Dacryocystocele and Subsequent Dacryocystectomy in a Patient With Bosma Arhinia Microphthalmia Syndrome (BAMS): A Case Report and Review of Literature.

Bosma arhinia microphthalmia syndrome (BAMS) is a rare syndrome consisting of several craniofacial abnormalities, including congenital arhinia. In this case report, the authors present the first case of a patient with BAMS and dacryocystocele who successfully underwent dacryocystectomy. Dacryocystectomy may serve as a viable surgical approach for dacryocystocele in patients with abnormal nasal anatomy. [J Pediatr Ophthalmol Strabismus. 2024;61(3):e16-e18.].

Clinical report of Bosma arhinia microphthalmia syndrome with a new variant on SMCHD1 gene. A case report.

The Bosma syndrome (BAMS: Bosma arhinia microphthalmia syndrome) is a condition first described in 1972. Since then, several reviews have published the cases looking for diagnostic criteria and associated genetic alterations. The mutation in the SMCHD1 gene (Structural Maintenance of Chromosomes flexible Hinge Domain containing protein 1) seems to explain a part of the development of the phenotype. Not all cases show the same alterations or meet the classic diagnostic criteria, and few have undergone genetic analysis. We present a case with a new variant in this gene and an update of the literature on this syndrome with the aim of improving the diagnosis and follow-up of these patients.

Cis-regulatory control of mammalian Trps1 gene expression.

TRPS1 serves as the causative gene for tricho-rhino phalangeal syndrome, known for its craniofacial and skeletal abnormalities. The Trps1 gene encodes a protein that represses Wnt signaling through strong interactions with Wnt signaling inhibitors. The identification of genomic cis-acting regulatory sequences governing Trps1 expression is crucial for understanding its role in embryogenesis. Nevertheless, to date, no investigations have been conducted concerning these aspects of Trps1. To identify deeply conserved noncoding elements (CNEs) within the Trps1 locus, we employed a comparative genomics approach, utilizing slowly evolving fish such as coelacanth and spotted gar. These analyses resulted in the identification of eight CNEs in the intronic region of the Trps1 gene. Functional characterization of these CNEs in zebrafish revealed their regulatory potential in various tissues, including pectoral fins, heart, and pharyngeal arches. RNA in-situ hybridization experiments revealed concordance between the reporter expression pattern induced by the identified set of CNEs and the spatial expression pattern of the trps1 gene in zebrafish. Comparative in vivo data from zebrafish and mice for CNE7/hs919 revealed conserved functions of these enhancers. Each of these eight CNEs was further investigated in cell line-based reporter assays, revealing their repressive potential. Taken together, in vivo and in vitro assays suggest a context-dependent dual functionality for the identified set of Trps1-associated CNE enhancers. This functionally characterized set of CNE-enhancers will contribute to a more comprehensive understanding of the developmental roles of Trps1 and can aid in the identification of noncoding DNA variants associated with human diseases.

TRPS1 modulates chromatin accessibility to regulate estrogen receptor alpha (ER) binding and ER target gene expression in luminal breast cancer cells.

Common genetic variants in the repressive GATA-family transcription factor (TF) TRPS1 locus are associated with breast cancer risk, and luminal breast cancer cell lines are particularly sensitive to TRPS1 knockout. We introduced an inducible degron tag into the native TRPS1 locus within a luminal breast cancer cell line to identify the direct targets of TRPS1 and determine how TRPS1 mechanistically regulates gene expression. We acutely deplete over 80 percent of TRPS1 from chromatin within 30 minutes of inducing degradation. We find that TRPS1 regulates transcription of hundreds of genes, including those related to estrogen signaling. TRPS1 directly regulates chromatin structure, which causes estrogen receptor alpha (ER) to redistribute in the genome. ER redistribution leads to both repression and activation of dozens of ER target genes. Downstream from these primary effects, TRPS1 depletion represses cell cycle-related gene sets and reduces cell doubling rate. Finally, we show that high TRPS1 activity, calculated using a gene expression signature defined by primary TRPS1-regulated genes, is associated with worse breast cancer patient prognosis. Taken together, these data suggest a model in which TRPS1 modulates the genomic distribution of ER, both activating and repressing transcription of genes related to cancer cell fitness.

