Spontaneous Closure of Congenital Cranial Defect: Is Early Surgical Intervention Warranted?

Infantile cranial development typically occurs in a predictable sequence of events; however, less is known about how the development occurs in isolated, nonsyndromic congenital craniofacial anomalies. Furthermore, the timing of pediatric cranioplasty has been extrapolated from adult studies. Thus, the management of nonsyndromic congenital craniofacial anomalies presents with unique challenges to the craniofacial surgeon. The authors describe the case of a baby girl who was born with right Tessier 3 cleft, cleft palate, anophthalmos, and severe left craniofacial microsomia with Pruzansky grade III left mandibular anomaly. By analyzing 3-dimensional chronological models of the patient, the authors found that her abnormal fontanelle initially increased in size until 22 weeks of age, with subsequent spontaneous closure at a rate of 60.53 mm2/y. Although similar cranial anomalies are typically surgically corrected early in life, delaying treatment until after 2 years of age may be appropriate in some patients, obviating surgical morbidity in the newborn period.

CLINICOROENTGENOLOGICAL PECULIARITIES OF THE CONGENITAL AND ACQUIRED CRANIOFACIAL ANOMALIES.

The aim of the research was to study the relationship between the X-ray changes in the bones of the skull, the structure of the upper respiratory tract and concomitant general somatic diseases in patients with congenital and acquired craniomaxillofacial anomalies. The study included 52 patients aged 1 to 3 and 3 to 7 years, with congenital and acquired lower micrognathia in 19 (36.53±5.3)% and upper micrognathia in 33 (63.46±5.3)%. There were used clinical methods (questioning, examination, palpation), instrumental methods (multispiral computer tomography, X-ray cephalometric analysis of the bones of the facial skeleton, oropharynx, and bony pharynx). The obtained results of the clinical and radiographic examination made it possible to assert that among the patients with congenital defects of the jaws, not only changes in the facial skeleton dominate, mostly in the form of upper micrognathia and, to a lesser extent, lower micrognathia, but also the presence of somatic developmental defects in the form of disorders of the nervous system, pathologies of ENT-organs and ophthalmic defects. The identified malformations caused the violations of a number of important functions: breathing, swallowing, chewing, and speech formation. This connection was followed in particular in patients with syndromic craniosynostosis, namely, underdevelopment of the skull base combined with upper micrognathia and retroposition of the maxillary complex in the skull. The frequency and spectrum of concomitant somatic pathology depended on the nature of dentofacial anomalies. All patients with upper micrognathia had craniostenosis with the deformations of the brain skull and eye sockets. Among the patients with lower micrognathia, all those examined were found to have disorders of the development of the ENT-organs.

Report of a novel variant in the FAM111A gene in a fetus with multiple anomalies including gracile bones, hypoplastic spleen, and hypomineralized skull.

Kenny-Caffey syndrome type 2 (KCS2) and osteocraniostenosis (OCS) are allelic disorders caused by heterozygous pathogenic variants in the FAM111A gene. Both conditions are characterized by gracile bones, characteristic facial features, hypomineralized skull with delayed closure of fontanelles and hypoparathyroidism. OCS and KCS2 are often referred to as FAM111A-related syndromes as a group; although OCS presents with a more severe, perinatal lethal phenotype. We report a novel FAM111A mutation in a fetus with poorly ossified skull, proportionate long extremities with thin diaphysis, and hypoplastic spleen consistent with FAM111A-related syndromes. Trio whole exome sequencing identified a p.Y562S de novo missense variant in the FAM111A gene. The variant shows significant similarity to other reported pathogenic mutations fitting proposed pathophysiologic mechanism which provide sufficient evidence for classification as likely pathogenic. Our report contributed a novel variant to the handful of OCS and KCS2 cases reported with pathogenic variants.

Parallel evolution of regressive and constructive craniofacial traits across distinct populations of Astyanax mexicanus cavefish.

