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1.
Int J Pediatr Otorhinolaryngol ; 180: 111964, 2024 May.
Article in English | MEDLINE | ID: mdl-38714046

ABSTRACT

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.


Subject(s)
Airway Obstruction , Middle Ear Ventilation , Pierre Robin Syndrome , Humans , Pierre Robin Syndrome/complications , Pierre Robin Syndrome/surgery , Male , Retrospective Studies , Airway Obstruction/surgery , Airway Obstruction/etiology , Female , Middle Ear Ventilation/methods , Infant , Tracheostomy , Child, Preschool , Airway Management/methods , Osteogenesis, Distraction/methods , Child
2.
Ann Plast Surg ; 92(6S Suppl 4): S382-S386, 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38725108

ABSTRACT

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.


Subject(s)
Mandible , Osteogenesis, Distraction , Pierre Robin Syndrome , Humans , Pierre Robin Syndrome/surgery , Pierre Robin Syndrome/complications , Osteogenesis, Distraction/methods , Retrospective Studies , Female , Male , Infant , Treatment Outcome , Mandible/surgery , Airway Obstruction/surgery , Airway Obstruction/etiology
3.
J Craniomaxillofac Surg ; 52(6): 792-797, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38644093

ABSTRACT

Congenital craniofacial malformations play an important role in upper airway obstruction. One of the main causes is mandibular hypoplasia which is present in the pierre robin sequence. Mandibular distraction osteogenesis (MDO) is one of the most commonly used treatments for the resolution of upper airway obstruction in patients that do not respond to a conservative treatment. We performed a long term follow up of syndromic and non-syndromic patients with pierre robin sequence where the lateral xrays were studied before surgery (T1), after MDO (T2) and long-term follow-up (T3, at least 5 years). Possible complications of the surgery were also studied through panoramic x-rays and clinical controls. The results evidenced an increase of mandibular length comparing T1-T2 and a good stability during the long-term follow-up. The Sd patients presented smaller mandible dimensions. We had not found any complications during the follow-up. The scars outcomes are modest, but none of the patients requested a surgical correction. Mandibular distraction osteogenesis is a treatment to be considered in patients with upper airway obstruction that do not respond to conservative treatments. The results of the surgery are stable and the complications in experienced hands are low.


Subject(s)
Airway Obstruction , Mandible , Osteogenesis, Distraction , Pierre Robin Syndrome , Humans , Osteogenesis, Distraction/methods , Airway Obstruction/surgery , Mandible/surgery , Mandible/abnormalities , Male , Female , Pierre Robin Syndrome/surgery , Pierre Robin Syndrome/complications , Pierre Robin Syndrome/diagnostic imaging , Child, Preschool , Child , Follow-Up Studies , Radiography, Panoramic , Infant , Adolescent , Treatment Outcome
4.
Clin Oral Investig ; 28(5): 247, 2024 Apr 11.
Article in English | MEDLINE | ID: mdl-38602599

ABSTRACT

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.


Subject(s)
Cleft Palate , Pierre Robin Syndrome , Humans , Infant , Cleft Palate/surgery , Hospitalization , Pierre Robin Syndrome/complications , Pierre Robin Syndrome/surgery , Retrospective Studies , Tongue
5.
J Clin Sleep Med ; 20(6): 995-997, 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38305773

ABSTRACT

The author recently observed a case involving a 12-year-old sighted girl who exhibited symptoms typical of non-24-hour sleep-wake rhythm disorder (N24SWD). This disorder, more commonly found in blind individuals, presents a unique challenge when diagnosed in those with vision. Several interventions can be attempted, ranging from behavioral adjustments to light therapy. Although melatonin has been noted for its effectiveness in realigning the patient's sleep-wake cycle, the use of ramelteon, a melatonin receptor agonist, has seldom been reported in managing N24SWD. However, this case illuminates the potential of ramelteon as another therapeutic option for sighted individuals with N24SWD. Further study is suggested to determine the potential of ramelteon in managing this disorder among sighted individuals of varying age groups. CITATION: Huang C-H. Managing non-24-hour sleep-wake rhythm disorder with ramelteon in a 12-year-old girl with Pierre Robin sequence and developmental delay: a case report. J Clin Sleep Med. 2024;20(6):995-997.


