Spectrum of Disease in Hospitalized Newborns with Congenital Micrognathia: A Cohort of 3,236 Infants at North American Tertiary-Care Intensive Care Units.

OBJECTIVE: To describe the spectrum of disease and burden of care in infants with congenital micrognathia from a multicenter cohort hospitalized at tertiary care centers. STUDY DESIGN: The Children's Hospitals Neonatal Database was queried from 2010 through 2020 for infants diagnosed with micrognathia. Demographics, presence of genetic syndromes, and cleft status were summarized. Outcomes included death, length of hospitalization, neonatal surgery, and feeding and respiratory support at discharge. RESULTS: Analysis included 3,236 infants with congenital micrognathia. Cleft palate was identified in 1266 (39.1%). A genetic syndrome associated with micrognathia was diagnosed during the neonatal hospitalization in 256 (7.9%). Median (IQR) length of hospitalization was 35 (16, 63) days. Death during the hospitalization (n = 228, 6.8%) was associated with absence of cleft palate (4.4%, P < .001) and maternal Black race (11.6%, P < .001). During the neonatal hospitalization, 1289 (39.7%) underwent surgery to correct airway obstruction and 1059 (32.7%) underwent gastrostomy tube placement. At the time of discharge, 1035 (40.3%) were exclusively feeding orally. There was significant variability between centers related to length of stay and presence of a feeding tube at discharge (P < .001 for both). CONCLUSIONS: Infants hospitalized with congenital micrognathia have a significant burden of disease, commonly receive surgical intervention, and most often require tube feedings at hospital discharge. We identified disparities based on race and among centers. Development of evidence-based guidelines could improve neonatal care.

Advancements in airway stents: a comprehensive update.

PURPOSE OF REVIEW: This review provides an overview of the evolving field of airway stenting (AS), highlighting its relevance in the management of central airway obstruction (CAO). It discusses recent advancements, including 3D-printed silicone stents (3DPSS), metallic stents, biodegradable stents (BS), and drug-eluting stents (DES), which are transforming clinical practice. The review underscores the ongoing challenges in patient selection, stent choice, and long-term management in the context of an evolving landscape. RECENT FINDINGS: Innovations, particularly 3DPSS, have shown promise in providing patient-specific solutions. These stents offer improved symptom relief, enhanced quality of life, and lower complication rates, especially for complex airway diseases. The use of BS and DES is explored, raising prospects for future applications. SUMMARY: The evolution of AS reflects a deepening understanding of airway obstructions. Recent innovations, such as 3DPSS, BS, and DES, show considerable promise in addressing the limitations of conventional stents. However, challenges related to complications, patient selection, and long-term management persist, demanding further research. Wide practice variations in the management of AS highlight the need for more clinical data and standardized guidelines. The search for the ideal stent continues, driven by the pursuit of better outcomes for patients with CAO.

Anesthetic considerations in interventional pulmonology.

PURPOSE OF REVIEW: In this review, we highlight the important anesthetic consideration that relate to interventional bronchoscopic procedures for the management of central airway obstruction due to anterior mediastinal masses, endoluminal endobronchial obstruction, peripheral bronchoscopy for diagnosis and treatment of lung nodules, bronchoscopic lung volume reduction and medical pleuroscopy for diagnosis and management of pleural diseases. RECENT FINDINGS: The advent of the field of Interventional Pulmonology has allowed for minimally invasive options for patients with a wide range of lung diseases which at times have replaced more invasive surgical procedures. Ongoing research has shed light on advancement in anesthetic techniques and management strategies that have increased the safety during peri-operative management during these complex procedures. Current evidence focusing on the anesthetic techniques is presented here. SUMMARY: The field of Interventional Pulmonology requires a tailored anesthetic approach. Recent advancements and ongoing research have focused on expanding the partnership between the anesthesiologist and interventional pulmonologists which has led to improved outcomes for patients undergoing these procedures.

Fetal diagnosis and management of pulmonary artery sling: A case series.