Life in complete darkness has driven the evolution of a suite of troglobitic features in the blind Mexican cavefish Astyanax mexicanus, such as eye and pigmentation loss. While regressive evolution is a hallmark of obligate cave-dwelling organisms, constructive (or augmented) traits commonly arise as well. The cavefish cranium has undergone extensive changes compared with closely-related surface fish. These alterations are rooted in both cranial bones and surrounding sensory tissues such as enhancements in the gustatory and lateral line systems. Cavefish also harbor numerous cranial bone asymmetries: fluctuating asymmetry of individual bones and directional asymmetry in a dorsal bend of the skull. This asymmetry is mirrored by the asymmetrical patterning of mechanosensory neuromasts. We explored the relationship between facial bones and neuromasts using in vivo fluorescent colabeling and microcomputed tomography. We found an increase in neuromast density within dermal bone boundaries across three distinct populations of cavefish compared to surface-dwelling fish. We also show that eye loss disrupts early neuromast patterning, which in turn impacts the development of dermal bones. While cavefish exhibit alterations in cranial bone and neuromast patterning, each population varied in the severity. This variation may reflect observed differences in behavior across populations. For instance, a bend in the dorsal region of the skull may expose neuromasts to water flow on the opposite side of the face, enhancing sensory input and spatial mapping in the dark.

Is Noninvasive Prenatal Screening Appropriate for Pregnant Women Age 35 or Older In Cases if Isolated Fetal Nasal Bone Abnormalities in The Chinese Han Population?

BACKGROUND Ethnic background may affect the prevalence of nasal bone absence and the length of the nasal bone. This study aimed to elucidate the significance of absent or hypoplastic fetal nasal bone in the Chinese Han population and to formulate an optimal management plan for patients age 35 or older in cases of isolated abnormal fetal nasal bone. MATERIAL AND METHODS We prospectively assigned pregnant women whose fetuses had nasal bone absence or hypoplasia to separate groups according to their choice for noninvasive prenatal screening (NIPS) between January 1, 2013, and December 31, 2018. Demographic data, ultrasound findings, results of conventional maternal serum screening and NIPS, fetal karyotype, pregnancy outcomes, and expenses associated with prenatal testing were recorded. The incidence and odds ratio of nasal bone abnormality and the sensitivity and specificity of different prenatal genetic screening tests were calculated. RESULTS A total of 1946 cases with fetal nasal bone absence or hypoplasia were included. Cases of isolated nasal bone abnormality (1736 cases) were divided into the NIPS group (Gr 1, n=429) and the non-NIPS group (Gr 2, n=1307). Sixty-four cases involved chromosomal abnormality. The sensitivity, specificity, and positive and negative predictive values of NIPS in Gr 1 were 100%, 100%, 100%, and 100%, respectively. The odds ratio of fetal chromosomal abnormalities for isolated fetal nasal bone abnormalities when maternal age was ≥35 was 4.615 (95% CI: 1.592-13.381). The cost-effectiveness ratio of contingent screening (NIPS first) was significantly lower than amniocentesis directly. CONCLUSIONS The nasal bone provides an important marker for chromosome abnormalities in some populations, but to a lesser extent in the Chinese Han population. NIPS is an excellent first option for follow-up among pregnant women age ≥35 in cases of absent or hypoplastic fetal nasal bone in the first trimester ultrasound scan.

Animal Models for Understanding Human Skeletal Defects.

Skeletal defects, such as cleft palate, scoliosis, and shortening of the limb bones are common in the human population. Animal models have been essential for characterizing the molecular and cellular mechanisms that underlie these and other skeletal disorders. This chapter will explore the cellular origins of the vertebrate skeleton and introduce a selection of animal models for human disorders of the skull and facial bones, spinal column, and limbs. The common genetic pathways that build the skeleton of various vertebrate species and how these similarities facilitate the study of human developmental processes in laboratory animals will be a focus of discussion. This chapter will also highlight how current genome editing technologies can be applied to model various perturbations of human chromatin structure in laboratory animals.

Anatomical and biochemical evidence for Treponema pallidum in a 19th to early twentieth century skeletal cadaver.

This report summarizes findings relating to the biochemical and skeletal evidence for Treponema pallidum in an unusually old case of congenital syphilis. In 1951, the Milwaukee Public Museum acquired skeletal remains from the Surgical School of Marquette University. The male was identified as a 60-65-year-old, that was suffering from congenital syphilis. His remains are now part of the anthropological collections of Wisconsin Lutheran College (Milwaukee, Wisconsin). Venereal Disease Research Laboratory (VDRL) and Rapid Plasma Reagin (RPR) tests were used to verify the presence of the bacteria-generated antibodies, while mass spectrometry testing provided indirect evidence for the historical treatment of the disease. Notably, antibody detection in human remains of this age is rare. These initial results support what is known of syphilis and its treatment prior to the wide scale, clinical use of penicillin therapy, and describe evidence for long-term skeletal symptoms of congenital syphilis in century-old human remains.