Subject(s)
Developmental Disabilities , Indenes , Pierre Robin Syndrome , Sleep Disorders, Circadian Rhythm , Humans , Female , Child , Indenes/therapeutic use , Sleep Disorders, Circadian Rhythm/drug therapy , Sleep Disorders, Circadian Rhythm/therapy , Developmental Disabilities/complications , Pierre Robin Syndrome/complications , Polysomnography
7.
J Craniofac Surg ; 35(1): 6-9, 2024.
Article in English | MEDLINE | ID: mdl-37622565

ABSTRACT

INTRODUCTION: While the literature is replete of clinical studies reporting on the Robin sequence (RS), population-based analyses are scarce with significant variability within the literature in terms of reported incidence, demographic parameters, and outcomes. The authors have conducted a 20-year population-based analysis to guide clinical practice. METHODS: A birth cohort was created from the available datasets in the Healthcare Cost and Utilization Project-Kids' Inpatient Database (HCUP-KID; 2000-2019). Robin sequence patients were identified and further stratified by syndromic status. Incidence, demographic parameters, and outcomes including mortality and tracheostomy rates were computed. A subset analysis comparing the isolated and syndromic cohorts was conducted. Data was analyzed through a χ 2 or t test. RESULTS: The incidence of RS was 5.15:10,000 (95% CI: 4.99-5.31) from a birth cohort of 7.5 million. Overall, 63.3% of the cohort was isolated RS and 36.7% had syndromic RS. Robin sequence patients had a significantly higher rate of cardiac (25.9%) and neurological (8.6%) anomalies compared with the general birth cohort and were most commonly managed in urban teaching hospitals ( P <0.0001). The pooled mortality and tracheostomy rates were 6.6% and 3.6%, respectively. Syndromic status was associated with a longer length of hospital stay (27.8 versus 13.6 d), tracheostomy rate (6.2% versus 2.1%), and mortality (14.1% versus 2.2%) compared with isolated RS ( P <0.0001). CONCLUSIONS: The true incidence of RS is likely higher than previously reported estimates. Isolated RS patients have a low associated mortality and tracheostomy rate and are typically managed in urban teaching hospitals. Syndromic status confers a higher mortality rate, tracheostomy rate, and length of stay compared with nonsyndromic counterparts.


Subject(s)
Pierre Robin Syndrome , Humans , United States/epidemiology , Retrospective Studies , Pierre Robin Syndrome/epidemiology , Pierre Robin Syndrome/surgery , Pierre Robin Syndrome/complications , Incidence , Length of Stay , Inpatients
8.
Ann Otol Rhinol Laryngol ; 133(1): 69-77, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37497838

ABSTRACT

OBJECTIVE: Robin sequence (RS) consists of micrognathia and glossoptosis that result in upper airway obstruction (UAO). In RS patients who undergo tracheostomy, long-term goals include natural decannulation (ND) without further surgical airway intervention. The objective of this study was to identify long-term trends in the rate and length of time to ND. METHODS: Retrospective chart review on 144 patients with RS treated from 1995 to 2020 at a pediatric tertiary care center. Patients were grouped by year of tracheostomy. Demographic data, UAO management, postoperative care, complications, and time to decannulation were recorded. RESULTS: Thirty-six patients met the inclusion criteria. Tracheostomy was performed at a median age of 45.5 days. 19 (53%) patients experienced ND at a median time of 66.1 months. ND rate was higher in non-syndromic patients (93% non-syndromic vs 27% syndromic; P < .0001) and during the first study period (1995-2006: 78%, 2007-2020: 28%; P = .003). Cox proportional-hazard regression demonstrated that white race [aHR 0.15 (0.03-0.8); P = .023] and higher birthweight [aHR 0.9 (0.8-0.98); P = .018] were associated with a higher likelihood of ND while a syndromic diagnosis had a negative association with ND [aHR 12.5 (3.3-50.0); P < .001]. CONCLUSIONS: Our study documented that ND in patients with RS who underwent tracheostomy was significantly associated with ethnicity, birthweight, and syndromic status. The negative impact on successful ND was most observed in patients with syndromic associations. Additionally, ND rates are lower in the 2007 to 2020 subgroup. We suspect this is because alternative management techniques such as tongue lip adhesion and mandibular distraction osteogenesis became primary surgical treatment in severe RS upper airway obstruction at our institution starting in 2007.


