Ectomesenchymal Chondromyxoid Tumor: A Rare Association With an Asymmetrical Soft Palate Cleft.

Ectomesenchymal chondromyxoid tumor (ECT) is a rare oral lesion first described by Smith et al. in 1995. These tumors are typically painless, slow growing and benign masses occurring predominantly on the anterior tongue dorsum. Prior to this seminal report, many ECTs may have been misdiagnosed due to the histological similarities with other lesions. Immunohistochemical stains aid in definitive diagnosis of an ECT. A total of 39 papers since published have reported 96 patients with ECT. Most lesions involve the anterior aspect of the tongue, with only 6 occurring in the posterior tongue and 2 involving the hard palate. ECTs are considered to develop from ectomesenchymal cells of neural crest cells that have migrated to the tongue during embryological development. This paper is of a rare case of ECT of the posterolateral tongue occurring in association with an unusual asymmetrical soft palate cleft. It is postulated that since the tongue develops before the formation of the soft palate, an ECT lesion occurring on the posterior aspect may have a causal contribution to the development of the soft palate cleft.

Gordon syndrome: literature review and a report of two cases.

The aim of this article is to publish a literature review and report on two new cases of Gordon syndrome (GS), a rare syndrome documented to have an autosomal dominant inheritance pattern or to occur sporadically; it is characterized by camptodactyly, cleft palate, and talipes equinovarus. We report two exceptional cases of GS where both patients were also diagnosed with congenital myopathy, and one developed malignant hyperthermia. These are the first two cases reported where patients were diagnosed with both GS and congenital myopathy or where GS is associated with malignant hyperthermia.

The Risk of Orofacial Cleft Lip/Palate Due to Maternal Ambient Air Pollution Exposure: A Call for Further Research in South Africa.

Background: Despite being underreported, orofacial cleft lip/palate (CLP) remains in the top five of South Africa's most common congenital disorders. Maternal air pollution exposure has been associated with CLP in neonates. South Africa has high air pollution levels due to domestic burning practices, coal-fired power plants, mining, industry, and traffic pollution, among other sources. We investigated air pollutant levels in geographic locations of CLP cases. Methods: In a retrospective case series study (2006-2020) from a combined dataset by a Gauteng surgeon and South African Operation Smile, the maternal address at pregnancy was obtained for 2,515 CLP cases. Data from the South African Air Quality Information System was used to calculate annual averages of particulate matter (PM) concentrations of particles < 10 µm (PM10) and < 2.5 µm (PM2.5). Correlation analysis determined the relationship between average PM2.5/PM10 concentrations and CLP birth prevalence. Hotspot analysis was done using the Average Nearest Neighbor tool in ArcGIS. Results: Correlation analysis showed an increasing trend of CLP birth prevalence to PM10 (CC = 0.61, 95% CI = 0.38-0.77, p < 0.001) and PM2.5 (CC = 0.63, 95% CI = 0.42-0.77, p < 0.001). Hot spot analysis revealed that areas with higher concentrations of PM10 and PM2.5 had a higher proclivity for maternal residence (z-score = -68.2, p < 0.001). CLP birth prevalence hotspot clusters were identified in district municipalities in the provinces of Gauteng, Limpopo, North-West, Mpumalanga, and Free State. KwaZulu-Natal and Eastern Cape had lower PM10 and PM2.5 concentrations and were cold spot clusters. Conclusions: Maternal exposure to air pollution is known to impact the fetal environment and increase CLP risk. We discovered enough evidence of an effect to warrant further investigation. We advocate for a concerted effort by the government, physicians, researchers, non-government organizations working with CLP patients, and others to collect quality data on all maternal information and pollutant levels in all provinces of South Africa. Collaboration and data sharing for additional research will help us better understand the impact of air pollution on CLP in South Africa.

South African speech-language therapists' opinion of their training in cleft lip and palate and craniofacial deformities.

