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Journal of Cranio-maxillo-facial... Apr 2024The objective of this study was to report outcomes of early cleft palate repair in infants born with Robin sequence (RS). A retrospective case series in a tertiary...
The objective of this study was to report outcomes of early cleft palate repair in infants born with Robin sequence (RS). A retrospective case series in a tertiary referral paediatric hospital was carried out, examining a consecutive series of 69 infants born with RS and cleft palate. A minimally invasive approach was taken to upper airway obstruction, with liberal nasopharyngeal airway (NPA) and non-invasive ventilation (NIV) use, guided by sleep studies. The palate was repaired between 6 and 9 months with a modified Malek technique. The most frequently used airway adjunct (59.4% of patients) was an NPA and the median duration of use was 5.6 months. All patients underwent a modified Malek cleft palate repair at a median of 7 months of age. Overnight oximetry demonstrated higher mean oxygen saturation (SpO) across the group from initial neonatal admission to discharge (median 96.5% (interquartile range [IQR] 95-98%) vs 97.45% (IQR 96.5-98%) (P = 0.2, N = 34). Of those with a cardiorespiratory polysomnogram, the obstructive apnoea-hypopnea index (OAHI) was significantly lower postoperatively (5.9 vs 2.8, P = 0.028). This study supports the use of non-surgical airway strategies and early cleft palate repair in infants born with RS and cleft palate.
Topics: Infant; Infant, Newborn; Humans; Child; Cleft Palate; Pierre Robin Syndrome; Retrospective Studies; Airway Management; Nasopharynx; Airway Obstruction
PubMed: 38448335
DOI: 10.1016/j.jcms.2024.02.006 -
National Journal of Maxillofacial... 2024Pierre Robin sequence poses a great challenge for anesthesiologists during laryngoscopy and intubation, making oxygenation and ventilation difficult. The role of early...
Pierre Robin sequence poses a great challenge for anesthesiologists during laryngoscopy and intubation, making oxygenation and ventilation difficult. The role of early surgical intervention is recommended for the improvement of the airway and overall survival of the neonate. The situation becomes even more challenging, when the neonate may not be fit for such surgical interventions. The present case posed such a challenge to the team. To the authors' knowledge, the decision to use a face mask as an interim life-saving measure was considered for the first time. This provided a greater window of opportunity for further course of action, only to be later managed by distraction osteogenesis of the mandible. The unconventional use of orthopedic appliances for the management of threatened airways may provide the clinician with time, where further management may be carried out. The present article will explain such a procedure that was carried out as a life-saving measure.
PubMed: 38690248
DOI: 10.4103/njms.njms_99_23 -
Frontiers in Physiology 2024[This corrects the article DOI: 10.3389/fphys.2023.1292523.].
[This corrects the article DOI: 10.3389/fphys.2023.1292523.].
PubMed: 38952871
DOI: 10.3389/fphys.2024.1400492 -
Plastic and Reconstructive Surgery Mar 2024The development of mandibular distraction osteogenesis (MDO) and tongue-lip adhesion (TLA) has led to significant improvements in respiratory outcomes for the Robin...
BACKGROUND
The development of mandibular distraction osteogenesis (MDO) and tongue-lip adhesion (TLA) has led to significant improvements in respiratory outcomes for the Robin sequence (RS) population. Despite such advances, there continues to be debate regarding management strategies. The authors present their experience managing the RS population with insights on technique selection.
METHODS
A retrospective review of RS patients treated at the senior author's institution from 2003 to 2021 was conducted. Baseline patient demographics and clinical parameters including feeding and respiratory status were recorded. Outcomes included the need for tracheostomy or tracheostomy, decannulation rates, and feeding status. Patients were evaluated through overnight oximetry and drug-induced sleep endoscopy (DISE). Outcomes were stratified according to management technique (MDO, TLA, versus conservative) and compared through statistical analysis.
RESULTS
Fifty-nine RS patients were included. Twenty-eight were managed conservatively, 19 underwent MDO, 10 underwent TLA, one underwent both TLA and MDO, and one underwent tracheostomy primarily. Overall, 1.7% of the cohort required a tracheostomy and 86% achieved oral feeding after the procedure. The MDO cohort had lower Apgar scores and mean birth weight compared with the conservative and TLA cohorts ( P < 0.05). There were no statistical differences in respiratory and feeding outcomes across all three cohorts.