OBJECTIVE: Pulmonary artery sling is a rare congenital anomaly accounting for 2% of all patients with vascular anomalies that cause airway obstruction. In the normal heart, the left (LPA) and right (RPA) pulmonary arteries arise in the intrapericardial space. However, in the pulmonary artery sling, the LPA trunk arises in the extrapericardial space from the posterior aspect of the mid RPA and courses posterior to the trachea causing tracheal compression and, at times, bronchial compression. While a full spectrum of congenital cardiac pathology can be identified before birth, only a few case reports document the prenatal diagnosis of an Left pulmonary artery sling (LPAS). METHOD: We retrospectively identified all cases of prenatal LPAS from three Canadian fetal cardiology centers (2015-2022). RESULTS: Using the 3-vessel-tracheal view via fetal echocardiography (FE), four fetuses from three pregnancies demonstrated abnormal origin of the LPA from RPA and echogenic trachea. In one of two affected monochorionic twins coronal imaging demonstrated a significant narrowing of the large airways consistent with significant airway obstruction. CONCLUSION: Prenatal detection of LPAS by FE is possible and should prompt an evaluation for airway obstruction in the coronal view. Investigating associated lesions and genetic testing are recommended for informed shared decision making.

Tracheobronchial stents: an expanding prospect.

The first dedicated tracheobronchial silicone stent was designed by the French pulmonologist Jean-Paul Dumon. The most common indications for stenting are to minimise extrinsic airway compression from mass effect, maintain airway patency due to intrinsic obstruction or treat significant nonmalignant airway narrowing or fistulae. Silicone stents require rigid bronchoscopy for insertion; however, they are more readily repositioned and removed compared with metallic stents. Metallic stents demonstrate luminal narrowing when loads are applied to their ends, therefore stents should either be reinforced at the ends or exceed the area of stenosis by a minimum of 5 mm. Nitinol, a nickel-titanium metal alloy, is currently the preferred material used for airway stents. Airway stenting provides effective palliation for patients with severe symptomatic obstruction. Drug-eluting and three-dimensional printing of airway stents present promising solutions to the challenges of the physical and anatomical constraints of the tracheobronchial tree. Biodegradable stents could also be a solution for the treatment of nonmalignant airway obstruction.

Predictors in the treatment of malignant central airway obstruction with silicone stents.

PURPOSE: To examine the role of the silicone stent in palliation of malignant central airway obstruction and identify potential preprocedural predictors for postprocedural outcome. METHODS: Patients treated with endoscopic insertion of tracheobronchial silicone stents for malignant central airway obstruction at Aarhus University Hospital from 2012 to 2022 were identified from electronic medical records. Statistical analyses were carried out to identify factors affecting Days Alive and Out of Hospital, complications and overall survival. RESULTS: 81 patients underwent a total of 90 tracheobronchial stent insertions. Days Alive and Out of Hospital (DAOH) for the first 30 days were affected negatively by urgent intervention, p < 0.001, preprocedural non-invasive respiratory support, p < 0.001, and preprocedural intubation, p = 0.02. Post-procedural oncological treatment was associated with a significant improved DAOH, p = 0.04. Symptomatology and lesion characteristics were not significantly associated with any impact on DAOH. Overall survival was poor (mean survival was 158 days), and only significantly affected by severe degree of dyspnea, p = 0.02, and postprocedural oncological treatment, p < 0.001. Complication where registered in 25.6% of cases within the first 30 days was observed. Procedure-related mortality was 3.7%. Based on chart annotations by an ENT-surgeon, 95% of the patients experienced relief of symptoms following stent insertion. CONCLUSIONS: Palliative tracheobronchial airway stenting with silicone stents is found to have a beneficial impact, more research is required for identification of predictors for postprocedural outcome based on preprocedural classifications.

Mandibular Distraction in Patients With Pierre Robin Sequence: A Multisurgeon Experience.