Impact of Le-Fort I osteotomy on anatomical and functional aspects of the nasal airway and on quality of life.

OBJECTIVES: Orthognathic surgery is a well-established procedure for skeletal deformities. Beneficial influences to the posterior airway space (PAS) have been described, but little is known about the subjective aesthetical and functional nasal aspects after orthognathic surgery. The aim of this study was to evaluate nasal airflow by anterior rhinomanometry and volumetric changes in the nasal airway space after mono- or bimaxillary surgery using cone-beam computed tomography (CBCT) and a new segmentation software. Furthermore, changes of patient's quality of life (QoL) should be assessed. METHODS: Ten patients (9 skeletal class malformation III, 1 skeletal class malformation I) were included. CBCT images, rhinological inspections and anterior rhinomanometries were performed before (T0) and after surgery (T1). All patients completed the FROI-17, the ROE and the SF-36 questionnaires. RESULTS: A significant postoperative gain for nasal airway volume compared with the baseline was shown (p < 0.014). No statistically significant differences between pre- and postoperative flow rates were found (p = 0.114). Pre- and postoperative cohorts did not differ in responses of disease-specific (ROE and FROI-17) and generic QoL questionnaires (SF-36). CONCLUSION: Maxillary relocation surgery leads to a significant increase in nasal airway space. Subjectively, orthognathic patients did not experience any functional but psychosocial aspects after bimaxillary surgery.

Analysis of Airway and Midface in Crouzon Syndromes.

BACKGROUND: Crouzon syndrome is associated with severe respiratory impairment of the upper airway due in part to midfacial dysmorphology. We calculated the distinctive nasal diameter and pharyngeal airway volume in patients with Crouzon syndrome and compared them with age-matched control subjects. METHODS: Children with computed tomography scans in the absence of surgical intervention were included. Computed tomography scans were digitized and manipulated using Surgicase CMF (Materialise). Craniometric data relating to the midface and airway were collected. For all linear measurements, mean percent increases or decreases were calculated relative to the size of control subjects, and volumetric assessment of the airway was tabulated. Statistical analysis was performed using t test. RESULTS: Twenty-six computed tomography scans were included (control n = 17, Crouzon n = 9). All children were in early mixed dentition. Pharyngeal airway volume was decreased in patients with Crouzon syndrome relative to control subjects by 46% (P = 0.003). The distance from the posterior tongue to the posterior pharyngeal wall decreased 31% when comparing the Crouzon group versus the control (P = 0.04). CONCLUSIONS: Three-dimensional analysis revealed notably decreased pharyngeal and nasal airway volumes in patients with Crouzon syndrome, but nasal bone tissue and soft tissue measurements showed very little change between patients and control subjects.

Complete bilateral Tessier's facial cleft number 5: surgical strategy for a rare case report.

The oro-ocular cleft number 5 according to the Tessier classification is one of the rarest facial clefts and few cases have been reported in the literature. Although the detailed structure of rare craniofacial clefts is well established, the cause of these pathological conditions is not. There are no existing guidelines for the management of this particular kind of cleft. We describe the case of a 19-month-old girl with a complete bilateral facial cleft. We describe the surgical steps taken to achieve the primary correction of the soft tissue deformation. Embryologic development and radiological approach are discussed, as are also the psychological and social aspects of severe facial deformities.

Chitayat syndrome: hyperphalangism, characteristic facies, hallux valgus and bronchomalacia results from a recurrent c.266A>G p.(Tyr89Cys) variant in the ERF gene.

