The Effect of Presurgical Nasoalveolar Molding on Oronasal Fistula Following Primary Palatoplasty.

OBJECTIVE: Presurgical nasoalveolar molding (PNAM) is widely used in cleft care protocol. This study investigated the correlation between PNAM and oronasal fistula after primary palatoplasty. METHODS: A case-controlled study of 80 unilateral and bilateral complete cleft palate patients who underwent cleft palate repair were enrolled. Patients were divided into 2 groups: (1) no PNAM use and (2) PNAM use. The incidence of oronasal fistula and postoperative complications were compared between groups. RESULTS: Forty patients in each group demonstrated the same baseline characteristics. The PNAM group showed a significantly lower postoperative oronasal fistula rate (15% versus 50%, P=0.003). Palatal cleft width wider than 12.5 mm increases the odds ratio of fistula formation by 1.19-fold (P=0.037), and the PNAM protected against postoperative palatal fistula formation (odds ratio 0.20, P=0.003). CONCLUSION: Presurgical nasoalveolar molding can reduce postoperative oronasal fistula in wide-gap Veau type III and IV cleft palate.

Risk Factors of Fistula Following Primary Palatoplasty.

BACKGROUND: Oronasal fistula (ONF) is a common complication following cleft palate surgery. This study aims to determine the prevalence of ONF and the factors that influence development of ONF following primary palatoplasty. MATERIALS AND METHODS: There were 234 patients undergoing primary palatoplasty during 2012 to 2016 included in this cross-sectional study. Patient demographics, surgeon's experience, age at the time of primary palatoplasty, cleft type by Veau classification, cleft width, and operative technique were recorded. The prevalence of fistulae was the primary outcome. Association of age at the time of repair, cleft type, cleft associated with syndromes, cleft width, and surgeon's experience with fistula rate were secondary outcomes. RESULTS: There were 234 consecutive patients (128 boys [54.7%] and 106 girls [45.3%]). The mean age at primary palatoplasty was 13 months. Oronasal fistulae occurred in 61 (26.07%) patients. There was a statistically significant association between postoperative oronasal fistulae and the following independent variables: Veau classification (adjusted odds ratio 2.1; 95% confidence interval [CI] = 1.45-3.1, P < 0.01), cleft associated with syndromes (adjusted odds ratio 4.76; 95% CI = 1.48-15.2, P < 0.01) and cleft width more than 11.5 mm (adjusted odds ratio 1.96; 95% CI = 1.00-3.85, P = 0.04). CONCLUSION: The overall number of fistulae was moderate in patients who had undergone primary palatoplasty in our center. Cleft severity as defined by the Veau classification, cleft width, and cleft associated with syndromes were predictive factors for development of postoperative fistulae.

Is Adjunctive Hyperbaric Oxygen Treatment Alone or with Surgery the Proper Management for Active and Persistent Osteoradionecrosis?

ABSTRACT: This study enrolled patients with stage 3 osteoradionecrosis. There were three treatment modalities: antibiotics, hyperbaric oxygen therapy (HBOT), and surgery. Complete healing was the primary outcome. Multiple logistic regression analysis demonstrated that surgery with HBOT improved wound healing better than HBOT alone.

Radiation ulcer treatment with hyperbaric oxygen therapy and haemoglobin spray: case report and literature review.

OBJECTIVE: To explore the effectiveness of a combination of hyperbaric oxygen therapy and haemoglobin spray in radiation ulcer treatment. METHOD: We reviewed the available literature and present a case report in which radiation ulcer was treated with a combination of hyperbaric oxygen therapy and haemoglobin spray. RESULTS: After 30 sessions of hyperbaric oxygen therapy (2.4 ATA; 90 minutes each session) and administration of haemoglobin spray, the wounds showed gradual progress towards healing and a good granulating base was achieved. The wounds were closed after two months using a small split thickness skin graft. CONCLUSION: A combination of hyperbaric oxygen therapy and haemoglobin spray was effective as a short course of treatment for radiation ulcers.

Adding herbal extracts to silicone gel on post-sternotomy scar: a prospective randomised double-blind study.