Secondary Bilateral Cleft Rhinoplasty: Achieving an Aesthetic Result.

BACKGROUND: Secondary rhinoplasty in patients with bilateral cleft lip poses ongoing challenges and requires a reliable method for achieving optimal outcomes. OBJECTIVES: The purpose of this study was to establish a safe and effective method for secondary bilateral cleft rhinoplasty. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Successful topical minoxidil treatment for hair density and length in trichorhinophalangeal syndrome type 1.

A 9-year-old girl presented with slow hair growth and hair thinning since birth. Additionally, she had short stature and abnormally short fingers; genetic testing confirmed the diagnosis of trichorhinophalangeal syndrome (TRPS) type 1. After 4 months of topical minoxidil treatment, hair density and length significantly improved diffusely throughout the scalp without evidence of hypertrichosis. This case underscores the therapeutic potential of topical minoxidil for TRPS, paving the way for improved patient quality of life.

A Comparative Evaluation of TRPS1 and GATA3 in adenoid cystic, secretory, and acinic cell carcinomas of the breast and salivary gland.

GATA3 is the most used marker to determine tumors' breast origin, but its diagnostic value in triple-negative breast cancer (TNBC) is limited. The newly identified TRPS1 is highly sensitive and specific for breast carcinoma, especially TNBC. Here, we compared the utility of TRPS1 and GATA3 expression in a subset of salivary gland-type breast tumors (including adenoid cystic, acinic cell, and secretory carcinomas [AdCC, ACC, and SC, respectively]), and we compared TRPS1 and GATA3 expression of such tumors with head and neck (H&N) and AdCC of upper respiratory tumors. TRPS1 was strongly expressed in basaloid TNBC and AdCCs with solid components, including 100 % of mixed and solid breast AdCCs. However, TRPS1 was positive in only 50 % cribriform AdCCs. Expression patterns of TRPS1 in H&N and upper respiratory AdCC were similar. TRPS1 was positive in 30 % of H&N cribriform AdCCs but was strongly expressed in mixed AdCC (67 %) and solid AdCC (100 %). In the upper respiratory AdCCs, TRPS1 was positive in 58.4 % of cribriform AdCCs and positive in 100 % of AdCCs with solid components. On the contrary, GATA3 was negative in predominant AdCCs of the breast, H&N, and upper respiratory tract. These data show that GATA3 and TRPS1 expression varies AdCCs. In addition, TRPS1 and GATA3 expression patterns were similar SC and ACC of breast and H&N. Both markers were positive in SC and negative in ACC. Therefore, TRPS1 and GATA3 cannot be used to differentiate salivary gland-type carcinomas of breast origin from those of upper respiratory or H&N origin.

Effects of nasoalveolar molding on nasolabial aesthetics in patients with cleft lip and palate during pre-adolescence: A systematic review and meta-analysis of retrospective studies.