Subject(s)
Airway Obstruction , Pierre Robin Syndrome , Humans , Child , Infant , Retrospective Studies , Treatment Outcome , Pierre Robin Syndrome/complications , Pierre Robin Syndrome/surgery , Birth Weight , Airway Obstruction/etiology , Airway Obstruction/surgery , Tracheostomy/adverse effects
9.
Cleft Palate Craniofac J ; 61(1): 79-86, 2024 01.
Article in English | MEDLINE | ID: mdl-36443934

ABSTRACT

OBJECTIVE: To investigate the subjective risk for obstructive sleep apnea (OSA) in adolescents and young adults with isolated Robin sequence (IRS). Additionally, to investigate the association of OSA risk with respiratory signs/symptoms, and retrognathia. DESIGN: Prospective, observational, and cross-sectional study. SETTING: Tertiary reference hospital for the rehabilitation of craniofacial anomalies. PARTICIPANTS: Adolescents and adults (n = 30) with IRS were clinically evaluated and screened through the Berlin Questionnaire (BQ) and Respiratory Symptoms Questionnaire. The maxillomandibular relationship was assessed on lateral cephalograms of those that reached skeletal maturity (n = 13). Polysomnography (PSG) was performed in a subgroup of 4 individuals. RESULTS: The mean age of the sample was 18.2 (±3.4) years, 17 (56.7%) were adolescents (14-19 years), and 16 were (53.3%) female, all presented a repaired cleft palate. CLINICAL PARAMETERS: Systemic arterial pressure (118.0 ± 4.1/76.3 ± 4.9 mmHg), body mass index (BMI) (20.9 ± 2.8 kg/m2), neck (33.2 ± 2.3 cm), and waist circumferences (72.0 ± 5.8 cm) were within normal ranges. A skeletal class I pattern was observed in 61.5% of the participants while a class II was seen in 15.4% of them. A high risk for OSA was detected in 16.7%, and it was associated with nasal obstruction, snoring and drowsiness, and a skeletal class II pattern (P ≤ .05). One patient presented with mild OSA (apnea-hypopnea index [AHI] = 10.1 events/hour) at the PSG exam. CONCLUSIONS: A high risk for OSA can be observed with a moderate frequency among adolescents and young adults with IRS, especially among those who are concurrently suffering from nasal obstruction, snoring and retrognathia.


Subject(s)
Nasal Obstruction , Pierre Robin Syndrome , Retrognathia , Sleep Apnea, Obstructive , Adolescent , Adult , Female , Humans , Male , Young Adult , Cross-Sectional Studies , Nasal Obstruction/complications , Pierre Robin Syndrome/complications , Pierre Robin Syndrome/diagnosis , Prospective Studies , Retrognathia/complications , Risk Factors , Sleep Apnea, Obstructive/diagnosis , Sleep Apnea, Obstructive/epidemiology , Sleep Apnea, Obstructive/etiology , Snoring
10.
Kobe J Med Sci ; 69(3): E115-E121, 2023 Nov 16.
Article in English | MEDLINE | ID: mdl-38018223

ABSTRACT

The purpose of this study was to provide the experience of a single center with mandibular distraction osteogenesis (MDO) in Pierre Robin Sequence (PRS) patients. A longitudinal research analysis was conducted to identify PRS patients who underwent MDO at Vietnam National Children's Hospital between 2019 and 2021. The following criteria were used to determine inclusion: 1) those pediatric patients with PRS who were not well handled with conservative therapy, 2) those who received MDO with internal mandibular distractors, and 3) no previous treatment elsewhere. Demographic data, postoperative complications, and surgical results were all evaluated. The inclusion criteria were met by 73 patients. There were no difficulties associated with our distraction strategy. The majority of individuals with tracheostomies were successfully decannulated, and the remainder were able to avoid tracheostomies. Using MDO in PRS is an effective technique to avoid future airway issues. The success rate was lower and the complication rate higher for patients who had a tracheotomy before distraction and for those who underwent distraction at an age older than 2 months. The presence of laryngomalacia, gastric reflux disease, cardiac abnormalities, and GI anomalies did not increase the likelihood of MDO failure in PRS patients.


