Describe the clinical characteristics of patients with severe microtia and accompanied malformations. Apply the modified Nagata tehnique (using the posterior auricular fascia instead of the temporoparietal fascia flap) for patients with microtia, so the surgery is suitable for Vietnamese people, achieve high results and few complications.
1 INTRODUCTION Microtia is a congenital condition caused by the abnormal development of the auricle during pregnancy to varying degrees, from mildly abnormal structural part of the ear to severe. Microtia greatly affects the aesthetic issue, which can even lead to inferiority complex due to being stigmatized and shunned by people around them. Therefore, these children need to get their ears corrected early to help them integrate and improve their quality of life Currently in the world, there are some methods of ear reconstruction: forming by autologous rib cartilage, shaping by artificial materials or installing artificial ear canal. In that, forming ear flaps with rib cartilage itself is still a reliable method, bringing longlasting aesthetic results However, in Vietnam, there are still not many researches on this method Therefore, we carry out this research project "Research on the results of ear reconstruction surgery by Nagata technique" to: Evaluate the results of ear reconstruction surgery by Nagata technique 1. The urgency At the ENT hospital, every day there are microtia patients to visit, consult and desire surgery so the need for ear reconstruction is great. In the current methods in the world, the making of artificial materials has just begun in Vietnam and prosthetic implant fitting has not been conducted in Vietnam. However, these two methods are still expensive compared to Vietnamese people and also have many disadvantages. Only the method of using rib cartilage itself is most appropriate with the advantage: the rib cartilage itself should not have a risk of graft rejection, the results of longterm stable surgery, relatively high aesthetic We chose the Nagata technique because it has the advantages: the time and the number of surgeries are shortened, the reconstructed ear looks more natural Because ear reconstruction is an extremely complex and sophisticated technique, requires a welltrained and regularly operated surgeon. We also hope that when conducting this topic, it will firstly help Vietnamese doctors to master the technique and surgery on a regular basis to improve the technique, reduce complications and help patients to have good results in terms of aesthetics, improved psychology as well as confidence in life 2. New contributions of the thesis Describe the clinical characteristics of patients with severe microtia and accompanied malformations Apply the modified Nagata tehnique (using the posterior auricular fascia instead of the temporoparietal fascia flap) for patients with microtia, so the surgery is suitable for Vietnamese people, achieve high results and few complications 3. The layout of the thesis: The thesis is presented with 110 pages including: 02 page introduction, 31 pages overview, 20page research objects and methods, 27page research results, 27page discussions, 1page conclusions and 1page proposal The thesis has 33 tables, 03 charts, 12 pictures, including 89 references arranged in the order of appearance in the thesis CHAPTER 1: BACKGROUND 1.1. Embryology and anatomy of the auricle 1.1.1. Embryology The outer ear consists of the auricle and ear canal, developing from the mesenchymal layer of the first and second pharyngeal arch. The auricle is made up of 6 auricular hillocks of His. At the 5th week of pregnancy, 3 hillocks arise from the mandibular arch (His 1,2,3) and the remaining 3 hillocks from the hyoid arch (His 4,5,6) opposite of the first pharyngeal arch Around the 12th week, 6 auricular hillocks converge together to create a defined structure of the ear The aurilce is in the same shape as an adult by about the 18th week. By 3 years it reaches 85% of adult size and the ear cartilage is almost complete by 5 years of age, although it continues to grow until about 9 years old, it reaches adult size. Microtia occurs when there is an abnormal problem during the development of the auricle in the embryonic period 1.1.2. Anatomy 1.1.2.1. Appearance: includes components: the helix, antihelix, tragus, antitragus, scapha, triangular fossa, concha and lobule 1.1.3. Auricular anthropometry The ears are located on either side of the head, related to the temporomandibular joint and the