OBJECTIVE: Mandibular distraction osteogenesis (MDO) is rapidly becoming a standard of care for management of patients with severe Pierre Robin sequence. The tongue is brought forward to alleviate airway obstruction. This study will look at an institutional, multisurgeon experience with MDO over 10 years. DESIGN: A retrospective chart review was conducted. SETTING: All patients who underwent MDO at the authors' institution from 2012 to 2022 were included. Three craniofacial surgeons performed all interventions. PATIENTS: Demographics, preoperative and postoperative respiratory and feeding status, and distraction data were collected for 27 patients meeting inclusion criteria. MAIN OUTCOME MEASURES: Primary outcomes were avoidance of a gastrostomy tube, avoidance of a tracheostomy, discharge from hospital on room air, and complications. A significance value of 0.05 was utilized. RESULTS: The average age at MDO was 135 days, mean activation phase was 13.6 days, mean distraction length was 14.9 mm, and mean consolidation phase was 64.2 days. A longer activation phase was associated with discharge with a gastrostomy tube and a shorter activation phase was associated with discharge on full oral feeds. The ability to discharge on room air was associated with a shorter latency phase, shorter activation phase, and decreased distance of distraction. CONCLUSIONS: The goal of MDO is to achieve full oral feeds with no respiratory support. Several different latency periods were used in this study, and a short latency period was demonstrated to be safe.

Jaw Thrust: A Simple Predictor of Success in Mandibular Distraction Osteogenesis.

BACKGROUND: The hypoplastic mandible in the congenital condition Pierre Robin sequence (PRS) displaces the base of the tongue posteriorly, which results in upper airway obstruction (UAO) that can potentially be corrected with mandibular distraction osteogenesis (MDO). Jaw thrust (JT) is routinely performed during evaluation of the airway; similar to MDO, it projects the mandible and tongue anteriorly to open the airway. The authors demonstrate that JT can be used as a criterion to predict successful MDO outcomes in infants with PRS. METHODS: The study was a single-center, retrospective chart review of infants diagnosed with PRS between 2016 and 2023. Data regarding their demographics, comorbid diagnoses, JT success, airway anomalies, laryngeal grade of view, apnea-hypopnea index, and perioperative course were statistically analyzed. RESULTS: Of the 16 patients included in the study, 11 had successful relief of their airway obstruction with JT and proceeded with MDO. The unsuccessful JT group had significantly greater proportions of females, birth prematurity, gastrostomies, tracheostomies, and longer hospital stays. In the successful JT group, both the mean laryngeal grade of view ( P =0.029) and mean apnea-hypopnea index ( P =0.025) improved significantly post-MDO. Post-MDO tracheostomy was also avoided in all but 1 patient who was not previously tracheostomized. CONCLUSIONS: There is no widely accepted algorithm to guide craniofacial surgeons on the optimal intervention for relieving UAO in infants with PRS. In our institutional experience, patients whose preoperative JT relieved UAO also successfully relieved UAO with MDO. In patients with PRS, JT may be a useful criterion for selecting appropriate candidates for MDO.

Successful Postnatal Tracheobronchoplasty for Unilateral Congenital High Airway Obstruction Syndrome due to Mainstem Bronchial Atresia.

INTRODUCTION: Unilateral congenital high airway obstruction syndrome (CHAOS) is caused by a complete obstruction of a mainstem bronchus with resulting hyperinflation and accelerated growth of one lung, severe mediastinal shift, and hydrops. Spontaneous perforation of the atresia has been observed in CHAOS which allows hydrops to resolve but hyperinflation, mediastinal shift and a critical airway obstruction persists as the perforation is usually pinhole-sized. CASE PRESENTATION: We present a case of unilateral CHAOS presenting at 26 2/7 weeks with observed-to-expected total lung volume (O/E TLV) of 203% with spontaneous perforation occurring at 28 weeks with resolution of hydrops but persistence of hyperinflation and mediastinal shift with an O/E TLV of 60.5% on 34 5/7 weeks' magnetic resonance imaging (MRI), successfully managed in a 35 5/7 weeks, 1,670 gm, growth restricted baby, by venoarterial extracorporeal membrane oxygenation (VA ECMO) and resection of the tracheobronchial atresia and tracheobronchoplasty on day of life 5. The baby was separated from ECMO on post-op day 12, required tracheostomy for positive end expiratory pressure for tracheomalacia at 4 months. CONCLUSION: At 2 years of age, she has met all developmental milestones, has been weaned to room air tracheostomy collar, and has been anticipating tracheal decannulation. There is persistent bronchiectasis in the hyperinflated right lung but no malacia. This is the first reported survivor of mainstem bronchial atresia suggesting the importance of preservation of the hyperplastic lung and airway reconstruction to normal long-term outcome.

Surgical management of brachycephalic obstructive airway syndrome: An update on options and outcomes.