Subject(s)
Osteogenesis, Distraction , Pierre Robin Syndrome , Humans , Infant , Child , Osteogenesis, Distraction/methods , Pierre Robin Syndrome/surgery , Pierre Robin Syndrome/complications , Vietnam , Retrospective Studies , Treatment Outcome , Mandible/surgery , Mandible/abnormalities
11.
Acta Paediatr ; 112(12): 2601-2610, 2023 12.
Article in English | MEDLINE | ID: mdl-37786287

ABSTRACT

AIM: We aimed to investigate the developmental outcome of children with Robin sequence (RS) for whom continuous positive airway pressure was the main strategy to release upper airway obstruction. METHODS: We included children with isolated RS or RS associated with Stickler syndrome who were aged 15 months to 6 years. We used the French version of the Child Development Inventory and calculated the developmental quotient (DQ) for eight different domains and the global DQ (DQ-global). We searched for determinants of risk of delay. RESULTS: Of the 87 children, for 71%, the developmental evolution was within the norm (DQ-global ≥86 or ≥-1 SD), 29% were at high risk of delay (DQ-global <86 or <-1 SD), and only 3% were at very high risk of delay (DQ-global <70 or <-2 SD). The DQs for expressive language and language comprehension were lower in our study population than the general population, but an improvement was noticed with the children's growth. CONCLUSION: Risk of a developmental delay was not greater for children with the most severe respiratory phenotype than the others. Children whose mothers had low education levels were more at risk than the others.


Subject(s)
Hearing Loss, Sensorineural , Pierre Robin Syndrome , Female , Humans , Child , Infant , Pierre Robin Syndrome/complications , Pierre Robin Syndrome/therapy , Paris , Child Development , Mothers
12.
Int J Oral Maxillofac Surg ; 52(12): 1250-1254, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37532615

ABSTRACT

The purpose of this article is to report the author's technique for using patient-specific distractors for customized distraction osteogenesis of the mandible in patients with Pierre Robin sequence and upper airway obstruction. The advantages of virtual planning and patient-specific plates in other aspects of craniomaxillofacial surgery, such as orthognathic and reconstructive surgery, have been reported previously. Similar to patient-specific plates, the theorized advantages of patient-specific distractors in infants with Robin sequence and upper airway obstruction include increased accuracy, decreased operating time, and less morbidity to vital anatomic structures such as the inferior alveolar nerve and developing tooth buds. This technique is novel in using patient-specific distractors in the craniomaxillofacial skeleton.


Subject(s)
Airway Obstruction , Osteogenesis, Distraction , Pierre Robin Syndrome , Humans , Infant , Pierre Robin Syndrome/complications , Pierre Robin Syndrome/surgery , Osteogenesis, Distraction/methods , Airway Obstruction/surgery , Mandible/surgery , Treatment Outcome , Retrospective Studies
13.
J Craniofac Surg ; 34(6): 1640-1643, 2023 Sep 01.
Article in English | MEDLINE | ID: mdl-37431904

ABSTRACT

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.


Subject(s)
Airway Obstruction , Cleft Palate , Osteogenesis, Distraction , Pierre Robin Syndrome , Humans , Infant , Retrospective Studies , Treatment Outcome , Mandible/surgery , Mandible/abnormalities , Airway Obstruction/etiology , Airway Obstruction/surgery , Osteogenesis, Distraction/methods , Pierre Robin Syndrome/complications , Pierre Robin Syndrome/surgery , Weight Gain
14.
Br J Oral Maxillofac Surg ; 61(7): 475-481, 2023 09.
Article in English | MEDLINE | ID: mdl-37453893

ABSTRACT

Pierre Robin Sequence (PRS) is a congenital craniofacial anomaly distinguished by the presence of micrognathia, glossoptosis, and upper airway obstruction. Cleft palate occurs in over 3/4 of patients with PRS. The wide U-shape cleft and airway dysfunction create challenges in clinical management. Currently, disputes exist on the treatment protocol and prognosis of cleft palate management among patients with PRS. This review is focused on the deformity features, intervention timing, technique selection, airway support, and outcome evaluation of cleft palate among patients with PRS, aiming to provide reference to further evolution in the management of PRS-related cleft palate.


Subject(s)
Airway Obstruction , Cleft Palate , Pierre Robin Syndrome , Humans , Cleft Palate/complications , Cleft Palate/surgery , Airway Obstruction/etiology , Airway Obstruction/surgery , Pierre Robin Syndrome/complications , Pierre Robin Syndrome/surgery , Prognosis , Outcome Assessment, Health Care
15.
Birth Defects Res ; 115(13): 1216-1221, 2023 08 01.
Article in English | MEDLINE | ID: mdl-37340830