OBJECTIVE: Silicone gel has been shown effective in improving healing post-sternotomy scars. It remains to be determined whether adding herbal extracts to the gel would augment the healing effect. METHOD: After median sternotomy, patients were randomised into two groups. Group 1: topical silicone gel plus herbal extract gel (Allium cepa, Centella Asiatica, Aloe vera and Paper Mulberry) and Group 2: silicone gel. Patients were treated for six months. The postoperative scars were assessed at three and six months by plastic surgeons using the Vancouver Scar Scale (VSS) and the patient assessment scar scale. RESULTS: Each group comprised 23 patients (n=46 in total). The VSS was significantly lower in Group 1 than in Group 2 (p=0.018 and p=0.051, respectively). In Group 1, the four differences from baseline were vascularity scores at three and six months (-0.391, p=0.025; -0.435, p=0.013, respectively), and pigmentation scores at three and six months (-0.391, p=0.019; -0.609, p=0.000, respectively). In Group 2, differences from baseline were the pigmentation and vascularity score at six months (-0.6609, p=0.000; -0.348, p=0.046, respectively). CONCLUSION: Our results suggest, post-sternotomy scars trend to have better vascularity and pigmentation when treated with silicone gel plus herbal extracts.

Preschool Age Anthropometric Evaluation of the Nose After Bilateral Complete Cleft Lip Repair.

OBJECTIVE: To evaluate the aesthetic outcomes of surgery in preschool-aged bilateral complete cleft lip patients by comparing their nasal aesthetic parameters with those of normal children. SETTING AND SAMPLE POPULATION: Twenty-six 4 to 6-year-old (preschool age) patients with bilateral complete cleft lip who underwent primary cheiloplasty and a control group of 30 children in Northeast Thailand of the same age were enrolled. MATERIALS AND METHODS: Nasal aesthetic parameters were analyzed in terms of 3 ratios and 1 angle using three-dimensional photographs. The data from bilateral cleft lip patients were compared with those from a control group. RESULTS: The nasal parameters of patients in the cleft group were the same as those in the control group in terms of nasal tip height (NTH), columella height (CH), and dome height (DH) (P values were 0.361, 0.494, and 0.086), but nasal width (NW) was greater in the cleft group (P < 0.001). The nasal aesthetic parameters differed significantly between the 2 groups in terms of CLA (P < 0.001) and ratio of NTH and NW (P < 0.001), but not in terms of the CH:NW (P = 0.190) and DH:CH ratios (P = 0.147). CONCLUSION: This treatment protocol for bilateral cleft lip was able to achieve most of the aesthetic goals in terms of NTH, CH, DH, ratio of CH to NW, and ratio of DH to CH. However, more surgical correction of NW and CLAs may be needed.

Knee reconstruction using a distally based anterolateral thigh flap: an anatomical cadaveric study.

OBJECTIVE: To study the vascular territory of the descending branch of the lateral circumflex femoral artery and its anastomosis around the knee and to identify the cutaneous perforator supply to the distally-based or reverse flow anterolateral thigh (dbALT) flap. In particular, the distance of the first perforator branch arising from the knee and above region, as well as the number of branches and position of each perforator, beneficial for effective flap design, are also examined. METHOD: Fresh cadaveric lower limbs were dissected and the distance of the first perforator branch arising from the knee and above regions, which are vascular supplies for flaps, as well as the number of branches and position of each perforator are described. RESULTS: A total of 54 thigh cadavers had at least two cutaneous perforator branches above the knee 10cm from the superior border of the lateral patella. The mean (± standard deviation) of the first perforator distance was 2.57±0.93cm above the superior-lateral border of the patella. All perforating branches were 4±2cm, 93% were 10±2cm from the superior lateral border of patella, and 100% were at 20±3cm (mid-thigh length) which were the traditional anterolateral thigh flap. CONCLUSION: The dbALT flap technique resulted in constant and reliable references about perforator sites. Thus, this type of flap is a viable option in patients who present with soft tissue defects around the knee and upper third of the leg.

Dorsalis pedis Perforator Flap: Cadaveric Anatomical Study.

Background: The Dorsalis pedis perforator flap is a thin and pliable fasciocutaneous tissue. No previous study has demonstrated the surgical anatomy among Asians. Material and Method: Demonstrate the surgical anatomy of Dorsalis pedis perforator flap in 12 limbs from Thai cadavers. Results: We found the Dorsalis pedis perforators in all limbs and average the distance of distal perforators was 3.25+0.5 cm proximal to the metatarso-phalangeal joint. The first dorsal metatarsal artery was mainly type 1 (83.3%) while another 16.7% were type 2. Conclusion: Dorsalis pedisperforator flaps were versatile with a constant surgical anatomy and acceptable donor site morbidity.