parotid gland in the front, the mastoid bone and the upper temporal region. The auricle is like 2 leaves with the free part opening behind, creating with the mastoid surface an angle of about 20 30º (auricular mastoid angle) Limit of normal auricle + Above: not higher than straight line crossing eyebrows + Lower: not lower than the straight line across the nose + The longitudinal axis of the auricle: is the straight line connecting the highest peak of the auricle to the lowest point of the lobule, this line is usually created with a vertical angle of 15 20º and parallel to the axis of the nose bridge + The anterior ear axis corresponds to the posterior edge of the branch on the mandibule Size of ears: average length of about 65mm long, 35 mm wide, with length / width ratio ≈ 2/1 1.2. Pathology of microtia 1.2.1. Epidemiological characteristics The incidence of microtia: ranges from 0.83 to 4.34 / 10,000 newborns, common among Asians, Pacific Islanders and Hispanic people (Spain and Portugal) Microtia is predominant in men, right ear is more common than left ear Microtia may be isolated, or in combination with other abnormalities, or may be part of the syndrome: OAVS system (OAVS: OculoAuriculoVertebral Spectrum) with the most classic manifestation is congenital Goldenhar syndrome or KlippelFeil deformities 1.2.2. Morphology of microtia 1.2.2.1. Morphological characteristics About 7090% of cases of microtia occur on one side with the prominence in men and more often in the right ear than the left Bilateral microtia: relatively rare with the ratio of about 0.05 ‰ 1.2.2.2. Classification of microtia: There are many ways to classify microtia but the most popular classification is Marx's (1926) He divided microtia into three categories: + Type I: the ear is smaller than normal and still has most of its normal structures (still with external ear canal) + Type II: the ear is missing 12 anatomical units of the ear canal (without earlobe or helix), the external ear canal is blocked or narrow + Type III: the ear structure is only a small part of peanut,, without external ear canal Later, Marx added Type IV: anotia: no auricle In this thesis, we classify III and IV as severe microtia which require total ear reconstruction 1.3. Ear reconstruction surgery Using autologous rib cartilage: featured with techniques of Brent and Nagata Using artificial rib cartilage (MEDPOR or polyethylene) Prosthetic implant 1.3.1 Ear reconstruction with autologous rib cartilage: a gold standard surgery 1.3.1.1. Age of the patient Brent thinks the age suitable for surgery is 6 years old. According to Nagata, the appropriate age is 10 years or older, or when the chest circumference> 60 cm 1.3.1.2. Four stage technique of Brent: Stage 1: Haversting of rib cartilage, constructing the framework, and inserting the framework in the pocket subcutaneously at the reconstructed ear location Stage 2: Lobule transposition Stage 3: Elevation of the reconstructed ear with a skin graft to create the auriculocephalic sulcus Stage 4: Tragal construction, conchal excavation, and simultaneous contralateral otoplasty 1.3.1.3. Two stage technique of Nagata: Stage 1: harvesting of the costal cartilages, fabrication of the three dimensional cartilage framework (3D frame) and the grafting of the 3 D frame to its proper anatomical location + Step 1: Creating an auricle template (similar to Brent technique) + Step 2: Haversting the ipsilateral rib cartilage. Perichondrium is preserved to avoid chest deformity after surgery. The cartilage pieces are sewn together with a special type of steel thread + Step 3: Implanting the framework in the pocket subcutaneously at the reconstructed ear location. + Step 4: Lobule transposition and tragus reconstruction Stage 2: At least 6 months after stage 1 Get the additional piece of cartilage (banked under the thoracic skin during the first stage) Take a free flap from groin with an appropriate size Elevate the framework Place the semilunar cartilage, fixed by the posterior auricular fascia Using a thickness skin graft to cover posterior auricular area * Advantages: 1. The time and the number of surgeries are shortened 2. The reconstructed ears look more natural * Defect: 1. The risk of lobule necrosis is higher (due to the lack of blood vessels). 2. The chest is weak (due to the large number of cartilage taken). 