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The proseal laryngeal mask airway with endotracheal tube in situ in the child. The neck contracture can be seen
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Escobar syndrome is a rare autosomal recessive disorder characterized by flexion joint and digit contractures, skin webbing, cleft palate, deformity of spine and cervical spine fusion. Associated difficult airway is mainly due to micrognathia, retrognathia, webbing of neck and limitation of the mouth opening and neck extension. We report a case of...
Citations
... It is characterized by multiple skeletal, genitourinary and orofacial anomalies (1). Contracture bands (pterygium) around the joints is the of cause restricted mobility in these patients which, added to the association with cleft palate, ankyloglossia, syngnathia, micrognathia, restricted mouth opening and spinal anomalies, among others (2,3), constitute a challenge for the anesthesia care team, especially as concerns airway management. There are few reports in the literature and it is worth highlighting this clinical case of consecutive anesthesia interventions in the same patient with evidence of progression of the disease which required an individualized approach to the airway, adaptation of the current algorithms in accordance with the clinical stage of the patient (4,5), and use of devices such as videolaryngoscope and laryngeal mask (LMA) for intubation, not previously described in patients with this pathology. ...
... Regarding the association between this syndrome and malignant hyperthermia, there is one study on the use of halothane (12); however, in all other cases described, halogenated agents were used, with no adverse events reported (2,13,14). Also, no adverse events associated with the use of neuromuscular blockers have been reported (3,15). ...
Escobar syndrome is a rare, autosomal recessive disease of unknown incidence. It is characterized by multiple skeletal, genitourinary and orofacial abnormalities. The multiple malformations (mainly orofacial) and restricted mobility of these patients pose a challenge to the anesthesia team, especially as regards airway management. We describe the clinical case of a pediatric patient diagnosed with Escobar syndrome who underwent two consecutive anesthesia interventions, with evidence of progressive airway anomalies that characterize this syndrome. The case required adaptation, according to the clinical stage of the disease, of the current algorithms used to approach an anticipated difficult airway in pediatrics, and the incorporation of new devices, not described so far in patients with this pathology, as part of the planning and execution phases.
... 2,10 Previous case reports have also mentioned initial failure with FI and using Proseal LMA as conduit for fibreoptic bronchoscope. 4,5 We used a 3.0 mm nasal ETT as a nasal airway and connected it to oxygen port for oxygen insufflation, which helped in maintaining SpO 2 , while trachea was successfully intubated by fibreoptic bronchoscope using oral route. ...
... 11 However, it has been refuted by subsequent case reports, and the use of volatile agent is considered safe in these patients. 4,8,12 We encountered intraoperative hyperthermia without other signs of malignant hyperthermia, which settled postoperatively in ICU. Patients with ES have mutation in gamma subunit of AChR, that is why some authors have avoided muscle relaxants. ...
Escobar syndrome (ES) is an autosomal recessive disorder characterised by the presence of pterygia in cervical, antecubital and popliteal regions. Anaesthesiologist encounter notable challenges in this syndrome, especially airway management due to associated malformations like cleft lip/ palate, micrognathia, syngnathia, ankyloglossia, neck contracture, cervical spine fusion, limited neck extension and craniofacial dysmorphism. In addition to difficult airway, anaesthesiologist may encounter other perioperative challenges. Here, we report a paediatric patient with ES, who required general anaesthesia for laparoscopic inguinal hernia repair and orchidopexy. Initial attempt with video laryngoscope failed due to inability to visualise epiglottis. Subsequent attempt with fibreoptic bronchoscope also failed due to rapid decrease in oxygen saturation. He was finally intubated with fibreoptic bronchoscope along with oxygen insufflation with a 3 mm internal diameter polyvinylchloride endotracheal tube inserted nasally and connected to oxygen supply. Further perioperative challenges faced were intraoperative hyperthermia and postoperative pneumothorax with mediastinal shift. To the best of our knowledge, this is the first case reporting pneumothorax with mediastinal shift as a post-operative complication and use of oxygen insufflation through nasal tube during fibreoptic intubation in paediatric patient with ES.
... These features limit mouth opening, prevent extension of the neck, displace the tongue posteriorly, and obscure visualization of the pharynx making anesthetic management challenging. [16][17][18][19] Despite these risks, all patients in our series were successfully intubated without anesthetic complications. Although implant-related complications occurred, there were no permanent neurological complications in either cohort. ...
Background:
The aims of this study were to characterize the spinal deformity of patients with Escobar syndrome, describe results of growth-friendly treatments, and compare these results with those of an idiopathic early-onset scoliosis (EOS) cohort to determine whether the axial stiffness in Escobar syndrome limited correction.
