Clinical Analysis Of Congenital First Branchial Cleft Anomaly

Background: Congenital first branchial cleft anomaly(CFBCA)is a rare congenital disease that occurs in the head and neck.The disease is caused by the failure of the first branchial arch and the second branchial arch during embryonic development,resulting in residual epithelium and incomplete branchial groove occlusion,accounting for 1%-8% of all cleft palate deformities [1],often In childhood,the main manifestations are the Pochet's triangle(a triangle that between the submental area,the hyoid bone,and the mastoid process),painless mass,repeated swelling and pus,and ear canal overflow liquid,etc[2].Olsen et al [3] divided the first branchial cleft anomaly into three categories according to the characteristics of branchial cleft: cyst type,sinus type,fistula type.Work et al [4] according to histopathological features,the first branchial cleft anomaly is divided into two types:Type I: originate from the ectoderm,is a membranous external auditory canal replication,manifested as a lesion that occurs in the middle of the external auditory canal and often extend to postauricular crease.These lesions are located above the facial nerve,parallel to the external auditory canal,and finally end in a cul-de-sac on a bony plate at the level of the mesotympanum.These lesions under the microscope are mostly cysts,lining the stratified squamous epithelium,forming keratin,no cartilage,with or without skin appendages.Type II: are duplication anomalies of the membranous external auditory canal and pinna.It contains the skin derived from the ectoderm and the cartilage derived from the mesoderm.The lesions are mostly located below the mandibular angle and are mainly sinus.The lesion enters the parotid gland upwards,passing through the lateral,medial or branch of the facial nerve.It may end inferior to the membranous external auditory canal or form a sinus in that location.The middle earis normal.The disease is rare in clinical practice.At present,some hospitals have insufficient understanding of the disease,treatment is not standardized,and the recurrence rate is high.Endoscopic examination,fistula angiography,B-ultrasound,CT,MRI and other examinations help to diagnose the disease.At present,the treatment of the disease is mainly based on surgical removal of the lesion.Because the disease is closely related to the facial nerve,the facial nerve anatomy + the first cleft fistula/cyst resection is advocated [5].If necessary,the ipsilateral parotid gland tissue is partially removed.If the lesion involves the external auditory canal,part of the external auditory canal skin or cartilage should be removed.In recent years,with the gradual recognition of the disease and the full evaluation of the disease before surgery,the treatment of the disease has achieved good results,and the recurrence rate is significantly lower than before.Objective: Retrospective analysis of 86 cases of congenital first cleft anomaly malformation,to explore the characteristics of the first cleft anomaly and its anatomical relationship with the facial nerve and external auditory canal,thereby guiding surgery and reducing complications.Methods: A total of 86 cases of congenital first cleft anomaly were collected from the Department of Otorhinolaryngology Head and Neck Surgery,the First Affiliated Hospital of Zhengzhou University from January 2010 to June 2018.The patient's gender,lesion location,age of onset,duration of disease,clinical symptoms,history of infection and history of incision and drainage,history of surgery,preoperative and postoperative examination results,location of preoperative lesion,intraoperative site were calculated.See the relationship between the lesion and facial nerve,postoperative complications and postoperative follow-up.Furthermore,the characteristics of congenital first cleft anomaly were statistically analyzed,the advantages and disadvantages of preoperative examination,the relationship between fistula or cyst and facial nerve,the choice of surgical plan,and the cause ofpostoperative recurrence.Result: Among the 86 patients with first cleft anomaly,46 were male,40 were female,51 were left(59.30%),and 35 were right(40.70%).There were no bilateral lesions.The age of onset was 1 to 60 years old,and the average age of onset was 16.36 years.The course of disease was from 0.5 months to 408 months,and the average disease duration was 49.12 months.There were 59 cases(68.60%)of minors(less than 18 years old)and 27 cases(31.40%)of adults(greater than or equal to 18 years old).Clinical manifestations were painless mass in 25 cases(29.07%),repeated swelling and painful pus in 61 cases(70.93%),external auditory canal overflow liquid in 8 cases(9.30%).61 cases had previous history of infection,of which 45 cases had previously used incision and drainage to treat infection,up to 10 times of incision drainage,and 1.9 times per case of average incision drainage;5 cases of infected patients with pus culture found golden yellow grapes Cocci;10 cases had the first branchial cleft fistula/cyst ectomy;30 patients underwent ultrasound before surgery,20 underwent CT before surgery,60 underwent MRI before surgery,and 15 underwent otoscopy Examination,8 cases found the inner mouth.According to Olsen classification,27 cases of cyst type were located behind the ear,36 cases of fistula type,23 cases of sinus type,17 cases of lesions located in the mandibular angle and below,and 42 cases around the ear.All patients underwent fistula/sinus and cyst ectomy in the quiescent period of inflammation.In the operation,30 cases of lesions were located in the deep surface or between the facial nerves,among them 11 cases were located in the area below the mandibular angle,19 cases were located in the area above the mandibular angle.There were 2 cases with external auditory canal atresia and hearing loss,1 case with middle ear malformation,and 1 case with small ear deformity.There were 8 cases of postoperative recurrence,of which 6 cases were located in the deep surface or branch of the facial nerve,2 cases were located in the superficial surface of the facial nerve,and there were repeated infections before surgery.There were 5 cases of mild facial paralysis,3 cases of parotid gland leakage,2 cases of external auditory canal stenosis,and 2 cases of wound infection.The postoperative pathological results were consistent with the diagnosis of cleft palate,including 30 cases of squamous epithelium and skin appendage and cartilage tissue,56 cases of squamous epithelium,skin appendage and cartilage tissue,ie Work II type 30 cases,Work I type 56 cases.Conclusion: Congenital first branchial cleft anomaly is rare,often in childhood,more common on the left side than on the right side,almost all occur in the Pochet's triangle(a triangle that between the submental area,the hyoid bone,and the mastoid process)often characterized by painless mass,repeated swelling and pain,pus and ear canal overflow liquid.According to Olsen classification,it can be divided into cyst type,sinus type and fistula type;according to Work type,it can be divided into Work I type and Work II type.Work Type I is mainly cyst type,and Work II type is mainly fistula/sinus type(P?0.05).The lesion of the congenital first branchial cleft anomaly that below the mandibular angle,and the younger congenital first branchial cleft anomaly is more likely to be located deep in the facial nerve or between the branches(P?0.05).The probability of recurrence after surgery of the lesion that located in the deep surface of the facial nerve or between the branches is greater than that of the lesion located in the superficial facial nerve(P?0.05).Complete resection of the fistula or cyst and its surrounding lesions during the quiescent period of inflammation is currently the most effective method for curing congenital first branchial cleft anomaly.According to the location of the lesion and related auxiliary examination,the relationship between the first branchial cleft anomaly and the facial nerve can be predicted,so as to select the appropriate surgical plan.If the cyst or fistula is located outside the parotid gland and it's route shorte,a fusiform incision can be made at the lateral fistula,and the external fistula including the diseased tissue is removed.If the cyst or fistula is deeper and involves the parotid gland,and it's route long,the facial nerve should be routinely dissected,and a facial nerve monitor can be used if necessary to avoid injury to the facial nerve trunk.When the lesion involves theexternal auditory canal,the adjacent external auditory canal and cartilage need to be removed together,and postoperative attention should be paid to prevent external auditory canal stenosis.This will not only completely remove the diseased tissue,but also reduce the risk of facial nerve injury and postoperative recurrence.