ABSTRACT

BACKGROUND: TARP syndrome, characterized by talipes equinovarus, atrial septal defect, Robin sequence, and persistent left superior vena cava, is an X-linked recessive condition caused by deleterious variants in RBM10. Vitelline vascular remnants (VVR) are a rare vitelline duct anomaly with approximately 26 cases previously reported. There are no previously reported cases of VVRs in patients with TARP syndrome. CASE: We present a male neonate diagnosed with TARP syndrome via trio whole exome sequencing who had classic features of this syndrome, although his course was additionally complicated by feeding intolerance with multiple episodes of abdominal distension. Serial imaging and contrast studies of the upper GI tract and small bowel demonstrated small bowel obstruction of unclear etiology. Given the poor prognosis associated with this condition, life-sustaining measures were withdrawn, and he passed away at 38 days of age. On autopsy, a VVR was unexpectedly identified with proximal bowel dilation, explaining his feeding intolerance. CONCLUSIONS: We highlight the importance of full post-mortem examination in understanding the complete spectrum of manifestations of genetic syndromes and provide a review of the literature.


Subject(s)
Clubfoot , Intestinal Obstruction , Pierre Robin Syndrome , Humans , Male , Infant, Newborn , Pierre Robin Syndrome/complications , Pierre Robin Syndrome/diagnosis , Pierre Robin Syndrome/genetics , Clubfoot/complications , Clubfoot/diagnosis , Clubfoot/genetics , Vena Cava, Superior , Phenotype , Intestinal Obstruction/etiology , RNA-Binding Proteins/genetics
16.
Braz. J. Anesth. (Impr.) ; 73(3): 344-346, May-June 2023. graf
Article in English | LILACS | ID: biblio-1439620

ABSTRACT

Abstract Pierre Robin Sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, and cleft palate that presents with airway obstruction and developmental delay with or without other congenital anomalies. These patients' anesthesia management is challenging because of difficult ventilation and intubation. Regional anesthesia methods should be considered for these patients on a case-by-case basis. This report presents primary use of regional anesthesia for circumcision of a 9-year-old boy with PRS.


Subject(s)
Humans , Male , Child , Pierre Robin Syndrome/surgery , Pierre Robin Syndrome/complications , Cleft Palate/surgery , Cleft Palate/complications , Airway Obstruction/surgery , Pudendal Nerve , Anesthesia, Conduction/adverse effects , Nerve Block/adverse effects
17.
J Craniomaxillofac Surg ; 51(4): 209-216, 2023 Apr.
Article in English | MEDLINE | ID: mdl-37117117

ABSTRACT

The purpose of this study was to assess the effect of tongue-lip adhesion (TLA) on the long-term speech and articulation outcomes of patients with Robin sequence (RS) after cleft palate repair. Outcomes were compared to those in patients with RS who required positioning alone and to patients with isolated cleft palate (ICP). All consecutive patients with RS (with or without TLA) versus isolated cleft palate (ICP) who underwent cleft palate repair were retrospectively reviewed. Speech and articulation included all assessments between the age of 3-6 years. Secondary speech operations, velopharyngeal insufficiency (VPI), hypernasality, and articulation errors by cleft-type characteristics (CTC), including 4 categories (passive), non-oral, anterior-oral, and posterior-oral. A total of 41 RS patients and 61 ICP patients underwent repair with sufficient follow-up. Of them, 23 patients underwent a TLA at median age of 12 days. Rates of hypernasality (p = 0.004), secondary speech operations (p = 0.004), and posterior oral CTC (p = 0.042) were higher in RS compared to ICP. Isolated RS had speech outcomes similar to those of ICP; however, syndromic RS patients needed more secondary speech operations compared to isolated RS (p = 0.043). TLA-RS patients did not demonstrate differences in speech outcomes or any CTCs (all p > 0.05) compared to non-TLA-RS patients, except for the anterior oral CTC (74% TLA-RS vs 28% non-TLA-RS, p = 0.005). Within the limitations of the study, it seem that TLA does not affect long-term velopharyngeal function in patients with RS. However, TLA-RS patients demonstrated higher rates of anterior-oral CTC, which might be related to a different positioning of the tongue after TLA. Every effort should be taken to treat patients with RS conservatively instead of with TLA because of this demonstrated a negative effect on one type of articulation error. However, if conservative therapy fails, a TLA is still a valuable adjunct in the treatment of RS, and cleft speech pathologists who treat such patients should be more aware of this phenomenon in order to improve long-term articulation outcomes.