BACKGROUND: Speech care of cleft lip and/or palate (CLP) and craniofacial deformities (CFD) is complex and lengthy and requires collaboration amongst different disciplines. Consequently, it is important to provide academic educational models that include didactics, online learning and clinical exposure in CLP and CFD treatment, and participation in established cleft palate multidisciplinary team management. OBJECTIVES: To obtain information regarding: (1) the perceived adequacy of CLP and CFD academic education of speech-language therapists (SLTs); (2) the professional services that SLTs offer to CLP and CFD patients; and (3) the educational needs of SLTs in this field. METHOD: A 54-item online survey to collect quantitative data was conducted by telephone and email using a randomised sample of SLTs in different areas of South Africa. RESULTS: The questionnaire was completed by 123 SLTs, 70% of whom had more than 10 years of professional experience. Of the respondents, 81% acknowledged their limited clinical exposure during their academic education. Only 42% of the professionals offer treatment for CLP and CFD patients. Of the respondents, 96% agreed on the need to improve CLP and CFD academic education, and the majority recommended certified courses, continued-education workshops and online resources. CONCLUSION: The findings indicate that SLTs academic training is perceived to be significantly limited in the cleft palate and craniofacial fields. Thus, there is a strong need at the undergraduate level for clinical training and exposure to multidisciplinary management. At post-graduate level there is a need to establish an educational strategy to meet the needs of SLTs providing CLP and CFD care. Participants suggested that programmes for continuing professional education, degree courses and online resources be designed to provide practising clinicians with updated information and guidance in management of CLP and CFD patients.

Holoprosencephaly with Clefts: Data of 85 Patients, Treatment, and Outcome: Part 2: Management, Surgical Treatment, and Unexpected Aspects of Holoprosencephaly Cleft Patients.

CONTEXT: Cleft patients with holoprosencephaly (HPE) provide a wide clinical spectrum. Besides accessory agenesis of facial tissue structures, spanning from a single central incisor to the columella, up to the entire prolabium-premaxilla complex, brain deformities with various functional deficits may prevail, just like normal brain development. Making a precise diagnosis, just like choosing the most appropriate treatment plan often is challenging. A literature and chart review comprising 85 HPE cleft cases at the Cleft Clinic of the University of Pretoria, South Africa, was performed. It yielded pertinent diagnostic criteria and collected information about pregnancy history, brain development and survival rate as well as the initial perioperative management and the course of postsurgical midfacial growth. AIMS OF PART 2: The aim is to highlight how the here presented classification system of HPE cleft patients according to their clinical picture may facilitate the most appropriate treatment protocol. MATERIALS AND METHODS: The classification system elaborated in Part I due to diagnostic criteria facilitated establishing classification related treatment protocol for 85 cleft cases with HPE. RESULTS: According to diagnostic criteria, HPE cleft cases can be subdivided into (1) columella complex agenesis (Ag-Colum), (2) prolabium-premaxilla-columella complex agenesis in cleft lip-alveolus deformities (Ag-CLA), (3) prolabium-premaxilla-columella complex agenesis in complete hard and soft palate clefts (Ag-CLAP), and (4) "standard" uni-or bilateral CLA or CLAP (HPE-Std-cleft), including cases with an atrophic premaxilla with or without single central incisors. Relevant treatment protocols according to the particular classification are highlighted with figures and intra-operative pictures. CONCLUSION: This paper addresses the following aspects in cleft patients with HPE: A subdivision into four groups, the 3-in-1 surgical approach, the anteriorly directed midfacial growth and maternal HIV infection.

Holoprosencephaly with Clefts: Data of 85 Patients, Treatment and Outcome: Part 1: History, Subdivisions, and Data on 85 Holoprosencephalic Cleft Patients.

CONTEXT: Cleft patients with Holoprosencephaly (HPE) constitute a controversy due to a variable facial appearance. HPE appearance varies from only a columella to a prolabium-premaxilla complex agenesis up to a common unilateral or bilateral cleft lip and palate with a single central incisor, various brain deformities, and/or even normal brain development. It is challenging to designate such various appearances, to understand their etiopathogenesis, and to choose the most appropriate management. Literature was reviewed for diagnostic criteria, pregnancy history, clinical findings, brain development, survival rate, initial perioperative management, and postsurgical midfacial growth in cleft patients with HPE. The findings were compared with a clinical database of 85 cleft patients with HPE at the Department of Maxillofacial and Oral Surgery, University of Pretoria. AIMS OF PART 1: The aim of the study is to overcome disparities widely existing among clinicians regarding definitive diagnostic criteria, especially in cases with a common appearance of a uni- or bilateral cleft lip alveolus or cleft lip, alveolus and palate deformity, and cases presenting facial structural agenesis. MATERIALS AND METHODS: A literature search related to diagnostic criteria was compared to results of a cleft HPE database from a single tertiary institution. RESULTS: HPE cleft cases can be allocated to one of the following subdivisions: (1) columella complex agenesis (Ag-Colum), (2) prolabium-premaxilla-columella complex agenesis in cleft lip-alveolus deformities (Ag-CLA), (3) prolabium-premaxilla-columella agenesis in cases with complete cleft lip alveolus palate (Ag-CLAP), and (4) standard type (holoprosencephaly in patients with a standard cleft) with uni- or bilateral CLA or CLAP, hard and soft palate cleft (hPsP), and atrophic premaxillae, with or without single central incisor. Further, incidence, variation in brain development, and appearances in HPE cleft patients of different races and gender, epilepsy, and early death are discussed. Conclusion: This paper adds new data and facts to the existing literature related to cleft lip and palate patients suffering from HPE.