This study aims to analyze long-term effects of nasoalveolar molding (NAM) as a part of cleft primary management protocols on nasolabial aesthetics for patients with non-syndromic cleft lip and palate by conducting a systematic review and meta-analysis. Six electronic databases and two journals were searched up to July 2023. Studies comparing nasolabial outcomes between NAM and non-NAM protocols were selected for further analysis. Nasolabial aesthetics were the outcome of interest. Data extraction, methodological quality assessment, risk of bias assessment, meta-analysis and subgroup analysis were performed. Seven retrospective cohort studies were selected for a qualitative review and four for a quantitative analysis. The risk of bias assessment was moderate for most studies. Only studies utilizing the Asher-McDade rating (AMR) were included for meta-analyses. The protocols with NAM exhibited a significantly lower AMR score for vermillion border than other protocols. AMR scores for nasal form and nasal symmetry from protocols with NAM were significantly lower than protocols without any pre-surgical infant orthopaedics (PSIO) but not significantly different from protocols with other PSIO techniques. The AMR score for nasolabial profile from protocols with NAM was not significantly different from other protocols. However, subgroup analysis demonstrated that protocol combining NAM and primary rhinoplasty significantly lowered AMR scores for nasal form, nasal symmetry and nasolabial profile. For patients with unilateral cleft lip with or without palate (UCLP), this study found that a protocol combining NAM and primary rhinoplasty improved nasolabial outcomes while a protocol with NAM alone offered only limited benefits. For patients with BCLP, the available evidence remains inconclusive. Performing NAM in combination with primary rhinoplasty improves nasolabial aesthetics in patients with UCLP. PROSPERO (CRD4202128384).

Use of Multiple Machine Learning Approaches for Selecting Urothelial Cancer-Specific DNA Methylation Biomarkers in Urine.

Diagnosing urothelial cancer (UCa) via invasive cystoscopy is painful, specifically in men, and can cause infection and bleeding. Because the UCa risk is higher for male patients, urinary non-invasive UCa biomarkers are highly desired to stratify men for invasive cystoscopy. We previously identified multiple DNA methylation sites in urine samples that detect UCa with a high sensitivity and specificity in men. Here, we identified the most relevant markers by employing multiple statistical approaches and machine learning (random forest, boosted trees, LASSO) using a dataset of 251 male UCa patients and 111 controls. Three CpG sites located in ALOX5, TRPS1 and an intergenic region on chromosome 16 have been concordantly selected by all approaches, and their combination in a single decision matrix for clinical use was tested based on their respective thresholds of the individual CpGs. The combination of ALOX5 and TRPS1 yielded the best overall sensitivity (61%) at a pre-set specificity of 95%. This combination exceeded both the diagnostic performance of the most sensitive bioinformatic approach and that of the best single CpG. In summary, we showed that overlap analysis of multiple statistical approaches identifies the most reliable biomarkers for UCa in a male collective. The results may assist in stratifying men for cystoscopy.

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

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Novel classification system for adult Asian secondary bilateral cleft lip with nasal deformity that guides surgical strategy.

BACKGROUND: Bilateral cleft lip is a congenital defect often accompanied by secondary lip and nose deformity. The current classification system for secondary cleft lip deformity has limitations in guiding surgical planning. In this article, we report a method for secondary bilateral cleft lip classification that can guide surgery on the basis of the pathological anatomy of the columellar and upper lip. METHODS: Photographs of patients were retrospectively classified into four types on the basis of the ratio of columellar height to alar base width (CH/AW) and upper lip protrusion (UP) to lower lip, as follows: type I - with CH/AW ≥ 0.2 and UP ≥ 0; type II - with CH/AW ≥ 0.2 and UP <0; type III - with CH/AW < 0.2 and UP ≥0; type IV - with CH/AW < 0.2 and UP < 0. Surgical treatments and the change of the nasal profile were documented. RESULTS: A total of 105 patients from January 2008 to December 2018 were included in this study. The nasal profile was significantly improved in type III and IV patients with postoperative CH/AW values close to normal. The upper lip was distinctively retruded in type II and IV patients before treatment, and the postoperative view revealed improved upper lip protrusion with UP values close to normal. Ninety-eight patients reported satisfactory outcomes after treatment. CONCLUSIONS: The new classification method described provides key information regarding the deformity of different types of secondary bilateral cleft lip patients and provides clear guidance for surgical planning on the basis of the anatomical defect of each type.

Molecular Genetic Analysis and Growth Hormone Treatment in a Three-Generation Chinese Family with Tricho-Rhino-Phalangeal Syndrome I.