Methods:
We used 2 multicenter databases to review the records of 8 patients with EOS associated with Escobar syndrome who had at least 2-year follow-up after initiation of growth-friendly treatment from 1990 to 2016. An idiopathic EOS cohort of 16 patients matched for age at surgery (±1 y), postoperative follow-up (±1 y), and initial curve magnitude (±10 degrees) was identified. A randomized 1:2 matching algorithm was applied (α=0.05).
Results:
In the Escobar group, spinal deformity involved 7 to 13 vertebrae and ranged from no vertebral anomalies in 3 patients to multiple segmentation defects in 6 patients. Mean age at first surgery was 5 years (range, 1.4 to 7.8 y) with a mean follow-up of 7.5 years (range, 4.0 to 10 y). Mean major curve improved from 76 degrees at initial presentation, to 43 degrees at first instrumentation, to 37 degrees at final follow-up (both P<0.001). Mean pelvic obliquity improved from 16 degrees (range, 5 to 31 degrees) preoperatively to 4 degrees (range, 0 to 8 degrees) at final follow-up (P=0.005). There were no differences in the mean percentage of major curve correction between the idiopathic EOS and Escobar groups at the immediate postoperative visit (P=0.743) or final follow-up (P=0.511). There were no differences between the cohorts in T1-S1 height at initial presentation (P=0.129) or in growth per month (P=0.211).
Conclusions:
Multiple congenital fusions and spinal curve deformity are common in Escobar syndrome. Despite large areas of congenital fusion, growth-friendly constructs facilitate spinal growth and improve curve correction. These results are comparable to those in idiopathic EOS.
Level of evidence:
Level III-case-control study.
... Escobar syndrome is a rare but challenging pathology for anesthetist. Among other it involves the management of pediatric difficult airways [6]. Children with Escobar syndrome have contractures of the joints, deformities of the spine and cervical fusion, which makes endotracheal intubation a real challenge especially in the context where children are less compliant to awake intubation [3,[7][8][9]. ...
... Other alternative techniques successfully used for intubation in pediatric patients with craniofacial anomalies are video laryngoscopy, airtraq optical laryngoscope, and glide scope. [8] Mathew et al. [9] also used proseal LMA for ventilation followed by intubation in such patient. ...
Multiple pterygium syndrome (MPS) is a very rare autosomal recessive disorder characterized by flexion of joint and digit contractures, skin webbing, cleft palate, deformity of the spine, and cervical spine fusion. Difficult airway is associated mainly due to micrognathia, retrognathia, webbing of the neck, and limitation of the mouth opening and neck extension. We are reporting a case of a 5-year-old female diagnosed with MPS and exhibiting a bilateral club foot and congenital vertical talus. The patient was posted for manipulation and above the knee casting under general anesthesia.
Escobar sendromu, çeşitli solunumsal ve ortopedik problemlere neden olan nadir görülen otozomal resesif geçişli bir hastalıktır. Bu sendromda anestezistler, servikal füzyon, boyun fleksiyon kontraktürüne bağlı hareket kısıtlılığı, yarık damak, mikrognati, ankiloglossi, kısıtlı ağız açıklığı gibi anomalilere bağlı olarak hava yolu yönetiminde önemli zorluklarla karşılaşabilmektedir. Anestezistler zor hava yoluna ek olarak, operasyon sırasında anestezinin korkulan komplikasyonu olan malign hipertermi riski gibi bir sorunla karşılaşabilmektedir. Bu vakada femur şaft fraktürü nedeniyle operasyon odasına alınan bir pediatrik hastanın anestezi yönetimini sunduk. Hastamızda zor entübasyona yol açabilecek bulguların olması sebebiyle ilk denememizi video laringoskop (VL) ile yaptık ve başarılı olduk. Perioperatif herhangi bir problemle karşılaşmadık.
The American Society of Anesthesiologists; All India Difficult Airway Association; European Airway Management Society; European Society of Anaesthesiology and Intensive Care; Italian Society of Anesthesiology, Analgesia, Resuscitation and Intensive Care, Learning, Teaching and Investigation Difficult Airway Group; Society for Airway Management; Society for Ambulatory Anesthesia; Society for Head and Neck Anesthesia; Society for Pediatric Anesthesia; Society of Critical Care Anesthesiologists; and the Trauma Anesthesiology Society present an updated report of the Practice Guidelines for Management of the Difficult Airway.
We report a case of a pregnant woman with multiple pterygium syndrome who presented for elective caesarean section. Neuraxial anaesthesia failed and the backup plan of awake intubation was extremely difficult.