BACKGROUND: In 1993, Chitayat et al., reported a newborn with hyperphalangism, facial anomalies, and bronchomalacia. We identified three additional families with similar findings. Features include bilateral accessory phalanx resulting in shortened index fingers; hallux valgus; distinctive face; respiratory compromise. OBJECTIVES: To identify the genetic aetiology of Chitayat syndrome and identify a unifying cause for this specific form of hyperphalangism. METHODS: Through ongoing collaboration, we had collected patients with strikingly-similar phenotype. Trio-based exome sequencing was first performed in Patient 2 through Deciphering Developmental Disorders study. Proband-only exome sequencing had previously been independently performed in Patient 4. Following identification of a candidate gene variant in Patient 2, the same variant was subsequently confirmed from exome data in Patient 4. Sanger sequencing was used to validate this variant in Patients 1, 3; confirm paternal inheritance in Patient 5. RESULTS: A recurrent, novel variant NM_006494.2:c.266A>G p.(Tyr89Cys) in ERF was identified in five affected individuals: de novo (patient 1, 2 and 3) and inherited from an affected father (patient 4 and 5). p.Tyr89Cys is an aromatic polar neutral to polar neutral amino acid substitution, at a highly conserved position and lies within the functionally important ETS-domain of the protein. The recurrent ERF c.266A>C p.(Tyr89Cys) variant causes Chitayat syndrome. DISCUSSION: ERF variants have previously been associated with complex craniosynostosis. In contrast, none of the patients with the c.266A>G p.(Tyr89Cys) variant have craniosynostosis. CONCLUSIONS: We report the molecular aetiology of Chitayat syndrome and discuss potential mechanisms for this distinctive phenotype associated with the p.Tyr89Cys substitution in ERF.

Role of Matrix Gla protein in midface development: Recent advances.

Craniofacial development is a delicate process that involves complex interactions among cells of multiple developmental origins, their migration, proliferation, and differentiation. Tissue morphogenesis of the craniofacial skeleton depends on genetic and environmental factors, and on specific signaling pathways, which are still not well understood. Developmental defects of the midface caused by the absence, delays, or premature fusion of nasal and maxillary prominences vary in severity; leading to clefts, hypoplasias, and midline expansion. In the current review, we focus on the importance of the chondrocranium in craniofacial growth and how its impaired development leads to midface hypoplasia. More importantly, we reported how Matrix Gla protein (MGP), a potent inhibitor of extracellular matrix mineralization, facilitates midface development by preventing ectopic calcification of the nasal septum. In fact, MGP may act as a common link in multiple developmental pathologies all showing midface hypoplasia caused by abnormal cartilage calcification. This brief review discusses the gap in knowledge in the field, raises pertinent questions, which remain unanswered, and sheds light on the future research directions.

Is Preoperative Maintenance of the Curve of Spee Associated With an Increase of Facial Height in Class II Short Face Orthognathic Patients? A Never Answered Dogmatic Question.

PURPOSE: To determine the association of maintaining the curve of Spee (COS) before surgery with post-treatment facial height in patients with Class II short face syndrome undergoing combined orthodontic and orthognathic treatment. MATERIALS AND METHODS: In this retrospective cohort study, the clinical and radiologic data of all patients with Class II short face syndrome who underwent combined orthodontic and orthognathic treatment were reviewed. The primary outcome variables were 1) preoperative COS and 2) post-treatment facial height. Depth of the COS and skeletal and soft tissue relations were measured on digital lateral cephalometric radiographs. Descriptive and bivariate statistics were performed. RESULTS: The sample was composed of 20 patients. Statistical analysis showed a significant increase of soft tissue facial height after treatment (P < .02). Preoperative depth of the COS was significantly associated with changes in sagittal skeletal relations (angle formed by the sella, nasion, and B point [SNB], correlation [cor] = -0.54, P < .02; angle formed by the A point, nasion, and B point [ANB], cor = 0.43, P < .06). These changes and changes in overjet were associated with the post-treatment increase of lower facial height (SNB, cor = 0.70, P < .001; ANB, cor = -0.69, P < .001; overjet, cor = -0.55, P < .049). The ratios of upper to lower soft tissue facial height and upper to lower lip height were improved to near normal values (1.0 and 0.5, respectively) for most patients. CONCLUSION: In patients with Class II short face orthognathism, the present study found that maintaining the COS before surgery was associated with 1) an increase of soft tissue facial height and 2) an improvement of the ratio of upper to lower facial height and the ratio of upper to lower lip height to near normal values. Moreover, the depth of the COS was correlated with the post-treatment increase of facial height through changes in skeletal relations and was related to the degree of severity of the mandibular deficiency.

Extramucosal pyriplasty without stenting for management of pyriform aperture stenosis.