INTRODUCTION: Tricho-rhino-phalangeal syndrome (TRPS) is a rare genetic disorder characterized by craniofacial and skeletal abnormalities, which is caused by variants in the TRPS1 gene. METHODS: Clinical information and follow-up data were collected. Whole-exome sequencing (WES) was performed for variants and validated by Sanger sequencing. Bioinformatic analysis was performed to predict the pathogenicity of the identified variant. Moreover, wild-type and mutated TRPS1 vectors were constructed and transfected into human embryonic kidney (HEK) 293T cells. Immunofluorescence experiments were performed to assess the localization and expression of the mutated protein. Western blot analysis and RT-qPCR were used to detect the expression of downstream genes. RESULTS: The affected family members had typical craniofacial phenotype including sparse lateral eyebrows, pear-shaped nasal tip, and large prominent ears, plus skeletal abnormalities including short stature and brachydactyly. WES and Sanger sequencing identified the TRPS1 c.880_882delAAG variant in affected family members. In vitro functional studies showed that the TRPS1 variant did not affect the cellular localization and the expression of TRPS1, but the transcriptional repression effect of the TRPS1 on the RUNX2 and STAT3 was disturbed. The proband and his brother have been treated with growth hormone (GH) for 2 years until now, and we have observed the improvement of the linear growth in both. CONCLUSIONS: The variant of c.880_882delAAG in TRPS1 was responsible for the pathogenesis of the Chinese family with TRPS I. The treatment of GH could be beneficial for the height outcome in TRPS I patients, and earlier initiation and longer duration of the therapy in prepubertal or early pubertal stage could be associated with better height outcomes.

Orofacial Deformities in 3 Related Rhesus Monkeys (Macaca mulatta) Resembling Human Binder's Syndrome.

Binder's syndrome is a rare congenital deformity characterized by midface hypoplasia, particularly around the nasomaxillary area. Genetic etiology or developmental failure caused by prenatal exposure to teratological agents has been considered. In this article, we present 3 related rhesus monkeys born with orofacial deformities similar to those found in infants with the Binder phenotype. For the first time, a primate biomodel for this condition is presented. The clinical description and association with management and environmental factors are discussed. These findings reinforce the knowledge about the relationship between possible vitamin K metabolism interference and Binder's syndrome.

Two novel mutations within FREM1 gene in patients with bifid nose.

BACKGROUND: Bifid nose is a rare congenital deformity and the etiology is unknown. The purpose of this study was to report genetic variation in family of patients with bifid nose. METHODS: Twenty-three consecutive patients who were diagnosed with mild bifid nose were operated with z-plasty from 2009 to 2021. Three underage patients (a pair of twins and a girl) from two family lines, who came to our hospital for surgical treatment, were enrolled. Whole exome sequencing and Sanger sequencing were conducted. Z-shaped flaps were created and the cartilago alaris major were re-stitched. Photographs and CT scan before and after surgery were obtained. Clinical outcomes, complications and patients' satisfaction were evaluated and analyzed. The follow-up time ranges from 2 to 3 years (2.4 ± 1.2 years). RESULTS: Most patients were satisfied with the outcome (96.2%). The nasal deformities were corrected successfully with z-plasty technique in one-stage. FREM1 c.870_876del and c.2 T > C were detected with Whole exome sequencing, which have not been reported before. The results of Sanger sequencing were consistent with those of Whole exome sequencing. CONCLUSIONS: The newly detected mutations of FREM1 have a certain heritability, and are helpful to make an accurate diagnosis and provide a better understanding of bifid nose mechanism. Z-plasty technique can be an effective technical approach for correcting mild bifid nose deformity.

One-year clinical observation of muscular force balance reconstruction technique for the correction of secondary nasal malformation after cleft lip surgery.