An Anatomical Study of Vascular Supply of the Distally Based Sural Artery Flap: A Cadaveric Study.

Objective: In this study we conducted the vascular anatomy of sural flap to determine the peroneal artery perforator contribution relative to anatomical landmark of tip of lateral malleolus, include the first peroneal artery perforator to identify safely pivot point of the flap. Material and Method: A retrospective study of an anatomical study of Vascular supply of the Distally Based Sural Artery Flap was performed by dissection on 12 fresh adult cadavers legs. We recorded number of the perforator, location of each perforators in relationship to the tip of lateral malleolus and location of first peroneal artery perforator. Results: The anatomical of vascular supply of the distally based sural artery flap, anatomical of peroneal artery perforators was identified and measured from anatomical landmark is tip of lateral malleolus. The mean number of perforators was 3.4 (range, two to five), grouped in 5 perforators at the following average locations proximal to the tip of lateral malleolus: first, at 6.3+0.9 cm; second, at 8.5+1 cm; third, at 11.17+1.4 cm; fourth, at 12.7+1.2 cm; and fifth, at 14.6+0.2 cm proximal to the tip of the lateral malleolus. Conclusion: Complete vascularization of venoneurofasciocutaneous sural flap was accomplished by peroneal perforator, the blood supply of the distally based sural venoneurofasciocutaneous flap can be pivoted at the lowest perforators in the posterolateral region, which are about 5.4 to 7.2 cm proximal to the tip of lateral malleolus.

Evaluation of nasolabial esthetics in 8- to 12-year-old patients with cleft lip and palate by healthcare professionals in Northeast Thailand.

Objective: To assess the aesthetics of the nasolabial appearance of patients with cleft lip and palate aged 8-12 years by experienced and inexperienced professionals. Methods: A cross-sectional study was conducted on 32 patients with cleft lip and palate, who underwent surgery and follow-up treatment at the Cleft Center. The research tools included a parent-completed survey providing GO graphic data and two-dimensional frontal view photographs of patients from both left and right sides. The aesthetic of the lip, nose, and nasolabial region was evaluated by an experienced and inexperienced medical professional team in groups of four individuals. In addition, data analysis was performed using descriptive statistics, mean values, standard deviations, Cronbach's alpha reliability coefficient, and the Wilcoxon signed-rank test. Results: The average age of patients was 10.32 years, mainly male (65.63%), had unilateral cleft lip and palate (62.50%), and underwent bone grafting (96.88%). Regarding the assessment in three aspects, the aesthetic score assessed by experienced evaluators is fair level (2.64 ± 1.09) with an acceptable value of 0.73. In contrast, the inexperienced evaluator showed a high level (2.43 ± 0.83) with a high inter-rater reliability acceptable value of 0.60. Inexperienced evaluators showed higher scores for the lip and nasolabial region than experienced evaluators, which was statistically significant (p-values < 0.01). Conclusions: The inexperienced evaluators showed significantly higher scores for appearance than experienced evaluators. This result can improve surgical techniques in future procedures to meet the needs of patients seeking lip and nasolabial appearance corrections across different age groups.

The perspectives of learners at a public medical school on the evaluation of an online learning management system for degree and non-degree courses.

BACKGROUND: There has been a rapid development and adoption of online learning in medical education. However, it is difficult to adopt the currently available online learning management systems (LMS). This study aimed to examine learners' perspectives on the evaluation of online LMS. METHODS: An online LMS was developed based on the evidence-based guidelines. Two cross-sectional studies were conducted. A short survey was conducted with 716 learners registered on the LMS to obtain their perspectives on the online participation. A satisfaction survey was conducted with 255 learners enrolled in the courses taught solely online. Data from the LMS monitoring system was used to report the uptake of online courses. Data were analyzed using descriptive statistics. RESULTS: Participants reported that the major factor influencing LMS uptake was the ability to be accessed anytime and anywhere (n = 556, 77.7%). The participants had good experience in using the LMS and were satisfied with it (n = 255, mean = 4.53, SD = 0.62). For online degree courses, the course had a high completion rate of 90% provided that a mark was assigned for course attendance. For non-degree courses, irrespective of whether they were free, paid, exam-based, or participation only, the completion rate was considered low (range 4.3-36.7%). CONCLUSION: Under a limited budget, a medical school in a low- to middle-income country could develop an effective online LMS to meet learners' needs. Our newly developed online LMS is relevant, accepted and to the satisfaction of the learners. Medical schools in the same context are encouraged to develop their own online LMS that serve and support learning in both degree and non-degree courses.