3. The risk of hair loss on the scalp 1.3.1.4. Symptoms Complications at the chest * Early complications Perforation of the pleura: Pneumothorax: Hematoma: caused by occlusion, slipping drainage in the chest * Late complications Thoracic deformity: Bad scars, keloid scars, hypertrophic scars: Complications at the ear * Early complications Necrosis of skin flap covered with framework Hematoma, condensation: caused by occlusion, closed drainage Infection: Cartilage inflammation: causing necrosis, deformed framework , affecting aesthetic results – Ischemia when lobule transposition * Late complications Bad scars, hypertrophic scars, keloid scars Changes in the morphology of the cartilage framework: + Errors in the position (right) of the ear, + Changes in skin color + Change in the size of the framework + Loss of the anatomy details. CHAPTER 2: SUBJECTS AND METHODS OF THE STUDY 2.1. Subjects 2.1.1. Selection criteria: Patients diagnosed with congenital severe microtia (type III, IV according to Marx) underwent ear reconstruction by Nagata technique at ENT Hospital Patient has full participation in 2 stages of surgery Having complete medical records. Follow up after 2nd stage at least 6 months Agree to join the research 2.1.2. Exclusion criteria Patient had surgery at another hospital;. Patients were operated without Nagata technique Patient was not followedup at least once after 2nd stage of surgery for months Patients do not agree to participate in the research. 2.1.3. Sample size: Because microtia is a rare disease, we selected a convenient sample size. In fact, in the 3 years from 2016 to 2019, we screened all 32 eligible patients for the study In which, 15 patients were retrospective and 17 patients were prospective 2.2. Methods 2.2.1. Study design: clinical intervention study, beforeafter control 2.2.3 Research location: Esthetic and Plastic Surgery Department ENT Hospital Research period: within 3 years from 2016 to 2019 2.2.4. The main evaluation parameters 2.2.4.1. Before surgery: age and gender Family history: The position of microtia: 1 or 2 sides, left or right External ear canal: narrow or completely blocked Accompanied malformations: Features of normal ear: Length, width, the distance between helixlateral canthus, the distance of ear from mastoid bone, the auricular mastoid angle Number of surgeries: Hospital stay of each time: Time between 2 stages: 2.2.4.2. After surgery Early and late complications of st stage at the chest, at the location of the reconstructed ear Managing complications Aesthetic results of the ear in various aspects: were evaluated after 4 followup times: 2 times after stage 1, 2 times after stage 2; Each visit is at least 3 months apart. + Location: position, the distance between helixlateral canthus, the distance helix mastoid, the auricular mastoid angle, the ear axis. + Size: length, width + Shape (13 anatomical details) + Other characteristics: ear thickness, skin color, unwanted hair, scar. Satisfaction level of the patient: 2.3. Steps to proceed 2.3.1 Designing samples of research records, collecting data Develop a sample medical record to collect data. Select patients according to the set criteria Explain the patient agrees to participate in the study and sign a commitment to agree to participate in the study Condut patient information collection according to the sample case: Administrative part: recording full name, age, gender, address, phone contact, hospital registration number, research record number to contact and evaluate after surgery For retrospective patients: retest according to the information in the sample medical records at the beginning of the study All retrospective patients have only completed stage 1 of the surgery, so we proceeded to conduct research at stage 2. For prospective patients: presurgery clinical examination: fully record in detail the morphological characteristics of the microtia ear 2.3.2. Planning the surgery: Use a piece of Xray film to draw hightlights key structure of the normal ear: For patients with bilateral microtia, we use a sample ear that matches the face of the patient (sample ears have 3 sizes: big, medium and small) Locate the reconstructed ear: Draw the shape and size of the ear canal to prepare for reconstruct at the position of the microtia ear, mark with an indelible marker pen or pump methylene blue pole at the top and bottom of the ear, the ear axis Locate the donor site at the ipsilateral chest Photographing patients before surgery. 