PURPOSE: The current management options of congenital pyriform aperture stenosis (CNPAS) are either conservative measures awaiting further growth of the bony nasal framework or surgical intervention that focuses on bone removal from the margin of the pyriform aperture (PA) without exposure of the nasolacrimal duct (NLD) followed by stenting. Recently, CT measurements of the nasal cavity in CNPAS have shed light that the site of maximal bony obstruction corresponds to the bony buttress encasing the NLD rather than the margin of the PA as initially thought. Herein, we present an extramucosal pyriplasty technique that can be used without stenting to enlarge the PA and achieve immediate and sustained relief of nasal obstruction. METHODS: Retrospective chart review of 4 patients with radiologically confirmed CNPAS who had undergone extramucosal pyriplasty without stenting during the period from 2012 to 2016. RESULTS: Three patients were full term without any clinically detectable congenital anomaly. The fourth patient was preterm infant who needed ICU management. On computerized tomography scan, the PA width ranged from 5.8 to 7.1 mm with a mean of 6.4 mm while site of maximal stenosis ranged from 5.4 to 6.8 with a mean of 6 mm. Extramucosal pyriplasty relieved nasal obstruction and restored normal oral feeding in all patients. Postoperative follow-up endoscopy revealed an adequately patent airway with no scarring, granulation or restenosis. CONCLUSIONS: Extramucosal pyriplasty with decompression of the NLD without stenting is a treatment modality for CNPAS that provides prompt sustainable relief of nasal obstruction and avoids the drawbacks of stenting and shortcomings of the current conservative methods.

Digital-Facial Translocation in Amniotic Band Sequence: Evidence of the Intrinsic Theory.

Amniotic band sequence is a complex congenital anomaly in which infants with typically no known genetic mutation have bands of maternal amniotic tissue wrapped around body parts, most commonly the limbs and digits. The authors report a novel variation on this presentation in 3 patients from 2 centers with complex craniofacial clefting and amniotic band sequence. They presented with hypertelorism, different forms of complex craniofacial clefting, and bands connecting ipsilateral hands to facial clefts, with digital-facial translocation in 2 cases. These findings support a model in which complex craniofacial clefts result in areas of exposed, sticky, and temporally and spatially coincident mesenchyme within the embryo that are susceptible to adherence of ipsilateral fetal hands. This strongly supports the intrinsic and adhesion theories of the etiology of amniotic band syndrome.

The Arkansas Tessier Number 3 Cleft Experience: Soft Tissue and Skeletal Findings With Primary Surgical Management: Four-Step Approach.

Tessier No 3 facial cleft (oro-nasal-ocular clefts) is the rarest and most challenging of all the Tessier clefts. Reports on Tessier No 3 clinical findings, surgical techniques, and outcomes are varied due to the scarcity of patients and the wide range of phenotypic findings. The authors present our experience of 2 children born with Tessier No 3 clefts who were both managed at the Arkansas Children's Hospital. Our purpose is to add knowledge on this rare craniofacial cleft by providing detailed soft tissue findings, skeletal findings, operative techniques, early postoperative outcome, and suggestions of a treatment protocol.Both were born at 38 weeks gestation and had multiple associated anomalies including: syndactyly, limb anomalies, cardiac defects, and encephalocele in Patient 1 and hydrocephalus and dysphagia in Patient 2. While both patients had a bilateral cleft lip and palate, Patient 1 had a severe left-sided cleft and Patient 2 had a right-sided incomplete cleft. A multidisciplinary team of specialists in Plastic Surgery, Otolaryngology, and Oculoplastics were assembled to devise a top-down approach for repair. In brief, our surgical sequence for both infants was a dorsal nasal Reiger flap to level the ala, cheek advancement flap along with medial canthal repositioning, and more traditional bilateral cleft lip repair using a modified Millard technique. Postoperatively, Patient 1 experienced some early scarring, medial canthal rounding, lagophthalmos, and cicatricial retraction of the lower lid and patient 2 demonstrated under-correction of the displaced ala but had satisfactory medial canthal position.Future evaluations will include serial photography and annual 3-dimensional computed tomography scans to evaluate the soft tissue and bony growth. After these initial procedures, both infants will be followed for routine cleft clinical and surgical care.

Repairing a Facial Cleft by Polyether-Ether-Ketone Implant Combined With Titanium Mesh.

The Tessier Number 4 cleft is one of the rarest, most complex craniofacial anomalies that presents difficulties in surgical treatment. In this article, we report a case of simultaneous facial depression, eye displacement, and medial canthus deformity. In this case, the maxillary bony defect was reconstructed using computer-assisted design computer-assisted manufacturing (CAD-CAM) polyether-ether-ketone (PEEK) material, and the orbital floor defect was repaired with AO prefabricated titanium mesh. Additionally, the medial canthus was modified with canthopexy and a single Z-plasty flap. Owing to its relative rarity and varied clinical presentations, no definitive operative methods have been accepted for Tessier No. 4 facial cleft. This study presents the combination of CAD-CAM manufactured PEEK material and titanium mesh as an alternative approach for reconstructing the bony defect of Tessier No. 4 facial clefts.

