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International Journal of Obstetric... Aug 2019We present the anaesthetic management of a parturient with kyphomelic dysplasia and Pierre Robin Sequence who underwent elective caesarean delivery. Potential...
We present the anaesthetic management of a parturient with kyphomelic dysplasia and Pierre Robin Sequence who underwent elective caesarean delivery. Potential anaesthetic issues and management strategies are discussed.
Topics: Abnormalities, Multiple; Adult; Anesthesia, Obstetrical; Bone Diseases, Developmental; Cesarean Section; Female; Humans; Infant, Newborn; Pierre Robin Syndrome; Pregnancy; Pregnancy Complications
PubMed: 30879933
DOI: 10.1016/j.ijoa.2019.02.007 -
Die Anaesthesiologie Oct 2022As part of surgical interventions in pediatric patients, children with craniofacial malformations and syndromes are presented in the field of oral and maxillofacial...
INTRODUCTION
As part of surgical interventions in pediatric patients, children with craniofacial malformations and syndromes are presented in the field of oral and maxillofacial surgery, anesthesia and also in all other clinical disciplines. In particular, the Pierre Robin sequence in the clinical context leads to a situation albeit a rare one, which should be given high attention in preoperative, intraoperative and postoperative care.
MATERIAL AND METHODS
In a retrospective analysis from 1993 to 2020 in the Department of Oral and Maxillofacial Surgery at the University Hospital Halle (Saale), a total of 54 patients were identified with syndromic changes and a need for surgical treatment. During this period, 12 patients with a Pierre Robin sequence were genetically confirmed, who received a total of 20 surgical interventions under general anesthesia at different times. Statistical analysis was performed using SPSS 17.0.
RESULTS
In 12 patients with a Pierre Robin sequence, 20 surgical procedures were performed with the patient under general anesthesia. The youngest patients had an average age of 6 months, the oldest 16 years at the time of the operation. The average age was 5.7 years. In addition to the genetic component, all children were assigned to the ASA I classification. The surgical indication was initially an isolated cleft palate in all patients, followed by further interventions such as dental restorations, corrective surgery in the area of the palate or ear nose throat (ENT) examinations. Drug induction of general anesthesia was weight-adapted using propofol 1%, fentanyl or remifentanil and rocuronium. In our study, out of 18 orotracheal intubations, only 2 patients had to be intubated by video laryngoscopy. One patient required nasal intubation and another was fitted with a laryngeal mask. The success rate of conventional intubation was 89.5%. Postoperatively, one infant had recurrent drops in saturation, so that reintubation was necessary.
Topics: Anesthesia, General; Child; Child, Preschool; Fentanyl; Hospitals, University; Humans; Infant; Laryngeal Masks; Pierre Robin Syndrome; Propofol; Remifentanil; Retrospective Studies; Rocuronium
PubMed: 35925195
DOI: 10.1007/s00101-022-01181-0 -
Korean Journal of Anesthesiology Jun 2021
Topics: Humans; Intubation, Intratracheal; Pierre Robin Syndrome
PubMed: 33070581
DOI: 10.4097/kja.20553 -
Pediatric Pulmonology Aug 2022Robin sequence is characterized by mandibular retrognathia, airway obstruction, and glossoptosis; 80%-90% also have a cleft palate. Various treatment approaches exist,...
Robin sequence is characterized by mandibular retrognathia, airway obstruction, and glossoptosis; 80%-90% also have a cleft palate. Various treatment approaches exist, and although controlled studies are rare, objective assessment of treatment outcomes that address the leading clinical issues, namely obstructive sleep apnea and failure to thrive, are essential. Sleep-disordered breathing may be detected using cardiorespiratory polygraphy or polysomnography. Pulse oximetry alone may miss infants with frequent obstructive apneas, yet no intermittent hypoxia. Among conservative treatment options, the Tubingen Palatal Plate with a velar extension shifting the tongue base forward is the only approach that corrects the underlying anatomy and that has undergone appropriate evaluation. Of the surgical treatment options, which are not necessarily the first line of therapy, mandibular distraction osteogenesis (MDO) is effective and has been most extensively adopted. Notwithstanding, it is puzzling that MDO is frequently used in some countries, yet hardly ever in others, despite similar tracheostomy rates. Thus, prospective multicenter studies with side-by-side comparisons aimed at identifying an optimal treatment paradigm for this potentially life-threatening condition are urgently needed.
