Pierre Robin sequenced a case in which a child with a row of jaw bones was towed into osteoporotic difficulty in tract treatment.

The child, female, 2 years 4 months old, 10 kg, was admitted to hospital for "discovering a cleft palate for more than 2 years".
After birth due to jaw contraction, upper palate cleft palate with breathing difficulties, drinking milk cough, diagnosed as "Pierre Robin sequence (Pierre Robin sequence, PRS), cleft palate", three months after birth line "double-sided jaw extender implantation (also known as jaw bone traction into osteopatic surgery)."
recovery after surgery, 6 months when the line "two-sided jaw extender removal surgery."
children are lagging behind their peers in growth and development.
admission diagnosis: cleft palate, after PRS surgery.
is intended to be repaired under general anaesthetic for cleft palate.
1d before surgery when the child went out to play did not see, parents complained that the child sleeps like side-lying, there is a slight snoring, diet in general, chest X-rays did not see obvious abnormalities, laboratory examination did not see obvious abnormalities.
the next day when the child entered the operating room found that the double-sided jaw is still retractable obvious, the jaw archaic scarring, small opening, only about 1.5 cm, caries, some teeth fall off, the right jaw teeth have loose, not clear pronunciation.
children in the room after intravenous injections of suffolk 2 sg and propofol 30 mg, mask breathable chest ups and downs well, through the mouth into the visual laryngeal mirror 2 times failed.
Deepening anesthesia, inhalation of heptafluoroether and intravenous injection of amber choline 15mg, visual laryngeal mirror barely entered the mouth, adjust the position of the laryngeal mirror but will not be able to show, through the laryngeal cover visual intrinsic hose guide the lower nasal intrinsic tube.
After the child's self-breathing recovery is good, compound propofol and heptofluoroether deepen anaesthetic, first insert the gas inside the empty cover 2.0 single tube throat cover, auxiliary breathing is good, through the throat cover inserted 2.8mm visual intestation hose, the sound door is clearly visible.
Exit the visual intrication hose, the lubricated 4.5-bagless catheter sleeve is inserted into the larynx on the visual intrication hose, and under the guidance of the soft mirror, the catheter is fed into the main air duct, assisted breathing chest ups and downs well, double lung stethoscope breathing Sound symmetrical, exhalation end carbon dioxide waveform is good, in the process of children HR110 to 145 times / minute, BP92 to 103 /48 to 56mmHg, SpO2 90% to 100%.
gradually cut the laryngeal cover 3 times and take out in turn, adjust the depth of the catheter, again listen to two lungs after good breathing properly fixed catheter.
propofol and rifentanyl micro-pump maintenance anaesthetic, surgery length 1h, in-surgery vital signs stable, PETCO242 to 48mmHg, no blood transfusion, surgical action pulse gas analysis pH7.307, PCO2 47.6mmHg, PO2 188.8mmHg g, BE-3.1mmol/L, Hb104g/L, Na-137.5mmol/L, K-3.78mmol/L, Ca2-1.22mmol/L, Cl-106mmol/L, AnGap11.9mmol/L.
the ICU after surgery, after the operation 48h pull tube, after the tube the next sunrise ICU, follow-up did not see abnormalities, 5d after rehabilitation discharged from hospital.
Discussion of PRS is mainly manifested in the development of the jaw bone after contraction, post-tongue fall and path obstruction, etc. , 58% to 90% of the children with cleft palate or high bow, at the same time can be combined with heart, eye and ear abnormalities.
children with mild PRS have no clinical manifestations, heavy patients can appear after birth to varying degrees of breathing and feeding difficulties, intellectual disabilities, recurrent lung infections, asphyxia, etc. , if not treated in a timely manner can cause neonatal death.
PRS has a variety of treatments, including the use of assisted feeding tools, to maintain forward or submerged position, placement of mouth or nasopharyngeal channels, lip and tongue adhesion, tracheotomy and jaw bone traction into osteopasis.
Jaws Traction Osteopolytic is the application of certain instruments to accelerate the growth of the jaw bone in the case of amputation of the jawbone, in order to improve breathing difficulties, feeding difficulties, while improving the small jaw malformation.
However, the application of this method in newborns also has certain injuries and potential risks, common complications include: incision infection, facial cellular tissue inflammation, sensory abnormalities, facial scarring caused by external fixation devices, etc.;
After two previous operations, the respiratory and nutritional conditions of the children in this case were improved, but the recovery of the jaw malformation correction was not good, and when the 2nd intake of internal fixed surgery anaesthetic, the pathogenic treatment was still difficult, considering the associated arthritis of the jaw.
the small opening degree consideration is related to the stiffness of the jaw joint.
The only chest X-ray examination before surgery did not indicate positive signs, there were no other relevant imaging examinations for special conditions of the air channel, preoperative visits did not see the child, it is not clear the air channel condition, these have brought great challenges to the anaesthetic management of cleft palate repair surgery.
The child has loose teeth, small open mouth, although no mask breathing difficulties, in the attempt to visual laryngeal mirror under the tube intrinsic failure, taking into account the visual intrinsic hose is thin, the oral presence of secretions, poor clarity, affecting the success rate of intestation, then switched to the hood visual intrinsic hose guide the lower tube intrinsic tube.
The successful insertion of the gas in the body of the hood No. 2 throat cover, good breathing, through the cover inserted lubricated soft mirror, will be tired of the sound door clearly visible, limited by the larynx, soft mirror model and the development of children, can only be put into the lubricated 4.5 no cystic tube catheter, although there is a certain leakage, but PETCO2 in the acceptable range, blood gas analysis results also support this point.
because there is no special exit laryngeal device, decided to segment the slit the laryngeal cover and take out in stages, in this process pay attention to the depth and position of the catheter to prevent slip.
after surgery, the child had the catheter removed in a state of full sobriety, and there were no abnormalities.
studies have shown that the risk of airway obstruction after cleft palate repair in children with PRS is significantly higher than that of simple cleft palate, mainly due to the high rate of airway obstruction in PRS disease itself, and the importance of perinatural monitoring is emphasized.
risk of cleft palate repair periosis in children with low-age PRS is mainly severe hypoxemia.
but there have been few reports of the risk of anaesthetic surgery for cleft palate surgery after PRS surgery to correct a malformation in the jaw.
In this case, the anesthesiologist subjectively believes that PRS line jaw bone traction into bone surgery treatment, jaw malformation will be corrected, trachea symptoms will be significantly improved, also did not consult the past medical history, coupled with the relatively large age of the child, the risk is reduced, so ignored the comprehensive assessment of the tract condition, and the operating room also did not carry out an imaging examination of the pathway, to the child's safety brought great danger.
Therefore, when performing surgery, children with PRS should improve the head and neck imaging examination, make accurate assessment of the air channel condition, and prepare the relevant equipment and drugs according to the difficult pathway treatment process.
therefore, the preoperative evaluation of children with PRS and the formulation of more rational and individualized anesthesia programmes should be further improved in the future.
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