Goldenhar syndrome combined with anaesthetic in ophthalmological surgery in children with laryne cartilage development

Sick children, male, 3 months old, 4kg, because of "discovery of left eye corneal swelling and right eye conjunctiva swelling 3 months" hospital, after the birth of the child to see the double eye swelling, double-eye swelling with the child growing up and increase, outpatientdiagnosis: corneal skin-like cyst, recommended corneal transplant surgery treatmentAfter the birth of the child due to breathing difficulties, haddiagnosiscongenital laryngric cartilage development is not fully developed, did not give special treatmentCheck body: clear consciousness, jaw bone development is incomplete, full cross-sectional, two-sided ear, heart and lung body check no special, limb spine no specialThere is nothing special about laboratory examinationsspecialist body: right eye swelling and corneal edge, left eyelid closure is not complete, the upper eyelid inner edge of the eyelid edge irregular defect, the upper skin-like tissue under the tibia protrusion protrusion conjoined conjunctiva surface, covering about 4/5 cornea under the tibiaThis proposed full hemp downstream cornea transplantAfter the child entered the room crying, it can be seen that the obvious inhalation of three concave signs, accompanied by the throat wheezing soundConventional placement of electrocardiogram, HR162 times/min, RR45 times/min, SpO2 100%, no venous accessGive 6% heptafluoroetheration pre-flush breathing circuit after retaining autonomous breathing mask inhalation induction, wait for the child's consciousness to disappear, the establishment of vein pathways, at this time HR139 times / minute, RR24 times / minute, VT35 to 40 ml;to improve the venous pathway to give midazolam 0.4mg, Atropine 0.08mg, fentanyl 5 sg, retain the child's self-breathing under place diren 1.0 ordinary laryngeal cover, autonomous breathing mode, HR146 times / minute, RR25 times / minute, VT38 to 45 ml, surgery to be 3% seven fluee ether maintenance, surgery at the beginning of the occa drop-off Liquid eye drops, the life signs of children in surgery is stable, throat mask ventilation satisfaction, surgery lasted 127min, surgery ended with heptafluoroetheration, 5min after the child sober removal of the larynging cover, remove the larynx after the child's quiet rest, HR139 times / minute, SpO2 100%, RR28 times / minute, mild inhalation three concave signs, safe transfer to PACU, after surgery, 2 children discharged from the hospitalAfter 1 week of ophthalmology clinic return visit, the child consciousness is clear, quiet rest, no obvious inhalation three concave signs, no anesthesia-related complicationsdiscuss Goldenhar syndrome syndrome (GS), a birth defect characterized by abnormalities in the eye, ears, face and spine early in the of embryos, including facial abnormalities, ear abnormalities, eye abnormalities, spinal developmental defects and congenital heart disease, and in some cases abnormal endocrine hormone levels Children with Goldenhar syndrome tend to combine the jaw systwtos, irregular dental column, high arch of the upper palate, hanging and cracking, these facial signs all indicate the risk of difficult airways, even as the child's age, difficult airways will become more and more serious some scholars suggested that patients with abnormal developmental jaws were diagnosed with a sensitivity of 100% and 96% specificity of their laryngosal exposure difficulties Throat cartilage development is not all the main occurrence of larynx wheezing in infancy, the current pathogenesis mechanism is not clear, may be due to larynx cartilage stunting, softening, inhalation of the upper soft tissue collapsed inwards, blocking the mouth of the throat cavity, making the throat volume become smaller; the child in this case is Goldenhar syndrome combined with congenital laryngeal cartilage development Before surgery, it can be seen that there are obvious signs of dysplasia in the jaw, cross-sectional facial fracture, throat wheezing, inhalation of three concave signs, etc The above signs indicate that there is the possibility of intubation, inability to breathe, collapse of the airway after surgery and re-intubation during anesthesia induction and resuscitation And the child is young, can not cooperate with the sobriety bureau hemp intubation, so the retention of autonomous breathing anesthesia induction should be the first choice Generally using heptatlyronootherther for induction, inhalation concentration of 6% to 8%, newborns and low-weight children can use the concentration of increasing method, in providing adequate sedation at the same time, can be good to retain autonomous breathing, while can evaluate whether there is ventilation difficulties Compared with the trachea intubation that retains autonomous respiration, the total hemp of the throat cover that retains the self-breathing is small in the airway stimulation, the damage is small, does not affect the motor tube epithelial hair movement, the incidence of infection in the lung after surgery is low, and reduces the risk of postoperative ejaduct edema, emphatic collapse and re-intubation At the same time retain ingenual breathing of the throat cover full hemp has a good tolerance, compared with tracheal intubation, can be relatively reduced induction and inoperative use of narcotic drugs Therefore, for such non-essential trachea intubation surgery, it is considered to retain the self-breathing underthetoma, in the guarantee of good ventilation at the same time, reduce the difficulty of artificial airway establishment and the incidence of various complications after surgery for those who must carry trachea intubation, do not muscle loose medicine before intubation, intubation should pay attention to such children's sound door exposure is not good, intubation difficult, pre-intubation preparation should be adequate, and should be operated by skilled people When anaesthetic resuscitation, taking into account the presence of respiratory dysmorphia in children should be highly alert to the removal of trachea ducts after the airway collapse, emphysema caused by the possibility of unable to breathe, wheezing, should be strictly grasp the timing and signs of the tube The of Airway management in children with Goldernhar syndrome is a top priority for the management of the entire anaesthetic , and choosing the right airway management plan can reduce the complications associated with anesthesia while meeting the needs of ventilation and surgery In addition, individualized anaesthetic programmes should be developed that take into account co-existing diseases