Facial Paralysis in Patients With Hemifacial Microsomia: Frequency, Distribution, and Association With Other OMENS Abnormalities.

Although facial paralysis is a fundamental feature of hemifacial microsomia, the frequency and distribution of nerve abnormalities in patients with hemifacial microsomia remain unclear. In this study, the authors classified 1125 cases with microtia (including 339 patients with hemifacial microsomia and 786 with isolated microtia) according to Orbital Distortion Mandibular Hypoplasia Ear Anomaly Nerve Involvement Soft Tissue Dependency (OMENS) scheme. Then, the authors performed an independent analysis to describe the distribution feature of nerve abnormalities and reveal the possible relationships between facial paralysis and the other 4 fundamental features in the OMENS system. Results revealed that facial paralysis is present 23.9% of patients with hemifacial microsomia. The frontal-temporal branch is the most vulnerable branch in the total 1125 cases with microtia. The occurrence of facial paralysis is positively correlated with mandibular hypoplasia and soft tissue deficiency both in the total 1125 cases and the hemifacial microsomia patients. Orbital asymmetry is related to facial paralysis only in the total microtia cases, and ear deformity is related to facial paralysis only in hemifacial microsomia patients. No significant association was found between the severity of facial paralysis and any of the other 4 OMENS anomalies. These data suggest that the occurrence of facial paralysis may be associated with other OMENS abnormalities. The presence of serious mandibular hypoplasia or soft tissue deficiency should alert the clinician to a high possibility but not a high severity of facial paralysis.

Clinical delineation of a subtype of frontonasal dysplasia with creased nasal ridge and upper limb anomalies: Report of six unrelated patients.

Frontonasal dysplasias are rare congenital malformations of frontonasal process-derived structures, characterized by median cleft, nasal anomalies, widely spaced eyes, and cranium bifidum occultum. Several entities of syndromic frontonasal dysplasia have been described, among which, to date, only a few have identified molecular bases. We clinically ascertained a cohort of 124 individuals referred for frontonasal dysplasia. We identified six individuals with a similar phenotype, including one discordant monozygous twin. Facial features were remarkable by nasal deformity with creased ridge and depressed or absent tip, widely spaced eyes, almond-shaped palpebral fissures, and downturned corners of the mouth. All had apparently normal psychomotor development. In addition, upper limb anomalies, frontonasal encephalocele, corpus callosum agenesis, choanal atresia, and congenital heart defect were observed. We identified five reports in the literature of patients presenting with the same phenotype. Exome sequencing was performed on DNA extracted from blood of two individuals, no candidate gene was identified. In conclusion, we report six novel simplex individuals presenting with a specific frontonasal dysplasia entity associating recognizable facial features, limb and visceral malformations, and apparently normal development. The identification of discordant monozygotic twins supports the hypothesis of a mosaic disorder. Although previous patients have been reported, this is the first series, allowing delineation of a clinical subtype of frontonasal dysplasia, paving the way toward the identification of its molecular etiology.

Surgery Navigation in Treating Congenital Midfacial Dysplasia of Patients With Facial Cleft.

AIM: To explore a new accurate way for the treatment of congenital midfacial dysplasia in facial cleft patients. MATERIALS AND METHODS: Between November 2015 and November 2016, 8 patients with nasal deformity and midfacial dysplasia (Tessier Nos. 3-11 cleft) were collected (median age, years; range = 15-20 years). Expanded frontal flap for nasal reconstruction and image-guided navigation-assisted surgery for modified nasal-maxillary-hard palatine osteotomy to advance the peri-pyriform bone structure were performed in all the patients. After 6 to 12 months of follow-up, the authors analyzed the differences between preoperative planning and postoperative results through computed tomography data. RESULTS: Patients were satisfied with surgery, and computed tomography data showed that there was little difference between preoperative planning and postoperative results with the navigation-assisted surgery. CONCLUSION: Using expanded frontal flap with navigation-assisted surgery for peri-pyriform advancement, the authors could treat congenital nasal deformity and midfacial dysplasia effectively, accurately, and safely in craniofacial cleft patients.