The Management Of Perianesthesia In Pediatric Patients With Hereditary Epidermolysis Bullosa

Research backgroundThe Inherited epidermolysis bullosa is caused by the mutation of COL7A1encoding the gene collagen type?,leading the change,lack or absence of dermal collagen fibers,increasing the brittleness of the skin and mucous membranes.After being affected by external forces,it is characterized by the appearance of large erosions in the corresponding parts.The skin mucous membranes are characterized by mechanical stress and spasm.There are different genetic characteristics and clinical manifestations of autoimmune hereditary diseases.The onset of EB for many years can be manifested as extensive cutaneous mucosal lesions involving the various systems of the body.The clinical manifestations are complex.It can involve the hands,feet,elbows,knees,eyes,oral mucosa,teeth,esophagus,intestinal tract,genitourinary tract,muscles,skeletal system and so on.EB subtypes follow the autosomal dominant inheritance/recessive inheritance pattern,and have nothing to do with gender,age,or ethnicity.Their performance is closely related to their clinical classification,often manifesting as family clustering.The disease is rare with it's incidence rate from 2 to 5 in 100000 worldwide.At present,EB is divided into four types clinically,involving epidermolysis bullosa simplex?EBS?,junctional epidermolysis bullosa?JEB?,dominant dystrophic epidermolysis bullosa?DEB?/recessive dystrophic epidermolysis bullosa?RDEB?,and Klindler syndrome.Each subgroup contains different subtypes and the clinical manifestations and incidence of each subtype are different,among which EBS is the most common and RDEB is the most serious.There are relatively more research on EB in dermatology,orthopaedics,pathology,genomics,and nursing currently,but the content of anesthetic management is rare,especially in China.Tissues covered by skin and mucous membranes may be affected.The clinical symptoms of EB are similar to burn wounds,if handled improperly,the areas will be infected.We should control the infection actively,promoting wound heal.Surgical treatment often requires anesthesia intervention,and intubation can easily lead to airway fistula,blocking airway,forming difficult airways,and threatening life.Therefore,EB anesthesia management is very challenging.Our hospital participated in the Butterfly Baby Charity Organization.By recruiting the EB patients in the country and providing operation for them in the public welfare unit,we have obtained some experience and advantages in the anesthesia management of EB patients who have undergone two-handed adhesion contracture and lysis.Objection1.To investigate the safety and feasibility of propofol-remifentanil-ketamine in the perianesthesia of children with EB.2.To summarize the experience of anesthetic management,postoperative pain,itching and other complications of EB patients during perianesthesia in order to provide reference for perioperative anesthesia in EB children.Methods25 ASA?-?patients with Inherited epidermolysis bullosa?EB 10 male 15female?,aged 3-16 yr,undergoing lysis and reconstructive surgery of both hands adhesions,from July 2015 to April 2017 in our hospital.Inclusion criteria:severe and abnormal EB patients;without significant heart,lung,liver,and renal dysfunction.Exclusion criteria:Severe underlying disease,excessive weight loss,severe anaemia,malnutrition uncorrected.The study had been approved by the Medical Ethics Committee of our hospital and asked his or her family to sign an informed consent for anesthesia.Strictly ban drink and food preoperative,with full preparation of gastrointestinal,intramuscular injection of ketamine 4-6mg.kg^-1 during preoperative with atropine0.01mg.kg^-1.Monitor ECG,HR,MAP,SpO2 of the patients with iPM12Multifunctional Monitor?Shenzhen Mindray China?.Connect the pediatric respiratory pipeline with WATO EX-55 anesthesia machine?Shenzhen Minray Co.,China?,set oxygen flow 2L.min^-1.The sphygmomanometer cuff is tied to the lower lambs in less skin lesions,under which covering with sterile gauze.The child was connected with a pulse oximetry probe on the back of the patient's foot and wrapped in sterile gauze with appropriate tightness.Connecting the ECG Electrode Pads with Mepitel?self-adhesive silicone dressing Mepitac Canada?,cutting off the sheet around it,remove the adhesive,and stick it to the area with no or less skin lesions.Apply erythromycin ointment on cornea to protect eyes.Open the central venous access under basic anesthesia.The deep venous catheter was fixed with a suture and covered with a sterile bandage at the puncture site.The sterile foil was prevented.Intravenous injection of tropisetron 0.2 mg.kg^-1 before anesthesia.Place the pediatric anesthesia mask over the patient's nose and mouth?with the mask undirectly touch the patient's face?,inject midazolam 0.08mg.kg^-1,ketamine 2mg.kg^-1 through intravenous to induce anesthesia.Pumping propofol 5mg.kg-1.min-1,remifentanil0.05-0.08ug.kg^-1.min^-11 with micropump to maintain anesthesia.Adjust the pumping rate of anesthetic according to the patient's condition during the operation,infuse sodium,potassium,magnesium,calcium and glucose injection to maintain the circulation stability.When physical activity appeared,give ketamine 1-2mg.kg^-1through injection.Observe the patient's breathing closely and immediately initiate mask ventilation if respiratory depression occurred.Protect the face with"beauty epidermis"when the mask contacts the skin,remove the oral secretions when the patients awakened.Observe and record systolic and diastolic blood pressure of the patients at respective times--before surgery?T1?,1 minute after surgery?T2?,30 minutes?T3?after the start of surgery,90 minutes?T4?,and at the end of surgery?T5?.Measure and record the Birmingham severity score at T1 and FACES pain rating scale?Wrong-Baker pain rating scale?before surgery?T0?,24 hours?T24h?after surgery,48 hours?T48h?postoperative,and 72 hours?T72h?after surgery.Record the operative time,fluid volume,blood loss,minimum SpO2,occurrence of respiratory depression,recovery time,postoperative examination for new bullae and skin lesions.The obtained data were processed by SPSS 22.0 statistical software,expressed as mean±standard deviation????±s?.Using one-way ANOVA t-test for repeated data,take?=0.05 as the level of inspection.Use GraphPad Prism6.0 software to mapping.Results1.There was no significant fluctuation in blood pressure and heart rate during surgery,and no serious cardiovascular events occurred.2.There were 5 cases respiratory depression occurred intraoperation,and 3 cases of new bullus occured at the end of the surgery.3.FACES pain scores of patients at T8h,T24h,T48h,T72h compared with T0,p<0.05,T72h compared T8h,p<0.05,T72h compared T48h,p<0.05,the differences were statistically significant.ConclusionThe management for pediatric patients with hereditary epidermolysis bullosa undergoing lysis and reconstructive surgery of both hands adhesions by using propofol-remifentanil-ketamine was safe and feasible.