Topics: Airway Obstruction; Humans; Infant; Osteogenesis, Distraction; Pierre Robin Syndrome; Polysomnography; Prospective Studies; Retrospective Studies; Sleep Apnea Syndromes; Sleep Apnea, Obstructive; Treatment Outcome
PubMed: 33580741
DOI: 10.1002/ppul.25317 -
The Cleft Palate-craniofacial Journal :... Nov 2023To characterize the prevalence and presentation of laryngomalacia and efficacy of supraglottoplasty (SGP) in a cohort of patients with Pierre Robin Sequence (PRS).
OBJECTIVE
To characterize the prevalence and presentation of laryngomalacia and efficacy of supraglottoplasty (SGP) in a cohort of patients with Pierre Robin Sequence (PRS).
DESIGN
Retrospective cohort study.
SETTING
Tertiary-care children's hospital.
PATIENTS, PARTICIPANTS
Consecutive patients with PRS born between January 2010 and June 2018.
MAIN OUTCOME MEASURES
Chart review included demographics, comorbid airway obstruction including laryngomalacia, timing of surgical interventions, clinical symptoms, sleep study data, and modified barium swallow study data.
UNLABELLED
126 patients with PRS were included; 54% had an associated syndrome, 64% had an overt cleft palate, and 22% had a submucous cleft palate. 64/126 were noted to have laryngomalacia (51%). Patients with concurrent PRS and laryngomalacia were significantly more likely to have submucous cleft palate ( = .005) and present with aspiration with cough ( = .01) compared to patients with PRS without laryngomalacia. Patients with concurrent laryngomalacia and PRS showed a significant decrease in apnea-hypopnea index (AHI) and obstructive AHI (OAHI) after mandibular distraction, with a median AHI and OAHI improvement of 22.3 ( = .001) and 19.8 ( = .002), respectively. Patients who underwent only SGP did not show significant improvement in these parameters ( = .112 for AHI, = .064 for OAHI).
UNLABELLED
The prevalence of laryngomalacia in our PRS cohort was 51%. Patients with PRS and laryngomalacia are more likely to present with overt aspiration compared to patients with PRS without laryngomalacia. These data support that laryngomalacia does not appear to be a contraindication to pursuing MDO.
Topics: Child; Humans; Infant; Retrospective Studies; Laryngomalacia; Pierre Robin Syndrome; Prevalence; Cleft Palate; Airway Obstruction; Osteogenesis, Distraction; Treatment Outcome
PubMed: 35668613
DOI: 10.1177/10556656221107298 -
Otolaryngologic Clinics of North America Dec 2000Pierre Robin sequence affects 1:8500 newborns and is characterized by cleft palate, micrognathia, and airway obstruction. Evolution in the understanding of the causes... (Review)
Review
Pierre Robin sequence affects 1:8500 newborns and is characterized by cleft palate, micrognathia, and airway obstruction. Evolution in the understanding of the causes and pathogenesis of this condition is reviewed. Clinical findings and treatment options are also addressed. An overview of syndromes commonly associated with Pierre Robin sequence is provided.
Topics: Airway Obstruction; Humans; Infant, Newborn; Maxillofacial Abnormalities; Pierre Robin Syndrome
PubMed: 11449785
DOI: 10.1016/s0030-6665(05)70279-6 -
The Cleft Palate-craniofacial Journal :... Jan 2021The aim of this study was to compare the dentoskeletal pattern of Treacher Collins syndrome (TCS) and nonsyndromic Pierre Robin sequence (PRS).
OBJECTIVE
The aim of this study was to compare the dentoskeletal pattern of Treacher Collins syndrome (TCS) and nonsyndromic Pierre Robin sequence (PRS).
DESIGN
Retrospective.
SETTING
Single center.
PATIENTS
Eighteen patients diagnosed with TCS (Group TCS) or PRS (Group PRS) in rehabilitation treatment at a single center. Group TCS was composed of 9 patients (4 male, 5 female) with a mean age of 12.9 years (standard deviation = 4.8). Group PRS was composed of 9 patients paired by age and sex with group TCS.
MAIN OUTCOME MEASURE(S)
Cone beam computed tomography-derived cephalometric images taken before the orthodontic or the orthodontic-surgical treatment were analyzed using Dolphin Imaging (Dolphin Imaging 11.0 & Management Solutions). Variables evaluating the cranial base, the maxillary and mandibular skeletal components, maxillomandibular relationship, the vertical components and the dentoalveolar region were measured. Intergroup comparisons were performed using tests. The significance level considered was 5%.