Dogs with a brachycephalic conformation often experience a collection of abnormalities related to their craniofacial conformation, which can lead to a variety of clinical signs such as stertor, exercise intolerance, respiratory distress, and gastrointestinal signs such as regurgitation, among others. This collection of abnormalities is termed brachycephalic obstructive airway syndrome (BOAS). With the rise in popularity of several brachycephalic breeds, veterinarians and veterinary surgery specialists are seeing these dogs with increasing frequency for surgical and medical treatment of these clinical signs, leading to an increased interest in developing surgical techniques for dogs with BOAS and evaluating objective methods of determining outcome after surgery. Advances in anesthetic management including standardized protocols and use of local nerve blocks to decrease opiate use may decrease postoperative complications. A variety of new or modified surgical techniques to manage hyperplastic soft palate and stenotic nares, among other BOAS components, have been developed and studied in recent years. Newer studies have also focused on risk factors for development of major complications in the postoperative period and on objective measurements that may help determine which patients will receive the most benefit from BOAS surgery. In this review, the newest studies focused on updates in anesthetic management, surgical techniques, and postoperative care will be discussed. Additionally, updated information on complication rates and outcomes for dogs undergoing surgical management of BOAS will be included.

Comparison of mortality of brachycephalic dogs undergoing partial staphylectomy using conventional incisional, carbon dioxide laser, or bipolar vessel sealing device.

OBJECTIVE: To compare mortality of dogs undergoing partial staphylectomy using conventional incisional, carbon dioxide (CO2 ) laser, and bipolar vessel sealing device (BVSD) techniques for the treatment of brachycephalic obstructive airway syndrome (BOAS). STUDY DESIGN: Retrospective multicenter cohort study. ANIMALS: A total of 606 client-owned English bulldogs, French bulldogs, and pugs. METHODS: Medical records from 2011 to 2021 were reviewed for signalment, history, surgical technique, length of hospitalization, and complications. Multivariate statistical analysis was performed to compare odds of mortality between the three techniques of staphylectomy. RESULTS: The overall mortality rate was 24/606 (4.0%). Of those 24 dogs, staphylectomy was performed with BVSD technique in 13 cases, with CO2 laser in nine, and using conventional incisional technique in two. Nine dogs were graded II or III laryngeal collapse, 14 were graded I, and one was unknown. BVSD technique was associated with mortality prior to discharge compared to the other two techniques (OR = 6.0, 95% CI: 1.3-28.4, p = .023). No differences were detected between conventional incisional and CO2 laser techniques. Concurrent higher grade (stage II or III) laryngeal collapse was independently associated with mortality prior to discharge (OR = 4.6, 95% CI: 1.8-11.8, p = .002). CONCLUSION: The use of BVSD and grade of laryngeal collapse were associated with a higher risk of perioperative mortality. CLINICAL SIGNIFICANCE: Clinical studies using a randomized trial design should be conducted to further determine the putative influence of surgical instrumentation in the perioperative mortality rate following multilevel surgery in dogs with BOAS.

Evaluation of the addition of adrenaline in a bilateral maxillary nerve block to reduce hemorrhage in dogs undergoing sharp staphylectomy for brachycephalic obstructive airway syndrome. A prospective, randomized study.

OBJECTIVE: To assess the use of adrenaline (0.00198%) in a bilateral maxillary nerve block to reduce intraoperative hemorrhage in dogs undergoing staphylectomy for the treatment of brachycephalic obstructive airway syndrome (BOAS). STUDY DESIGN: Prospective, randomized, double-blinded controlled study. SAMPLE POPULATION: A total of 32 client owned, clinically affected dogs undergoing a cut and sew sharp staphylectomy for treatment of BOAS. A total of 16 dogs randomly assigned to adrenaline (A) group and 16 to no-adrenaline (NA) group. METHODS: A bilateral maxillary nerve block was performed in all dogs prior to staphylectomy using lidocaine alone (group NA) or a combination of lidocaine and adrenaline (group A). Total hemorrhage was measured by weighing cotton-tipped applicators and swabs used on precision scales. In addition, a semi-quantitative hemorrhage score (1-5) was determined. Dogs were monitored for intraoperative hemodynamic stability during the procedure. RESULTS: Total hemorrhage was significantly (p = .013) lower in group A compared with group NA. The median semi-quantitative hemorrhage score was significantly lower for group A (p = .029) compared with group NA. No significant adverse effects were noted due to adrenaline usage. CONCLUSION: This study demonstrated that the use of adrenaline in a bilateral maxillary nerve block results in significantly lower intraoperative hemorrhage during cut and sew sharp staphylectomy and appeared safe to use. CLINICAL SIGNIFICANCE: Potential clinical advantages include improved visualization, prevention of unnecessary blood loss and reduction in risk of blood aspiration.