En bloc groin node resection reconstructed with external oblique flap for solitary metastatic cholangiocarcinoma: a case report.

Cholangiocarcinoma requires complete surgical resection for cure. Even so, the recurrence and metastasis rates are high, and further treatment is typically through palliative systemic chemotherapy. Curative-intent resection of metastatic site may provide survival benefit in selected cases. However, there were no previous reports of groin node dissection in cholangiocarcinoma. We have reported the first case of intrahepatic mass-forming cholangiocarcinoma with isolated synchronous groin node metastasis, successfully treated with   resection of the liver mass followed by groin node resection, reconstructed with musculofascial flap. A 73-year-old man presented with right upper quadrant abdominal pain radiating to the right groin for two months. Magnetic resonance cholangiopancreatography revealed a 3.1 × 1.2 cm enhancing mass between hepatic segment 4 and the anterior peritoneum, invading the abdominal wall. Computed tomography of the abdomen revealed a 2.4 × 2.2 cm focal enhancing mass at the anterior aspect of the right lower abdominal wall, just anterior to the right inguinal ligament and iliac vessel. He underwent en bloc resection of hepatic segment 4, gallbladder, and anterior abdominal wall, and the histology result is cholangiocarcinoma. After systemic chemotherapy, he underwent en bloc resection of the right groin mass, reconstructed with external oblique musculofascial flap. The patient was able to achieve a 20-month recurrence free survival after the final operation. This case has demonstrated that in a carefully selected case, resection of distant metastasis cholangiocarcinoma can provide survival benefits, even in the rare site of metastasis.

The use of nanocrystalline silver for the treatment of massive soft tissue defects with exposed bone.

BACKGROUND: In a complex wound with exposed tendon, joint or bone caused by trauma, soft tissue reconstruction is required to obtain function and aesthetic coverage. Surgical reconstruction can be performed using a variety of different techniques. OBJECTIVE: The authors report their experience using a nanocrystalline silver with or without vacuum assisted wound closure to salvage the exposed vital structures such as bone, joint or tendon in traumatic wounds. MATERIAL AND METHOD: The present study included 12 patients with 15 wounds that exposed vital structures especially in the lower extremities. After adequate debridement, four wounds were treated with nanocrystalline silver dressing and vacuum assisted wound closure, and ten wounds were treated in an out-patient program using nanocrystalline silver dressing. RESULTS: A preliminary evidence of impressive result was achieved with subsided infection and complete bony coverage by granulation tissue after treatment. Subsequently, a secondary healing or a split thickness skin graft was applied. CONCLUSION: The authors present the satisfactory results of alternative techniques that may be used for the massive soft tissue defects with exposed bone.

Comparison of wound dehiscence and parent's satisfaction between spoon/syringe feeding and breast/bottle feeding in patients with cleft lip repair.

BACKGROUND: Cleft lip and cleft palate are the most common craniofacial anomalies affecting approximately 2.49 of every 1,000 children born in North-east of Thailand. Srinagarind Hospital has 100-150 cases of cleft lip each year. Children with cleft lip and palate need surgical procedures as soon as possible. After lip repair the normal recommendation is not using bottle or breast feeding for 2 weeks to avoid tension at the sutured area during sucking and possible cause of wound dehiscence. So this is quite complicated for the parents, and patients feel frustrated, cry, and move their head around, because of hunger which cannot easily be satisfied. Previous research found that sucking does not cause wound dehiscence, but mentioned no detail about severity of cleft. OBJECTIVE: Primary objective is to compare surgical wound dehiscence between breast feeding/bottle and spoon/syringe feeding after lip repair. MATERIAL AND METHOD: This is an experimental study: non-inferiority trials study. The population is the patients with cleft lip who underwent lip repair in Inpatient Department 3C, Srinagarind Hospital, Faculty of Medicine, Khon Kaen University. The study period is during May 2010-February 2013. The total sample size in the present study is 192 participants, 96 cases breast/bottle feeding, 96 cases spoon/syringe feeding. The wound dehiscence rate was analyzed by Z-test. Parents'satisfaction is a qualitative data and was analyzed through content analysis. RESULTS: No statistical significant diference between breast/bottle and spoon/syringe groups (p-value = 0.320, 95% confidence interval -0.031-0.010). Parents were more satisfied to feed children by breast/bottle and patients were more relaxed with breast/bottle feeding. CONCLUSION: Breast/bottle feeding and syringe/spoon feeding have the same result on the surgical wound. Breast/bottle feeding are not causes of wound dehiscence.