CONCLUSIONS
A therapeutic algorithm was developed with insight into the use of DISE and risk stratification with overnight oximetry to guide procedural selection. Using this approach, safe and satisfactory respiratory outcomes were achieved with a low tracheostomy rate. Risk stratification is possible without polysomnography, and DISE is a promising tool (that requires further validation) for procedural selection in this population.
CLINICAL QUESTION/LEVEL OF EVIDENCE
Therapeutic, IV.
Topics: Child; Humans; Infant; Treatment Outcome; Pierre Robin Syndrome; Airway Obstruction; Lip; Retrospective Studies; Osteogenesis, Distraction; Mandible; Algorithms; Hospitals
PubMed: 37104466
DOI: 10.1097/PRS.0000000000010587 -
The Journal of Craniofacial Surgery Jan 2024Though it has made significant strides, Vietnam remains a resource-constrained country of 98 million people. Vietnam National Children's Hospital (VNCH) provides...
Implementation of Mandibular Distraction Osteogenesis for Patients With Pierre Robin Sequence in a Developing Country Through International Collaboration: A Paradigm for Success.
OBJECTIVE
Though it has made significant strides, Vietnam remains a resource-constrained country of 98 million people. Vietnam National Children's Hospital (VNCH) provides tertiary care to a catchment of 40 million people and is the sole national children's hospital. As such, it is one of the few referral centers in the country equipped to take care of patients diagnosed with Pierre Robin sequence (PRS) as this requires pulmonary, critical care, otolaryngology, and plastic surgery expertise. Before 2015, the only surgical options were tongue lip adhesion or tracheostomy. Only 20% of patients successfully avoided tracheostomy, mechanical ventilation, or death. From 2015 to 2019, mandibular distraction osteogenesis (MDO) was introduced by visiting international surgeons on a short-term basis. Since 2020, local surgeons at VNCH have refined their technique and widely use MDO independently. This report seeks to capitulate their experience and identify factors leading to success.
METHODS
A retrospective review was conducted of patients diagnosed with PRS at VNCH from 2015 to 2022. Paper records were digitized, translated, and reviewed for inclusion criteria, including demographics, indications, hospital course, and postoperative outcomes.
RESULTS
Complete records satisfying inclusion criteria were available for 53 patients with a diagnosis of PRS who underwent MDO from 2020 to 2022. From 2015 to 2019, there were 19 cases of MDO, though records were incomplete. The median age at the time of MDO was 50 ± 43 days. Forty patients (75.5%) had isolated PRS and 13 (24.5%) were syndromic. Forty-four patients (83%) had a cleft palate. Fifty-one (96.2%) of patients required preoperative supplemental oxygen or mechanical ventilation. The active distraction and consolidation phase was 4.8 ± 1.3 months. The median days to discharge after surgery was 19.0 ± 8.3 days. Median weight at birth, at the time of surgery, and at the time of device removal were 6.8 ± 1.2, 7.7 ± 1.9, and 14.8 ± 2.8 pounds, respectively. Fifty-two patients (98.1%) had obstructive sleep apnea preoperatively with an average Apnea Hypopnea Index of 25.0 ± 10.6. Post-MDO, only 4 (7.5%) had obstructive sleep apnea and the average Apnea Hypopnea Index was 5.2 ± 0.6. No patients (0) required a tracheostomy for a 100% success rate.
CONCLUSIONS
The tremendous success of the implementation of MDO by local surgeons in Vietnam after its introduction by visiting international surgeons illustrates a paradigm for capacity-enhancing global surgical endeavors. Mandibular distraction osteogenesis has replaced tongue lip adhesion as the surgical treatment of choice for PRS patients at VNCH. Surgical techniques can be transferred to operating environments with basic infrastructure through collaboration and resource optimization. These results demonstrate that global surgical engagement may be scalable and repeatable with direct benefits for patients in lower-middle-income countries.
PubMed: 38231188
DOI: 10.1097/SCS.0000000000009934 -
Yonago Acta Medica Nov 2023Cleft lip and/or palate (CL/P) can be accompanied by other congenital anomalies. We conducted a long-term evaluation of the associations between cleft patterns, sex...