Fractured Metallic Tracheostomy Tube: A Rare Presentation of Bronchial Foreign Body.

BACKGROUND: Tracheostomy is an important life-saving surgical procedure that could be used to secure the lower airway. It can however serve as a source of airway compromise when fragments from it are deposited. This condition must be diagnosed early enough, and appropriate intervention should be done to forestall potential morbidity and mortality. CASE REPORT: A 56-year-old tracheostomized patient presented to the accident and emergency department with a 4-hour history of sudden onset choking cough and labored breathing. The shaft of his tracheostomy tube (TT) was fractured and subsequently aspirated while he was attempting to remove and clean the inner tube that morning. He has used the TT for about 6 years and lost to follow-up clinic visits. The chest radiograph showed the metallic foreign body lodged within the trachea. He had an emergency rigid bronchoscopy via the tracheostomy stoma, and the object was retrieved. All respiratory symptoms subsequently resolved, and a check radiograph showed normal findings. CONCLUSION: Tracheostomy tube fracture and aspiration should be ruled out in every tracheostomized patient with sudden onset acute respiratory symptoms. Otolaryngologists must always emphasize the need for proper handling of TT, regular follow-up, and tube replacement when due.

Acute airway obstruction requiring nasotracheal intubation following hypoglossal neuromonitoring: a case report.

BACKGROUND: Intraoperative neurophysiological monitoring (IONM) is utilized for both the localization of critical structures and for real time detection and prevention of intraoperative neurological injury. Use of IONM to monitor the hypoglossal nerve is performed during neurosurgical, otolaryngological, and vascular procedures to improve surgical outcomes. There is a paucity of literature describing potential complications of IONM of the hypoglossal nerve, especially with respect to airway compromise. Here we present our findings regarding a case of acute airway obstruction following hypoglossal nerve monitoring. CASE PRESENTATION: A 54-year-old male was admitted for left far-lateral craniotomy and microsurgical clipping of a left posterior inferior cerebellar artery (PICA) aneurysm. Following induction and intubation but prior to the procedure start, the patient was placed in the ¾ prone position with the left side up and his neck was flexed approximately 10 degrees. He then underwent placement of subdermal needle electrodes into the facial muscles, trapezius muscles, soft palate, and tongue for IONM. The procedure lasted 523 minutes and was completed without complication. However, approximately one hour after emergence from general anesthesia, the patient experienced progressive difficulty breathing secondary to severe lingual swelling. He required emergent placement of a nasotracheal tube guided by a fiberoptic bronchoscope. He remained intubated for 3 days and was treated with dexamethasone, after which the swelling resolved, and the patient was successfully extubated. CONCLUSIONS: Acute lingual edema is a potentially life-threatening phenomenon that can lead to rapid airway compromise. Generally, causes of acute lingual swelling include hemorrhage, edema, infarction, and infection. In the case described above, we suspect traumatic injury to the tongue's vascular supply caused a deep tissue hematoma leading to postoperative acute lingual swelling and airway obstruction. With the widespread use of IONM, it becomes essential for providers to be aware that perioperative airway compromise is a potentially life-threatening complication, especially with respect to monitoring of the hypoglossal nerve. Awake fiberoptic nasotracheal intubation can successfully be employed to establish an emergency airway in such situations.

Is a Difficult Airway Team Needed for Intubation at Removal of Mandibular Distraction Devices for Infants With Robin Sequence?