OBJECTIVES: The long-term effect of muscular force balance reconstruction technique combined with intranasal fixation for correcting secondary nasolabial deformity after unilateral cleft lip was evaluated. The aim was to provide a basis for further improving the surgical treatment effect of secondary nasolabial deformity of acleft lip. METHODS: A total of 40 patients aged 4-28 years with secondary nasal deformity and unilateral cleft lip were selected as research subjects. The two-dimensional photo measurement analysis method was used in comparing the surgical results before and immediately after the operation (7 d) and 1 year after the operation. RESULTS: Columellar angle, nostril height ratio (NHR), alar rim angle, alar rim angle ratio, and nostril shape (NS) increased dimmediately after the operation, whereas alar base width ratio (ABWR) and nostril width ratio decreased (NHR) immediately after the operation (P<0.01). The ABWR, NHR, and NS immediately after the operation were not significantly different from those 1 year after the operation (P>0.05). CONCLUSIONS: Muscular force balance reconstruction technique combined with intranasal fixation is effective in the repair of unilateral secondary nasolabial deformity, and stable results can be obtained 1 year after surgery.

Perception of aesthetic features after surgical treatment of craniofacial malformations by observers of the same age: An eye-tracking study.

The aim of this study is to evaluate where exactly children and adolescents of the same group look when they interact with each other, and attempt to record and analyse the data recorded by eye-tracking technology. MATERIALS AND METHODS: 60 subjects participated in the study, evenly divided into three age categories of 20 each in pre-school/primary school age (5-9 years), early adolescence (10-14 years) and late adolescence/transition to adulthood (15-19 years). Age groups were matched and categorized to be used both for creating the picture series and testing. Photographs of patients with both unilateral and bilateral cleft lip and palate were used to create the series of images which consisted of a total of 15 photos, 5 of which were photos of patients with surgically treated cleft deformity and 10 control photos with healthy faces, that were presented in random order. Using the eye-tracking module, the data on "area of first view" (area of initial attention), "area with longest view" (area of sustained attention), "time until view in this area" (time of initial attention) and "frequency of view in each area" (time of sustained attention) were calculated. RESULTS: Across all groups, there was no significant difference for the individual regions for the parameters of initial attention (area of first view), while the time until first fixation of one of the AOIs (time until view in this area) was significant for all facial regions. A predictable path of the facial scan is abandoned when secondary facial deformities are present and attention is focused more on the region of an existing deformity, which are the nose and mouth regions. CONCLUSIONS: There are significant differences in both male and female participants' viewing of faces with and without secondary cleft deformity. While in the age group of the younger test persons it was still the mouth region that received special attention from the male viewers, this shifted in the male test persons of the middle age group to the nose region, which was fixed significantly more often and faster. In the female participants, the mouth and nose regions were each looked at for twice as long compared to the healthy faces, making both the mouth and the nose region are in the focus of observation.

The Morphological and Functional Effects of the Endoscope-Assisted One-Stage Approach for Crooked Nose.

BACKGROUND: A crooked nose is an external nose deformity predominantly caused by congenital aplasia or acquired secondary to trauma or surgery, often accompanied by a deviated nasal septum. Patients with crooked nose have dual needs to improve both esthetic and functional problems. METHODS: The clinical and photographic information of 48 patients diagnosed with a crooked nose and nasal septum deviation treated from January 2018 to January 2022 was acquired. The morphology and functional effects were investigated by evaluating the general condition of the operation, measuring the esthetic indexes of the nose, and subjectively scoring. RESULTS: For both morphology and function, endoscopy-assisted one-stage correction showed positive results in this study. The external nose deviation distance postoperatively measured 1.28 (0.85, 1.97) mm, which significantly decreased from the preoperative value of 3.96 (3.31, 5.29) mm. The scores of doctors and irrelevant medical students on nose morphology increased significantly from 4.75±1.88 and 3.84±0.76 to 6.48±1.21 and 7.21±0.67, respectively. The rhinoplasty outcome evaluation score and the "nasal obstruction symptom evaluation "score of patients were both significantly improved ( t = -7.508 and t =6.310, respectively, P < 0.001). CONCLUSION: Endoscope-assisted one-stage correction of the crooked nose can restore nasal morphology, improve the symptoms of nasal obstruction, and achieve patient satisfaction. It is a minimally invasive, safe, effective, and fast recovery approach for patients who need to solve both esthetic and functional problems.