2.3.3. Ear reconstruction by Nagata: includes 2 stages: 2.3.3.1. Stage 1: Create a 3D cartilage framework with the ipsilateral rib cartilage, lobule transposition and tragus reconstruction Step 1: Take rib cartilage: Often use rib cartilage 6, 7, 8, 9 to sculpting cartilage framework. Specifically: take a block of rib cartilage 6, 7 to create the basic frame, take the whole rib cartilage 8.9 to the adjacent section with the rib, preserve the perichondrium Bury the excess cartilage pieces under the chest skin for 2 nd stage. Sculpte the rib cartilage into the details of the normal ear, stitching to fix the details with steel thread Step 3: Create a skin pocket: Redefine the ear landmarks: ear axis, highest and lowest points Create skin pockets by undermining postauricular scalp area to a specified size, not too wide, not too tight. Control the bleeding carefully Step 4: Implant the cartilage framework into the skin pocket: Implant the cartilage framework beneath the skin corresponding to the location of the reconstructed ear that was located in step 1. Turn the earlobes into position and reconstruct the tragus Step 5: Closed drainage close the skin pocket wound bandage: Put 2 closed drains, apply antibiotic light compress Follow up after surgery: + At the chest: Bleeding, hematoma: drainage usually withdrawn after 24 hours; pneumothorax. + At the location of the ear: Keep negative pressure of drains. Evaluate skin color: pink or hematoma, purple, black, necrotic Observe if the main anatomical details are clear, whether the new ear in right place. 2.3.3.2. Stage 2: Elevate the cartilage framework: after 1 st stage at least 6 months. Take a piece of cartilage waiting at 1 st stage. Take a thickness skin graft in the groin area Cut the skin behind on the cartilage framework 5mm from the atrial edge of the cartilage, all the way to the scales behind the ears. Elevate the cartilage framework up and forward, reposition the ear if needed. Place the semilunar cartilage padding on the cartilage frame, cover and fix by posterior auricular fascia, collating so that it is proportional to the opposite side. Fixed stitching of skin grafts on the back of the framework. Fixation with bolster. Follow up after surgery: After surgery, patients are given antibiotics, analgesic, anti imflamation Examining to detect and handle complications: infections, skin flap, regular observation of flap color Bolster is removed after 57 days. 2.3.4. The corrective surgery After 2nd stage surgery, depending on the surgical results on the shape, size and position of the reconstructed ear, there may be corrective surgery for perfection: Correction of hypertrophic or keloid scars. Edit unclear anatomical details, misalignment. Edit the auricular mastoid angle 2.3.4.1. Evaluate general results after surgery Assessing the results of 1st stage surgery: with the following criteria: 10 + At the chest: points: for each of the following criteria: Bleeding requires intervention after surgery; drainage of pleura, necrotic infection with necrotic, nonhealing scar. 1 point: for each of the following criteria: Bleeding must intervene during surgery; pleural suture, edema, no infection 2 points: for each criterion: No bleeding; no punctured pleura, good scars + At the ear: 0 point for each criterion: Closed drainage is lost, open to be sewn or continuous aspiration; hematoma; infected surgical incisions, scarred necrosis; skin flap necrosis > 1cm; chondritis cartilage destruction. point for each criterion: leaked drains must be applied with antibiotic grease; Hematoma requires no intervention; wound without infection, no necrosis; skin flap necrosis 10mm. The difference in ear width is less than the length. This difference is mostly smaller, that is, the reconstructed ear is usually smaller than the good ear. Table 3.18. The angle of the auricular mastoid compared to the healthy side: The angle through examinations is almost unchanged, most only differ below 100. Only 1 patient had a 10200 difference. Table 3.21. The position of the ear examinations: The high and low position of the ear through examinations is unchanged (or only changes below 5mm). Nearly ½ of the ears are same level with the right ear, one fifth of the ears are higher and one third of the ears are lower than the good. Table 3.22. Axis of the reconstructed ear through examinations: On the 1 st and 2nd examinations, the axis is the same: there are 18 ears on the right axis, 7 ears on the front axis and 8 ears on the rear axis. On the 3rd anf 4th examination 3 and 4 the axis was similar and improved: there were 22 right ear axes, 4 ear deviated front axis and 7 ear rear axis deviation Table 3.23. Ear thickness through examinations: Ear