Subject(s)
Cleft Lip , Cleft Palate , Pierre Robin Syndrome , Velopharyngeal Insufficiency , Humans , Child, Preschool , Child , Infant, Newborn , Cleft Palate/surgery , Cleft Palate/complications , Retrospective Studies , Speech , Pierre Robin Syndrome/complications , Pierre Robin Syndrome/surgery , Treatment Outcome , Velopharyngeal Insufficiency/etiology , Velopharyngeal Insufficiency/surgery , Tongue , Cleft Lip/complications
18.
J Oral Maxillofac Surg ; 81(6): 716-720, 2023 06.
Article in English | MEDLINE | ID: mdl-36931318

ABSTRACT

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.


Subject(s)
Airway Obstruction , Osteogenesis, Distraction , Pierre Robin Syndrome , Humans , Infant , Male , Female , Retrospective Studies , Treatment Outcome , Pierre Robin Syndrome/surgery , Pierre Robin Syndrome/complications , Tracheostomy , Mandible/surgery , Mandible/abnormalities , Intubation, Intratracheal , Airway Obstruction/surgery
19.
Pediatr Neurol ; 141: 72-76, 2023 04.
Article in English | MEDLINE | ID: mdl-36774683

ABSTRACT

BACKGROUND: The Pierre Robin Sequence presents heterogeneous symptoms, and each newborn can manifest from mild breathing and feeding difficulties to severe complications, as well as a predisposition to present changes in growth and neuropsychomotor development in the first years of life. OBJECTIVE: The aims were to evaluate and associate the neuropsychomotor development of zero- to 12-month-old children with Pierre Robin sequence (PRS) in the personal-social, fine motor-adaptive, language, and gross motor aspects. METHODS: The subjects of the study were 17 infants of both sexes with PRS admitted to the special care unit (SCU) of a reference hospital in the interior of the state of São Paulo, Brazil, in the age range of 20 days to 263 days. Developmental assessments were performed using the Denver Development Screening Test II. The evaluations were carried out in the SCU, with duration of 30 minutes each. Statistical analysis was descriptive using the Mann-Whitney test, two-proportion equality test, and Spearman correlation. The level of significance was set at 0.05. RESULTS: According to Denver Development Screening Test II, median 78.5 of the babies were at risk for developmental delay identified by the Denver II Test (n = 14, 82.4%). For the developmental areas analyzed by the test there was statistically significant difference in language area. CONCLUSION: The babies aged up to 12 months with PRS in this study presented risks for delay in neuropsychomotor development in language, gross motor, fine motor-adaptive, and personal-social aspects, and this finding should be considered to set goals in family orientation and intervention.


Subject(s)
Pierre Robin Syndrome , Infant , Infant, Newborn , Male , Child , Female , Humans , Aged , Pierre Robin Syndrome/complications , Brazil , Language , Retrospective Studies
20.
Pediatr Emerg Care ; 39(3): 159-161, 2023 Mar 01.
Article in English | MEDLINE | ID: mdl-36791027

ABSTRACT

INTRODUCTION: Video laryngoscopy (VL) has been proposed to increase the likelihood of successful intubation in patients with predicted difficult airways such as those with Pierre Robin sequence (PRS). Prior studies have focused on the performance of anesthesiologists, who are generally considered airway experts. Our primary aim was to investigate the success rate of intubation using VL compared with direct laryngoscopy (DL) when attempted by pediatric residents on a PRS model. METHODS: Participants were administered a 5-minute refresher video on 2 VL techniques (CMAC, conventional geometry VL, and McGrath, unconventional geometry VL) and DL. The participants were asked to intubate the AirSim PRS infant manikin. The order of VL and DL use was randomly selected. All intubations were video recorded, and the recordings were analyzed by 3 anesthesiologists blinded to the participant's identity and previous experience. RESULTS: Seventeen of 23 residents succeeded in intubating the PRS model using DL. Only 9 residents succeeded in intubating the PRS model using VL (conventional or unconventional geometry). Intubation success rate was higher when comparing DL with VL ( P = 0.04) and similar when comparing VL devices ( P = 0.69). DISCUSSION: Contrary to expectation, the intubation success rate was lower using VL than with DL among pediatric residents. This should be considered when designing residency training and in real-life resuscitation.


Subject(s)
Laryngoscopes , Pierre Robin Syndrome , Infant , Humans , Child , Laryngoscopy/methods , Cross-Over Studies , Manikins , Pierre Robin Syndrome/complications , Pierre Robin Syndrome/therapy , Intubation, Intratracheal/methods , Video Recording
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