there were no major complications (such as wound infection, dehiscence, exposure, graft extrusion, and pulmonary involvement). The novel technique can harvest a lateral segment of costal cartilage for use in the reconstruction of nasal deformities secondary to cleft lip in a one-stage procedure, with minimal donor-site morbidity.

The utilization of diced cartilage-fascia (DC-F) graft in the augmentation rhinoplasty of a case with Binder syndrome

The Binder syndrome is a congenital anomaly mostly seen in females characterized by a flat facial profile and flat nose with absent nasal spine, short columella, flat nasal dorsum, hypoplastic lower lateral cartilages (LLC) and septum, class-III malocclusion, and cleft lip and palate. A 26-year-old female patient with a Binder syndrome was referred to the clinic with a flat nasal dorsum, hypoplastic LLC, deformed nostrils, and hypoplastic columella. She had a history of bilateral cleft lip and palate, ocular strabismus, hypothyroidism, and growth retardation. An augmentation rhinoplasty with an open approach was performed in January 2019. The seventh costal cartilage and the rectus fascia were preferred as autologous grafts. An extended columellar strut graft was prepared and fixed to the nasal spine. Two lateral crural strut grafts was prepared and placed. The hypoplastic LLC were fixated to these grafts. A dorsal onlay graft was settled. Then, the diced cartilage-fascia (DC-F) graft was prepared and it was settled in over the nasal dorsum. A smaller and spherical DC-F graft was constructed to be placed over the extended columellar strut graft for increasing the tip projection. One-year postoperative results of this case confirm that the DC-F graft conserved its volume and shape. The growth retardation has not been described in Binder syndrome in the current literature, and this is the first case of nasal augmentation with DC-F graft in Binder syndrome. DC-F graft is a reliable alternative for the augmentation of the nasal dorsum in Binder syndrome. Level of evidence: Level V, therapeutic study.