Variations of the isolated cleft of the hard palate.

A retrospective study was undertaken of 3100 facial cleft cases in order to analyse and determine the incidence of the isolated cleft of the hard palate. This presented as a very rare occurrence in three different variations, each having specific clinical appearances. The literature search revealed three publications each describing a single isolated hard palate cleft. However, these publications described hard palate clefts witnessed with other additional submucosal clefts or due to infection of the hard palate. They differ, in most cases, from the development and appearances of the clefts described in this paper. This single paper describes 13 cases, demonstrating the three variations of the isolated cleft of the hard palate.

Pierre Robin sequence: Subdivision, data, theories, and treatment - Part 1: History, subdivisions, and data.

CONTEXT: The disorder currently accepted as Pierre Robin syndrome/anomaly/sequence (PRS) has been plagued by controversy ever since initially being described. Controversy exists not only about the appropriate terminology and etiopathogenesis of the disorder but also about its management. Therefore, clinical findings and treatment outcomes of a large database of 266 PRS cases were compared with the current state of knowledge in scientific literature related to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch-up growth, midfacial hyperplasia, and the timing of management. AIMS OF PART 1: Display disparities of the widely published subject of PRS that exist within the literature. SUBJECTS AND METHODS: A literature search related to diagnostic criteria was compared to findings of one of the largest PRS databases worldwide. RESULTS: Regarding diagnostic criteria two subdivisions, the Fairbairn-Robin triad (FRT) and the Siebold-Robin sequence (SRS) can be clearly distinguished. Both present with micrognathia and glossoptosis, the former with, the latter, however, without a palatal cleft. CONCLUSIONS: According to clear diagnostic criteria, PRS has to be subdivided in the future into FRT and SRS cases, as they may require different treatment approaches.

Pierre Robin sequence: Subdivision, data, theories, and treatment - Part 2: Syndromic and nonsyndromic Pierre Robin sequence.

CONTEXT: The disorder currently accepted as Pierre Robin syndrome/anomaly/sequence (PRS) has been plagued by controversy ever since initially being described. Controversy exists not only about the appropriate terminology and etiopathogenesis of the disorder but also about its management. Clinical findings and treatment outcomes of a large database of 266 PRS cases were compared with the current state of knowledge in the scientific literature, relating to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch-up growth, midfacial hyperplasia, and the early management. AIMS OF PART 2: Contribute to the sparse scientific knowledge about pathogenesis and involved genetics. SUBJECTS AND METHODS: An analysis of this large database was conducted focusing on genetic involvement, family history, and the incidence of additional syndromes. RESULTS: Beside of differences related to clinical signs of dyspnea, feeding problems and mortality rates, various concomitant syndromes, and genetic abnormalities were found in cases of Fairbairn-Robin triad (FRT) and Siebold-Robin sequence (SRS), in addition to differences in relation to clinical signs of dyspnea, feeding problems, and mortality rates. CONCLUSION: Multiple FRT cases presented with various concomitant syndromes and genetic abnormalities, but only one type occurred in two SRS cases. The latter presented a significantly different mortality rate when compared to the FRT subgroup.

Pierre Robin sequence: Subdivision, data, theories, and treatment - Part 3: Prevailing controversial theories related to Pierre Robin sequence.