Role of silicone derivative plus onion extract gel in presternal hypertrophic scar protection: a prospective randomized, double blinded, controlled trial.

Use of silicone derivative and onion extract had been reported in the prevention of hypertrophic scarring. Our experience showed the preventive use of silicone derivative plus onion extract gel on hypertrophic scars after median sternotomy. In a randomized, double blinded, placebo-controlled study, 60 patients after median sternotomy incisions were separated into two groups. All patients were treated either with silicone derivative plus onion extract gel (Cybele(®) scagel) or placebo gel twice daily for a total treatment period of 12 weeks. During each visit, pain and itching scores were graded by the patients and scar characteristics were observed by surgeons using the Vancouver scar scale. Pain and itch score values from patients' who applied silicone derivative plus onion extract gel was less than another group (P < 0·05). Pigmentation was significantly different between two groups (P < 0·05) and the reduction of scores on vascularity, pliability, height in treated group was not superior to the untreated group. No adverse events were reported by any of the patients. A silicone derivative plus onion extract gel is safe and effective for the preventing the hypertrophic scarring after median sternotomy.

Knowledge and satisfaction of caregivers of patients with cleft lip-palate at the Tawanchai Cleft Center.

The Northeast region has the highest rate of cleft lip-palate (CLP) deformities in Thailand. This born handicapped condition leads to complicated problems in many ways. The best treatment is managing the system to have good coordination by an interdisciplinary team that rehabilitate patients' physical and mental conditions so they can recover as well as possible. This study aimed to determine the (1) knowledge of taking care of patients and (2) service satisfaction of caregivers resulting from the development of Tawanchai Cleft Center's nursing care system as a 1-stop coordinating service in 2010. The study included 106 caregivers. We used a questionnaire to assess the knowledge level and the service satisfaction. Most of the patients had left unilateral complete cleft lip and palate (41%). The mother was the caregiver most of them time (68%); 43% had completed primary education, and 40% worked in agriculture. The average knowledge level among caregivers for the specialized care of patients with CLP was a good level ((Equation is included in full-text article.)= 3.71, SD = 0.81). The average knowledge level concerning the cause, the treatment guideline, and treatment by a multidisciplinary team of patients with CLP was a moderate level ((Equation is included in full-text article.)= 3.28, SD = 0.85). The satisfaction of caregivers regarding service at the In- and Out-patient Surgery Department was very good for all items ((Equation is included in full-text article.)= 3.54, SD = 0.54). The one-stop coordination service developed for caregivers neatly fit the needs of CLP patients and resulted in a moderate to good level of knowledge and a very good level of satisfaction regarding the specialized care needed.

Predictive MERRA-2 aerosol diagnostic model for oral, oropharyngeal and laryngeal cancer caused by air pollution in Thai population.

Introduction: Head and neck cancers were generally characterized with many possible causes. Exposure to outdoor particulate matter affected to multiple organ systems but it was unknown whether which species in PM was an association with cancer incidence. Objectives: The study aimed to examine the oral- oropharyngeal- laryngeal cancer incidence and accumulated air pollution-related cancers in the spatial patterns. Methods: Observational study was conducted, and the Poisson log-linear models were used which were analyzed on subgroups-specific incidence rates by national references of Thailand and NASA's database of aerosol diagnostics model (MERRA-2). Results: With a significant influence on increasing of 1 µg/m3 black carbon, organic carbon, Dust-PM2.5, and SO4 were associated with increased cancer risk in 1.433 times (95%CI: 1.215-1.690), 1.272 times (95%CI: 1.139-1.420), 3.640 times (95%CI: 2.011-6.589), and 1.704 times (95%CI: 1.334-2.177), respectively. Conclusion: This study indicated that oral-oropharyngeal-laryngeal cancer incidence could worsen because of adverse air pollution conditions. These issues should be addressed and the importance of the monitoring procedure for dust-PM2.5, sulfate, black carbon, and organic carbon should be emphasized.

Buccal Fat Pad: Adjunctive Procedure for Lateral Defect Coverage following Primary Palatoplasty.