RESULTS
Intergroup differences in the mandible size and growth pattern were observed. Group TCS showed a smaller mandibular length (Co-Go, Co-Gn) and a higher palatal plane (SN-Palatal Plane) and mandibular plane angles (SN-Go.Gn) compared to group PRS. No differences between TCS and PRS were observed for the sagittal position of the maxilla, maxillomandibular relationship, and dental components.
CONCLUSIONS
Treacher Collins syndrome presented a decreased mandible and a more severe vertical growth pattern compared to PRS.
Topics: Cephalometry; Female; Humans; Male; Mandible; Mandibulofacial Dysostosis; Pierre Robin Syndrome; Retrospective Studies
PubMed: 32613853
DOI: 10.1177/1055665620937499 -
The Nigerian Postgraduate Medical... Jun 2007'Pierre Robin' is one of the most readily recognised eponyms in medicine, yet it is a poorly understood nonspecific grouping of malformations that has no prognostic... (Review)
Review
OBJECTIVES
'Pierre Robin' is one of the most readily recognised eponyms in medicine, yet it is a poorly understood nonspecific grouping of malformations that has no prognostic significance. Previously known as 'Pierre Robin syndrome', the way this diagnostic entity is viewed is now undergoing change. It is the purpose of this paper to review previous thinking about Robin and provide an update on recent clinical observations.
SOURCES OF DATA
A computerised literature search using MEDLINE, EMBASE, AJOL and OMIM was conducted for published articles up to March 2006. Mesh phrases used in the search were: Pierre Robin syndrome, Robin anomalad and Robin sequence (RS).
RESULTS
This relatively uncommon association of micrognathia with cleft palate and upper airway obstruction which was initially thought to be a specific disease and entire treatment regimens established to deal with presumed problems is now understood to be a grouping of clinical findings that does not represent a distinct multiple anomaly syndrome. The condition is therefore now described as 'Pierre Robin sequence'. Evidence of distinct cytogenetic anomalies has also highlighted the aetiological heterogeneity associated with RS in recent times.
CONCLUSION
Infants with Robin sequence can present with varied problems, some of them emergencies. Clinicians must be aware of the high prevalence of associated syndromes and the possible contribution of other syndromic features to the problems for proper patient care. Candidate loci and potential candidate genes are currently being proposed in the literature for RS.
Topics: Humans; Infant, Newborn; Pierre Robin Syndrome; Prognosis
PubMed: 17599114
DOI: No ID Found -
Plastic and Reconstructive Surgery Nov 2020
Topics: Airway Obstruction; Humans; Patients; Pierre Robin Syndrome
PubMed: 33136958
DOI: 10.1097/PRS.0000000000007320 -
The Journal of Craniofacial Surgery Sep 2016Tongue-lip adhesion (TLA) involves surgically tethering the tongue forward to the lower lip and is a technique to relieve airway obstruction caused by glossoptosis and... (Review)
Review
BACKGROUND
Tongue-lip adhesion (TLA) involves surgically tethering the tongue forward to the lower lip and is a technique to relieve airway obstruction caused by glossoptosis and retrognathia. The purpose of the present study was to evaluate the effectiveness of TLA for the treatment of airway compromise in patients with Pierre Robin sequence (PRS).
METHODS
A comprehensive literature review was performed. Inclusion criteria consisted of patients having undergone isolated tongue lip adhesion and results that included airway outcome. Selected manuscripts were analyzed with regards to patient demographics, principle diagnosis, pre and postintervention airway status, and complications.
RESULTS
Thirteen manuscripts met inclusion criteria, yielding 268 patients with PRS who underwent TLA. The mean age at the time of procedure was 30.5 days. Tongue lip adhesion proved to be successful in relieving airway obstruction caused by PRS in 81.3% (n = 218) of patients. Nonsyndromic patients benefited from a higher success rate as compared with the syndromic cohort (91.5% and 79.8% respectively, P = 0.0361). Eight patients who were initially successfully managed with TLA required a repeat procedure due to dehiscence.
CONCLUSIONS
Tongue-lip adhesion is a safe and effective technique and is associated with lower morbidity and mortality as compared with mandibular distraction osteogenesis and tracheostomy and should be considered in patients with PRS who fail conservative management. The greater success and lower complication rates in nonsyndromic patients reinforce the importance of proper patient selection and consideration of other techniques such as mandibular distraction osteogenesis should be given in patients with associated syndromic diagnoses.
Topics: Airway Obstruction; Child; Humans; Lip; Osteogenesis, Distraction; Pierre Robin Syndrome; Tongue; Treatment Outcome
PubMed: 27548826
DOI: 10.1097/SCS.0000000000002721