CONTEXT: The disorder currently accepted as Pierre Robin syndrome/anomaly/sequence (PRS) has been plagued by controversy ever since initially being described. Controversy exists not only about the appropriate terminology and etiopathogenesis of the disorder but also about its management. Therefore, clinical findings and treatment outcomes of a large database of 266 PRS cases were compared with the current state of knowledge in the scientific literature related to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch-up growth, midfacial hyperplasia, and the early management. AIM: The aims of Part 3 debate the controversial biological theories relating to PRS. MATERIALS AND METHODS: Oligo-/poly-hydramnios, mandibular catch-up growth, and midfacial hyperplasia, the three in the literature most prevailing theories related to PRS, have been compared and discussed with the findings provided by this large database of 266 Siebold-Robin sequence (SRS) and Fairbairn-Robin triad (FRT) cases. RESULTS: History and clinical findings evaluated in this database refute the first two theories. Although manifold midfacial appearances were demonstrated in FRT cases, a third of all SRS cases presented with mid-facial hyperplasia. CONCLUSION: The three main biological theories regarding PRS could not be verified after thorough analysis of the database.

Pierre Robin sequence: Subdivision, data, theories, and treatment - Part 4: Recommended management and treatment of Pierre Robin sequence and its application.

CONTEXT: The disorder currently accepted as Pierre Robin syndrome/anomaly/sequence (PRS) has been plagued by controversy ever since initially being described. Controversy exists not only about the appropriate terminology and etiopathogenesis of the disorder but also about its management. Therefore, clinical findings and treatment outcomes of a large database of 266 PRS cases were compared with the current state of knowledge in the scientific literature related to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch-up growth, midfacial hyperplasia, and early management. AIMS OF PART 4: To provide a systematic treatment protocol for Fairbairn-Robin triad (FRT) and Siebold Robin sequence (SRS) patients based on clinical findings and experience with 266 PRS cases. SUBJECTS AND METHODS: A plethora of treatment modalities and their outcome in literature have been compared to those applied in this database and their outcomes. RESULTS: The management of SRS/FRT depends on various factors including compromised airways, feeding difficulties, as well as the sequence of the reconstructive ladder. CONCLUSION: Based on the novel PRS subdivisions, a stepwise sequential treatment approach is outlined, addressing the particular needs of each disorder systematically.

A radiological evaluation of alveolar bone regeneration between the left and right mandibles and maxillae of the Chacma baboon.

There is a lack of information in comparing the healing rate between the left and right sides of the maxilla and mandible. Osteogenesis of alveolar bone was evaluated with digital radiology by comparing differences in bone density (BD) at different time points within the left and right maxilla and mandible. Alveolar bone defects were created in five healthy Chacma baboons. Standardised x-ray images were acquired over time and the densities of the selected trauma areas were measured pre-operatively, post-operatively and at 3 and 6 weeks post-operatively. Differences in densities were statistically tested. There was no significant difference when the grey scale averages of the combined first and fourth quadrants (right side) and combined second and third quadrants (left side) were compared pre-operatively (t = 0.70), immediately post-operatively (t = 0.34), 3 weeks post-operatively (t = 0.40) and 6 weeks post-operatively (t = 0.66). There was also no significant difference between the values for the first and second quadrants (maxilla) pre-operatively (t = 0.37), immediately post-operatively (t = 0.30), 3 weeks post-operatively (t = 0.30) and 6 weeks post-operatively (t = 0.38); the third and fourth quadrants (mandible) were also not significantly different pre-operatively (t = 0.29), immediately post-operatively (t = 0.69), 3 weeks post-operatively (t = 0.07) and 6 weeks postoperatively (t = 0.06). However, the results showed an increased predisposition of the right side to regenerate faster than the left side and indicated sufficient information to investigate the effect of laterality and preferred side of mastication on the rate of healing and alveolar BD in the maxilla and mandible.

Nonsyndromic palate Synechia with floor of mouth.

To discuss the embryological basis, sequela and management of intraoral synechia, and to report on the incidence of this condition at a facial cleft deformity clinic (FCDC), with specific attention to two rare cases of mucosal bands involving the floor of the mouth and palate. Review of the literature and a retrospective analysis of FCDC and case report of two cases. During the period of 30 years (1983-2013), the FCDC - University of Pretoria has managed in excess of 4000 cases. A review of the clinic statistics revealed only six cases in which intraoral synechiae occurred. The rarity of this condition at the FCDC is in keeping with the rare incidence in the international literature. Four syndromic cases were identified. Three cases were cleft palate lateral synechia syndrome, and one was an orofacial digital syndrome. Two nonsyndromic cases were identified, and both cases involved the floor of the mouth and palate. The attending physicians and surgeons should be aware of the most appropriate timing for management of this condition, in order to avoid unwanted sequelae. Supportive care should be provided, and emergency airway protocol should be available for all cases. A differential diagnosis should be considered which includes syndromic conditions.