BACKGROUND: The buccal fat flap is an encapsulated mass originating from a specific fat tissue that is easily accessed and richly vascularized. The aim of this study was to report the effect of using the buccal fat flap on the oronasal fistula rate in primary palatoplasty. METHODS: A case-controlled study of 94 patients who underwent primary cleft palate repair. Patients were divided into two groups: (1) two-flap palatoplasty with buccal fat flap for coverage of lateral hard palate defect and (2) conventional two-flap palatoplasty. The incidence of oronasal fistula and postoperative complications were compared between groups. Multivariate analysis was performed to determine the risk factors of oronasal fistula development. RESULT: Forty-seven patients in each group demonstrated the same baseline characteristics. The buccal fat group showed a significant lower postoperative oronasal fistula rate (2.13 percent versus 21.28 percent, p = 0.008) and smaller fistula size (2 mm versus 4 mm, p = 0.049). A cleft width wider than 11.5 mm increased the odds ratio of fistula formation by 8.44-fold (p = 0.047), and the use of buccal fat protected against postoperative palatal fistula formation (OR, 0.08, p = 0.019). CONCLUSION: The use of buccal fat flaps for lateral hard palatal defect coverage in primary palatoplasty can reduce the rate of postoperative palatal fistula, especially in cases of wide palatal cleft. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Challenges in evaluation, management and outcome of the patients with Treacher Collins Syndrome.

BACKGROUND: The challenges for management of Treacher Collins Syndrome (TCS) are the analysis of deformities and staged soft tissue and bony reconstruction. OBJECTIVE: To present clinical and radiologic findings, the evaluation methods and the concept of staged reconstruction for patients with TCS. MATERIAL AND METHOD: The authors reviewed the clinical and radiologic findings, the evaluation methods, the staged reconstruction and early surgical outcome of three patients with TCS treated at Srinagarind Hospital, between 1994 and RESULTS: One patient underwent evaluation by CT scan for planning, reconstruction of the zygoma and orbit, correction of the lower eyelid coloboma, bilateral staged ear reconstruction with costal cartilage. At the age of 8 years, he had acceptable initial outcome with planned middle ear management and staged facial reconstruction. Palatoplasties of a cleft palate were performed in two patients, CONCLUSION: TCS is a rare craniofacial deformity but poses challenges in evaluation, management and reconstructive surgery. The development of Craniofacial Center and interdisciplinary management is important for provision proper evaluation, initial management and longitudinal care, including appropriate timing for staged reconstruction of bone, ear and soft tissue reconstruction in patients with TCS. This treatment should be tailored and balanced with the functional and psychological needs of patient and his/her family. In developing countries, funding from other resources, including the not-for-profit foundations, is needed in order to increase patients' accessibility, and improve the quality of the entire treatment program.

Pierre Robin sequence: challenges in the evaluation, management and the role of early distraction osteogenesis.

BACKGROUND: The challenges for the management of Pierre Robin Sequence (PRS) are the evaluation and management of airway and feeding difficulty from glossoptosis and associated cleft palate. OBJECTIVE: To present the clinical findings, management, outcome and the role of early distraction osteogenesis in patients with PRS. MATERIAL AND METHOD: The medical records were reviewed of patients with PRS seen and managed by the authors at Srinagarind Hospital, Khon Kaen University between 2001 and 2011. RESULTS: Fifteen patients with PRS were seen and managed. The female-to male ratio was 1.8 to 1 (9 girls, 5 boys). All of the patients presented with a small mandible, retrodisplaced tongue and upper airway difficulty. One patient had cleft lip only and one patient had cleft lip with cleft palate. Patients were primarily from the provinces of Khon Kaen and Mahasarakham. Conservative management was successful in 12 patients while the 3 with tracheostomy required distraction osteogenesis and the tracheostomy was subsequently successfully decanualated. At the last follow-up, most of the patients had proper catch-up and mandibular growth. CONCLUSION: Primary management of airway insufficiency in patients with PRS can be managed in a prone position with or without nasopharyngeal airway, prolonged intubation, tongue-lip adhesion, mandibular distraction osteogenesis and tracheostomy. The present study confirmed that proper conservative management can be used to manage most of the patients with PRS. However, early mandibular distraction should be considered when (a) indicated in patients with respiratory insufficiency to avoid tracheostomies or (b) successfully decannulating tracheostomies. Interdisciplinary team management is needed to ensure proper evaluation, improve care and optimum outcome.