PURPOSE: Difficult airway teams (DATs) are typically present to assist intubation at the initial mandibular distraction osteogenesis (MDO) operation for infants with Robin sequence (RS). In some institutions, the RS diagnosis triggers a "difficult airway" label for the infant, requiring DAT presence for future operations. By the time of distractor removal, however, breathing and airway anatomy are significantly improved. The objective of this study was to measure intubation difficulty and perioperative respiratory complications at MDO device removal as a proxy for the necessity for coordination with a DAT. METHODS: This is a retrospective study including infants with RS from 2013 to 2021 who had MDO during infancy. Patients were excluded if they had a tracheostomy or MDO device failure. Predictor variables included demographic data, comorbidities, and apnea-hypopnea indices (AHIs) from pre- and immediate post-MDO polysomnograms. The primary outcome measures were number of intubation attempts, laryngoscopy grade, and perioperative respiratory events at the distractor removal operation. Descriptive statistics were computed including Fisher's exact, paired sample t-tests, and Wilcoxon rank tests, and P < .05 was considered statistically significant. RESULTS: The sample included 47 (60% male) patients with a mean age at MDO of 12.0 ± 15.7 weeks. Significant improvement in AHI was seen after MDO (pre-MDO: 26.8 ± 18.4 events/hour; post-MDO 2.78 ± 2.66 events/hour; P < .001). Average number of intubation attempts decreased from 2.09 ± 1.36 to 1.30 ± 0.75 (P < .001) and the most common post-MDO laryngoscopy grade was 1 (69%). There were no intraoperative and 2 (4%) minor postoperative respiratory events, both in patients with repaired congenital cardiac disease and not related to traumatic intubation. CONCLUSION: Neither difficult intubations nor perioperative respiratory events associated with intubation trauma were seen at distractor removal, suggesting that specialty airway assistance is not routinely needed after successful MDO. DAT presence should be determined on a case-by-case basis based on specific patient risk factors.

When bronchoscopy does not suffice for airway foreign body removal: a series of cases requiring tracheotomy.

PURPOSE: To illustrate the importance of tracheotomy in difficult cases of foreign body inhalation and to enumerate the indications of the same. METHODS: A retrospective analysis of 5 cases in which the standard rigid bronchoscopic approach had to be combined with the open surgical approach (tracheotomy) for the removal of the inhaled foreign body for different indications. RESULTS: Combining the two procedures lead to successful removal of foreign body and restoration of airway in all the cases. Tracheostomies whenever performed were temporary. CONCLUSIONS: Foreign body aspiration is an otorhinolaryngologic emergency that can rapidly prove fatal if not well-handled. Though most cases can be dealt with by traditional approaches, a proportion of the cases may prove challenging and need a quick tailoring of response according to the situation at hand. An approach combining tracheotomy with the traditional bronchoscopic approach is one such way and in well-selected cases, can have a significant impact on the outcome; sometimes even in terms of life and death.

Vascular Anatomy for the Prevention of Sublingual Hematomas: Life-Threatening Complication of Genioplasty.

After genioplasty, the occurrence of bleeding is rare; however, rapid enlargement or displacement of the tongue secondary to lingual or sublingual hematoma can lead to life-threatening airway obstruction, necessitating prompt recognition, and immediate management. Therefore, the investigators aimed to evaluate the underlying etiologies of sublingual hematoma and relevant anatomy to facilitate early recognition of the initial presentation of these hematomas and appropriate management. The authors conducted a literature review on cases of delayed sublingual hematoma after genioplasty. The authors also report a case of delayed hematoma after performing genioplasty. The anatomical structures involved with the development of rare and serious hematomas therein are the sublingual and submental arteries, which are located in the sublingual area, rendering them susceptible to injury during genioplasty. The results of this study suggest that submental artery ligation should be performed for proper management of airway obstruction, if symptoms of sublingual bleeding are observed during the surgical procedure. If there is continuous bleeding despite the submental artery ligation, sublingual artery ligation should be performed.

The Role of Airway Management on Feeding Difficulties in Children With Pfeiffer Syndrome.