The tensor stitch for soft palate closure revisited.

The tensor veli palatini muscle is involved in opening of the Eustachian tube during chewing and swallowing, allowing for equilibration of pressure between the middle ear and external environment. In patients with cleft palate, abnormal musculature in the region of the cleft results in Eustachian tube dysfunction. A palatoplasty with muscle repositioning is advocated to reduce the incidence of otitis media, which is a result of this. A special suture is described which can be incorporated into a palatoplasty procedure to further reduce the incidence of otitis media. This suture is inserted around the tendon of the tensor veli palatini muscle bilaterally, and when activated under tension in the midline, it results in opening of the Eustachian tube with improved middle ear ventilation. This novel suture results in a reduction in the incidence of otitis media due to improved middle ear ventilation and reduces tension across the suture margins.

Maxillary distraction resulting in facial advancement at Le Fort III level in cleft lip and palate patients: a report of two cases.

An 18-year-old female and a 14-year-old male who had previously received surgery for primary repair of a nonsyndromic cleft lip and palate (including alveolar defect bone grafting) unintentionally developed facial advancement at the Le Fort III level after surgical correction of their maxillary hypoplasia. The Le Fort I osteotomy, originally performed for their maxillary dentoalveolar hypoplasia, was an incomplete osteotomy. It was performed without down-fracture, leaving the pterygomaxillary and septal junctions intact. The gradual advancement of the maxilla during distraction osteogenesis was planned to correct the hypoplastic maxilla, and also prevent subsequent hypernasality; however, during the distraction procedure by means of a rigid external device both patients developed an unintentional facial advancement at the Le Fort III level.

Ozone treatment of alveolar bone in the cape chacma baboon does not enhance healing following trauma.

In the international literature, the role of Ozone (O3) in the advancement in alveolar bone healing in the absence of bone pathology was not tested before. The purpose of this study was to evaluate alveolar bone regeneration after a bone defect was created and treated with a single topical administration of O3. Alveolar bone defects were created on five healthy chacma baboons. One side of the maxilla and mandible was topically treated with a single treatment of an O3/O2 mixture (3,5-4 % O3), while the opposite sides were not treated and thus served as control. Regeneration was measured radiologically, using a standardized gray scale, as the increase in bone density in the treatment area at 3 and 6 weeks post-operative and was statistically analyzed using multivariate analysis of variance (MANOVA). There were no significant differences in densities observed between the O3/O2 mixture treatment and the control (p > 0.05). A single O3 treatment did not increase alveolar bone healing over a 3- and 6-week period in the mandible and the maxilla.

A comparison of mandibular and maxillary alveolar osteogenesis over six weeks: a radiological examination.

INTRODUCTION: Insufficient information exists on comparing radiological differences in bone density of the regeneration rate in the alveolar bone of the maxilla and mandible following the creation of similar defects in both. METHODS: Alveolar bone defects were created from five healthy Chacma baboons. Standardized x-ray images were acquired over time and the densities of the selected defect areas were measured pre-operatively, directly post-operatively and at three- and six weeks post-operatively. Differences in densities were statistically tested using ANOVA. RESULTS: The maxilla was significantly more radiologically dense (p = 0.026) than the mandible pre- operatively. No differences were obtained between the maxilla and mandible directly postoperatively and three- and six weeks post-operatively respectively; i.e. densities were not significantly different at the different time points after the defects had been created (three weeks: t = 1.08, p = 0.30; six weeks: t = 1.35, p = 0.19; three to six weeks: t = 1.20, p =0.25). The increase in density in the mandible was 106% (8.9 ± 7.6%/time versus 4.3 ± 2.7%/time) over three weeks, 28% (15.0 ± 8.1%/time versus 11.7 ± 8.0%/time) over six weeks and 56% (12.5 ± 9.7%/time versus 8.0 ± 6.9%/time) over three-to-six weeks and was higher than in the maxilla over the same intervals. CONCLUSIONS: Radiological examination with its standardized gray-scale analysis can be used to determine the difference in bone density of the maxilla and mandible. Although not statistically significant, the mandible healed at a faster rate than the maxilla, especially observed during the first three weeks after the defects were created.