The Management of Posttraumatic Nasal Deformities.

Nasal bone fractures are the most common fractures of the facial skeleton and are often accompanied by bony, cartilaginous, and soft tissue injuries. These injuries are often complex, and when untreated or inadequately treated, can lead to posttraumatic nasal deformity. The most common deformities are the crooked nose and the saddle nose. Both deformities may result in significant cosmetic and functional concerns. The treatment of these deformities can be complex, requiring careful evaluation of the nose and thoughtful planning to correct the cosmetic deformity and restore functional integrity. The rhinoplasty surgeon will benefit from having a large repertoire of techniques to achieve these repairs. In this article, we discuss the options and concepts for the management of nasal bone fractures as well as complicated posttraumatic nasal deformity. Level of evidence is not available.

Three-dimensional reconstruction reveals the correlation between the extent of alveolar clefts and secondary nasal deformity in adults.

OBJECTIVES: This study aimed to explore the relationship between alveolar cleft and secondary nasal deformity post unilateral cleft lip repair in adults. METHODS: A total of 27 patients aged 16-30 years old with unilateral secondary nasal deformity and alveolar cleft were included, 13 of whom underwent bone grafting. Spiral CT data of all preoperative and postoperative patients who had alveolar bone grafting were collected. Then, Mimics software was used for three-dimensional reconstruction to evaluate the correlation between the width, height, and volume of the alveolar cleft and those of the nasal deformity. The difference in nasal deformity before and after alveolar bone grafting was also explored. RESULTS: The width of the alveolar cleft was positively correlated with the difference in bilateral nostril floor width (P<0.05). As the effective depth of the alveolar cleft increased, the sub-alare inclination angle largened (P<0.05). However, no significant difference was found in the nasal deformity between before and after alveolar bone grafting. CONCLUSIONS: Alveolar cleft is closely related to secondary nasal deformities post unilateral cleft lip repair, especially nasal floor deformities. Alveolar bone grafting benefits adult patients for the improvement of secondary nasal deformities post unilateral cleft lip repair.

The Effectiveness of Releasing the Lower Lateral Cartilage Through Intranasal Z-plasty Incision on the Vestibular Web During Secondary Correction of Nasal Deformities in Complete Unilateral Cleft Lip and Palate.

The primary goal in the secondary correction of unilateral cleft lip nose deformity is to achieve symmetry of the nose and nostril. This study aimed to investigate the efficacy of freeing the lower lateral cartilage from the pyriform ligament through an intranasal Z-plasty incision on the vestibular web in adult patients with complete unilateral cleft lip and palate. Thirty-six patients with complete unilateral cleft lip and palate, who underwent open rhinoplasty between August 2014 and December 2021, were identified retrospectively. Five parameters for nose form and nostril symmetry were measured on basal views through 2-dimensional photographic analysis. The patients were divided into subgroups with or without septoplasty. Cleft-to-non-cleft ratios between the Z (13 patients) and non-Z groups (23 patients) were compared using the Mann-Whitney U test. The mean follow-up was 12.9 months (6-31 mo). In the Z group, there were significant differences between the preoperative and postoperative values for nostril angulation, regardless of septoplasty (all P <0.05). Despite septoplasty, significant differences in the postoperative changes in nostril angulation were found between the Z and non-Z groups (all P <0.05). Intranasal Z-plasty on the plica vestibularis is an effective technique for releasing the lower lateral cartilage, improving the nostril asymmetry in cleft lip nose deformity.