BACKGROUND: Pfeiffer syndrome is characterized by craniosynostosis, mid-face hypoplasia, broad thumbs, and often multilevel airway obstruction. Airway management is often required, including the use of positive airway ventilation, nasopharyngeal airway (NPA), or tracheostomy. OBJECTIVE: The objective of this study was to assess the impact an airway adjunct can have on feeding difficulties in children with Pfeiffer syndrome. METHODS: Retrospective review of patients diagnosed with Pfeiffer syndrome from January 1998 to January 2020 at one of England's 4 supraregional Craniofacial Units, Alder Hey Children's Hospital. Speech & Language Therapy case notes and medical notes were used to gather data, as well as the Oral Feeding Score component of the UK Craniofacial Outcome Score. RESULTS: Eleven patients were included. Six patients had no airway adjunct (55%): 3 had tracheostomy (27%) and 2 patients had NPA (18%). All patients with airway adjuncts were percutaneous endoscopic gastrostomy/percutaneous endoscopic jejunostomy fed. Those who did not require an airway adjunct had an Oral Feeding Score of 4.60 (SD: 0.49). The children who went on to have an airway adjunct had a mean preintervention Oral Feeding Score of 2.4 (SD: 0.8). The mean feeding score (postairway adjunct) in the NPA group was 2.0, compared with the tracheostomy group scoring 3.0. CONCLUSIONS: Children with Pfeiffer syndrome who require airway intervention have more significant feeding problems requiring feeding intervention. Although there were small numbers included in this study, there is a suggestion that airway adjuncts can contribute to feeding difficulties, particularly NPAs.

Feeding Outcomes After Mandibular Distraction for Airway Obstruction in Infants.

Robin sequence is a congenital issue resulting in airway obstruction, difficulty feeding, and failure to thrive. Mandibular Distraction Osteogenesis is used to improve airway obstruction in these patients, but little data exists characterizing feeding outcomes following surgery. This study aims to evaluate feeding outcomes and weight gain following mandibular distraction for airway correction in infants. A single-center retrospective chart review was conducted, and patients under 12 months old who underwent mandibular distraction between December 2015 and July 2021 were included in the study. The presence of cleft palate, distance of distraction, and polysomnography results were recorded. The primary outcomes were the length of distraction, need for nasogastric tube or G-tube at discharge, time lapsed to achieve full oral feeds, and weight gain (kilogram). Ten patients met the criteria. Of those 10 patients, 4 were syndromic, 7 had a cleft palate, and 4 had a congenital cardiac diagnosis. The average length of stay postsurgery was 28 days. Eight patients achieved full oral feeds in an average of 65.6 days. Five patients required nasogastric tube or G-tube at discharge, with 3 of these patients later transitioning to full oral feeds. All patients gained weight 3 months postsurgery with an average of 0.521 kg/mo. Patients who achieved full oral feeds gained an average of 0.549 kg/mo. Patients with supplementation gained an average of 0.454 kg/mo. All patients demonstrated improvement in airway obstruction with an average postoperative apnea hypopnea index of 1.64. Further investigation is necessary to identify challenges seen in feeding after mandibular distraction osteogenesis and improve care.

Tri-lobed Tongue: Rare Manifestation Accompany With Pierre Robin Sequence.

BACKGROUND: The tongue is an essential organ accounted for proper deglutition and articulation. Surgical repair should be planned soon after diagnosis of any structural abnormality to prevent later speech and swallowing disorders. The lobulated tongue could be isolated (sporadic) or in association with other disorders. Pierre Robin Sequence (PRS) consists of the clinical trial of congenital micrognathia, glossoptosis, and airway obstruction with variable inclusion of a cleft palate. We present the case of a rare congenital tri-lobed tongue with Pierre Robin sequence and its surgical management in our hospital setting. CASE PRESENTATION: Six-month-old boy presented with severe retrognathia, high arch, complete isolated cleft palate, and a bizarre mass in the oral cavity instead of his tongue that led to disruption of his swallowing. The mass (deformed tongue) check clearly, and the normal shape of the tongue was restored through multiple local randomized flaps. Dramatic improvement in swallowing was noticed 6 months after surgery during postoperative follow-up. DISCUSSION: We present the case of a patient with a tri-lobed tongue with Pierre Robin sequence characterized by severe retrognathia, high arch, and complete isolated cleft palate. This seems to be the first reported case of this particular craniofacial anomaly. CONCLUSION: The management of infants with the Pierre Robin sequence is complex, and much still needs to be learned and practiced. Congenital tri-lobed tongue with a cleft as part of the Pierre Robin sequence is a very rare malformation. Early repair of the tongue is important to assist the baby in adapting to speech and swallowing as they grow.