BACKGROUND
Cleft lip and/or palate (CL/P) can be accompanied by other congenital anomalies. We conducted a long-term evaluation of the associations between cleft patterns, sex distribution, and accompanying congenital anomalies of patients with CL/P.
METHODS
The medical records of 739 patients with CL/P, seen between January 1967 and December 2020, were retrospectively reviewed. Fisher's exact test was used for statistical analysis.
RESULTS
Among the 739 patients with CL/P, the male-to-female ratio was 1.1. Regarding the cleft pattern, 121 (16.4%), 104 (14.1%), 280 (37.9%), 198 (26.8%), and 36 (4.9%) patients had cleft lip (CL), cleft lip and alveolus (CLA), cleft lip and palate (CLP), cleft palate (CP), and submucous cleft palate (SMCP), respectively. Congenital anomalies were identified in 107 (14.5%) cases, of which 53 (49.5%) had congenital heart disease. The frequencies of congenital anomalies patients with in CL/P were 14/225 (6.2%), 36/280 (12.9%), 43/198 (21.7%), and 14/36 (38.9%) for a combination of CL and CLA, CLP, CP, and SMCP, respectively. Accompanying syndromes and chromosomal anomalies were identified in 40 (5.4%) cases, in which Pierre Robin sequence (16 cases of CP and 4 cases of SMCP) was the most frequent.
CONCLUSION
No sex differences were observed in CL/P, and CLP and CP were the most common cleft patterns. Congenital anomalies associated with CL/P were dominated by congenital heart disease and were most frequently identified in CP and SMCP cases. Notably, the Pierre Robin sequence, a complex syndrome characterized by micrognathia, glossoptosis, respiratory obstruction, and a U- or V-shaped CP, was found in cases of both CP and SMCP, and accounted for the symptoms in most cases.
PubMed: 38028265
DOI: 10.33160/yam.2023.11.009 -
International Journal of Pediatric... May 2024Investigate an association between upper airway obstruction (UAO) management in Robin Sequence (RS) and need for bilateral myringotomy and tubes (BMT).
OBJECTIVE
Investigate an association between upper airway obstruction (UAO) management in Robin Sequence (RS) and need for bilateral myringotomy and tubes (BMT).
METHODS
Retrospective chart review of RS patients treated at a tertiary free-standing pediatric hospital from 1995 to 2020 was performed. Patients were grouped based on airway management: conservative, tracheostomy, tongue-lip adhesion (TLA), and mandibular distraction osteogenesis (MDO). Demographic data, cleft palate (CP) association, numbers of BMT and ear infections, and audiogram data including tympanograms were collected. One-way ANOVA and Chi-square/Fisher's exact tests were used to compare continuous and categorical data, respectively. Multivariable regression analysis was used to compare BMT rates between treatment groups.
RESULTS
One hundred forty-eight patients were included, 70.3 % of which had CP. Most patients (67.6 %) had at least one BMT; 29.1 % required two or more BMT. The rate of BMT was higher in patients with CP compared to those with intact palates (p = 0.003; 95 % CI 1.30-3.57) and those treated with tracheostomy (p = 0.043; 95 % CI 1.01, 2.27). Surgically managed patients were more likely to have hearing loss (67.5 % vs. 35.3 %, p = 0.017) and ear infections (42.1 % vs. 20.0 %, p = 0.014) pre-compared to post-procedure for airway management.
CONCLUSION
Most RS patients require at least 1 set of BMT. Those with CP and/or treated with tracheostomy had a higher likelihood of needing BMT. Rate of hearing loss and ear infection was higher in surgically managed RS patients. Patients with RS and overt CP require a statistically higher number of BMTs compared to those with either submucous cleft palate or intact palate.
Topics: Humans; Pierre Robin Syndrome; Male; Retrospective Studies; Airway Obstruction; Female; Middle Ear Ventilation; Infant; Tracheostomy; Child, Preschool; Airway Management; Osteogenesis, Distraction; Child
PubMed: 38714046
DOI: 10.1016/j.ijporl.2024.111964 -
Clinical Oral Investigations Apr 2024There is a paucity of information about the possible risk factors that could identify patients with Robin sequence (RS) who are more prone to developing obstructive...