Gene p63: In ectrodactyly-ectodermal dysplasia clefting, ankyloblepharon-ectodermal dysplasia, Rapp-Hodgkin syndrome.

INTRODUCTION: An analysis was made of three different syndromes associated with p63 gene mutations, known as ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC), ankyloblepharon-ectodermal dysplasia clefting syndrome (AEC or Hay-Wells) and Rapp-Hodgkin syndrome (RHS). The postoperative complications associated with their cleft reconstructions were also evaluated. MATERIALS AND METHODS: Extensive demographic information, in particular of the clinical appearances, associated malformations, and the types and complications of the reconstructive surgical procedures, were recorded of these syndromic cases occurring in a database of 3621 facial cleft deformity patients. The data was analyzed using the Microsoft Excel program. RESULTS: A total of 10 (0.28%) cases of p63 associated syndromes were recorded: EEC (6), RHS (3), and AEC (1). The following clinical cleft appearances were noted - EEC = 6: CLA 1 -right side unilateral (female); CLAP 4 - right side (1) + left side (1) unilateral (male + female); bilateral (2) (males); hPsP 1 (female) (divided in 3 Black, 2 White, 1 Indian); RHS = 3: CLAP 2 (White males); hPsP 1 (White female); AEC = 1: CLAP bilateral (White male). Other features of the syndromes were: skin, hand, foot, tooth, hair and nail involvement, and light sensitivity. Postoperative complications included: (i) stenosis of nasal opening, especially after reconstruction of the bilateral cleft lip and the columella lengthening (2 cases), (ii) premaxilla-prolabium fusion (2 cases), (iii) repeated occurrence of oro-nasal fistula in the hard palate (4 cases), and (iv) dysgnathial development of midfacial structures (3 cases). DISCUSSION: Three different p63 associated syndromes (EEC, AEC, and RHS) were diagnosed (0.27% of the total facial cleft deformities database). The majority of the cases presented with a bilateral CLAP in males only. A number of females and males had unilateral CLA. The hPsP-cleft was recorded in females only. The associated ectodermal component most probably had a profoundly negative influence on postoperatively wound healing, which was observed in particular at the nasal openings, the premaxilla sulcus and in the hard palate mucosa. The reconstruction of p63 associated syndromes is a greater challenge than the usual cleft reconstruction to the surgeon.

Exceptional isolated cleft of the hard palate.

We describe an extremely rare type of isolated cleft of the hard palate, which extends the published classification of the Type III cleft.

Airway and feeding problems in infants with Fairbairn-Robin triad deformities.

BACKGROUND: The majority of patients with Pierre Robin sequence in the subdivision Fairbairn- Robin triad (FRT), are born with glossoptosis, retro-/micrognathia and cleft or agenesis of the palate leading to feeding difficulties and airway obstruction. There is limited literature on these problems, and on methods used to address them. OBJECTIVES: Community nurses in the Facial Cleft Deformity Clinic evaluate associated airway obstruction and feeding problems and devise nursing interactions to address these. This retrospective study examined the incidence of airway and feeding difficulties in the neonatal, pre-surgical period, as well as the surgical and nutritional management of these infants. METHOD: Retrospective records of 215 infants with FRT were examined and data on incidence, airway and feeding difficulties and surgical and nutritional management was collected. Descriptive statistics, including average and percentage values, were compiled. RESULTS: The incidence of FRT amongst the cleft palate patients was 6.0%, with 37.7% of these having feeding difficulties. However, surgical interventions such as glossopexy (5.6%), distraction osteogenesis (0.9%) and tracheotomy (2.3%) for airway management were seldom required. Most of the infants who had upper airway obstruction and feeding problems were handled by means of suction and drinking plates, along with additional specific feeding aids. This reduced airway obstruction in 70.6%, and feeding problems in 62.4% of these infants. CONCLUSION: Based on this study's finding the introduction of the suction and drinking plate and the use of specific types of feeding devices and surgical management can improve growth and development in infants with FRT.