OBJECTIVES
There is a paucity of information about the possible risk factors that could identify patients with Robin sequence (RS) who are more prone to developing obstructive airway complications after palate closure. This study aimed to compare the respiratory complication rates in patients with RS and isolated cleft palate (ICP).
MATERIALS AND METHODS
In this retrospective study, we reviewed the medical records of 243 consecutive patients with RS and ICP who were treated at Amsterdam University Medical Centers over the past 25 years. We collected preoperative data on previous treatment, diagnostic findings, surgical technique, weight, and presence of congenital anomalies.
RESULTS
During cleft palate closure, patients with RS were older (11.9 versus 10.1 months; p = 0.001) and had a lower gestational age than those with ICP (37.7 versus 38.5 weeks; p = 0.002). Patients with RS had more respiratory complications (17 versus 5%; p = 0.005), were more often non-electively admitted to the pediatric intensive care unit (PICU) (13 versus 4.1%; p = 0.022), and had a longer hospital stay duration (3.7 versus 2.7 days; p = 0.011) than those with ICP. The identified risk factors for respiratory problems were a history of tongue-lip-adhesion (TLA) (p = 0.007) and a preoperative weight of < 8 kg (p = 0.015). Similar risk factors were identified for PICU admission (p = 0.015 and 0.004, respectively).
CONCLUSIONS
The possible risk factors for these outcomes were a low preoperative weight and history of TLA. Closer postoperative surveillance should be considered for patients with these risk factors.
CLINICAL RELEVANCE
Identifying risk factors for respiratory complications could provide clinicians better insight into their patients and allows them to provide optimal care for their patients.
Topics: Humans; Infant; Cleft Palate; Hospitalization; Pierre Robin Syndrome; Retrospective Studies; Tongue
PubMed: 38602599
DOI: 10.1007/s00784-024-05647-w -
Journal of Clinical Orthopaedics and... Feb 2024Pierre Robin sequence is a rare congenital disorder with variable associations. A two-year-old female child was brought in with complaints of an abnormal gait and right...
Pierre Robin sequence is a rare congenital disorder with variable associations. A two-year-old female child was brought in with complaints of an abnormal gait and right lower limb shortening. A comprehensive clinical evaluation unveiled facial characteristics resembling those of Pierre Robin Sequence, including micrognathia, a cleft palate, and glossoptosis. The child also exhibited retrognathia, a low posterior hairline, bilateral developmental dysplasia of the hips, right foot syndactyly, and a left ectopic kidney as confirmed by ultrasonography. Radiological examination showed bilateral developmental dysplastic hips. The connection between Pierre Robin sequence and the presence of bilateral dysplastic hips and an ectopic kidney is an unusual and rare combination.
PubMed: 38323076
DOI: 10.1016/j.jcot.2024.102336 -
Journal of Cranio-maxillo-facial... Jan 2024The study aimed to evaluate the mid-term effect of MDO in children with Robin sequence (RS). In this case series, 13 patients with RS who underwent MDO were followed up...
The study aimed to evaluate the mid-term effect of MDO in children with Robin sequence (RS). In this case series, 13 patients with RS who underwent MDO were followed up for more than 5 years. Data were collected using clinical history and physical examination. Polysomnography was performed and endoscopic evaluations of the airway was performed if patients still presented obstructive signs of upper airways and/or dysphagia. The patients' clinical signs improved in the mid-term after versus before MDO (inspiratory noise, 92,3% vs 30,8%; apnea, 84,6% vs 7,7%; cyanosis, 76,9% vs 0%; desaturations, 69,2% vs 0%; and suprasternal/intercostal retractions, 61,5% vs 0%; p < 0.05). Statistically significant improvement was noted in the following polysomnographic parameters evaluated in the pre and postoperative mid-term: apnea-hypopnea index, total sleep time and desaturation index (p < 0.05). Within the limitations of the study it seems that MDO is an effective surgical option for children with RS, not only in the short term as previously demonstrated, but also in the mid-term.
Topics: Child; Humans; Infant; Polysomnography; Retrospective Studies; Pierre Robin Syndrome; Osteogenesis, Distraction; Apnea; Treatment Outcome; Mandible; Airway Obstruction
PubMed: 37884434
DOI: 10.1016/j.jcms.2023.08.004