thickness is the same across the visits, only 7/33 ear thickness is equivalent to healthy ear, and the rest (26/33 ears) are all thicker than healthy ears. No ears are too thick compared to healthy ears 16 Table 3.24. Skin color: Through examinations, most skin are the same color with the surrounding skin, only 1 ear has a different skin color than the surrounding skin Table 3.25 Unwanted hair condition in the skin flap: 1/3 of the patients have unwanted hair in the skin flap, while 2/3 of the patients have no unwanted hair. In the case of hair flap there are many different levels: hair on the front, the hair is extreme on the helix, more hair, less hair Table 3.26. How to treat unwanted hair in skin flap: Among 11 patients with hair in skin flap, 5 patients did not handle anything, while 6 patients had to have periodic haircuts, none of them had laser hair removal 3.2.12. Characteristics of anatomy details Table 3.27. Frequency of anatomy details N 10 11 12 13 Details Crus of helix Upper 1/3rd of helix Middle 1/3rd of helix Lower 1/3rd of helix Superior and inferior crus of antihelix Middle part of antihelix Antitragus Tragus Lobule Scaphoid fossa Triangle fossa Cymba concha Cavum concha Total details / 33 30 32 31 30 18 24 21 26 32 30 15 12 33 % 90,9 96,9 93,9 90,9 54,5 72,7 63,6 78,8 96,9 90,9 45,4 36,4 100 Comment: Among the 13 anatomical details, the detail observed, which appeared most in all ears was the cavum concha (100%). Details appear at least, less than a quarter of cases are triangular fossa with a frequency of 45.4%. The details with high frequency of succession are: Upper 1/3rd of helix and earlobe (96.9%); Middle 1/3 rd of helix (93.9%); crus of helix, lower 1/3rd of helix , scaphoid fossa (90.9%). The details that appear less are: 78.8% tragus; middle part of antihelix 72.7%; superior and inferior crus of antihelix are opposite to 54.5% 17 Table 3.28. Rating points according to Mohit Sharma n % Score ≤ 5 0 68 12,1 911 23 69,7 ≥12 18,2 Total 33 100 Comment: No ears have less than 5 details There are 4 ears with 6 8 details There are 23/33 ears with 9 11 details And especially there are 6 ear with 1213 details of anatomy 3.2.13. Evaluate the overall results 3.2.13.1. Early results of surgery Table 3.29. Assessing the early results of surgery: The majority of the ear lobes (27/33) have very good results with no complications at the chest and reconstructed ear. There were 6 patients with good results with minor complications of the reconstructed ear or rib cartilage such as pleural perforation, hematoma. No patients had poor results 3.2.13.2. Late results of surgery: Table 3.30. Assess the late outcome of surgery At the chest: Better results at the reconstructed ear. 78,8% achieved good results because there were no patients with chest deformity and beautiful scars; There were 6 patients achieved good results ie there were hypertrophic scars or keloids in the chest area At the reconstructed ear: very good results with patients, patients with good results, 12 patients with satisfactory results And especially 1 patient with poor results 3.2.13.3. Aesthetic results about the position and size of the ears Table 3.31. Evaluating aesthetic results about ear position, size The majority of ears (81.8%) had good location and size results, which were relatively similar and well balanced for healthy ears. Especially 1 ear has very good results. There were 5 ears that achieved results ie either the ear size was not commensurate with the good ear, or the ear position was not in balance with the healthy ear. No ears had poor results 18 3.2.13.4. Aesthetic results on anatomical details: Table 3.32. Evaluating aesthetic results of ear anatomy: 23 out of 33 ears have 9 ÷ 11/13 details of the ear, ie having relatively enough shape of the ear. Having 6 ears with 12/13 details. Only 4 ears have only 6 ÷ 8 details, none has less than 6 details. As a result, the shape of the ear is mainly good and very good (29/33 ears) for 87.9%. No ears have poor results Table 3.33. Satisfaction level of patients: The majority of patients were satisfied and very satisfied with the reconstructed ear, accounting for 78.1% (25/32 patients), only 21.9% of patients were normal with surgical results CHAPTER 4: DISCUSS 4.1. Clinical characteristics of patients with severe microtia 4.1.1. Age In our study, most of patients had surgery mainly between the ages of 1020 years old. This is a very suitable age for Nagata technique because at this time, the rib has grown enough to take the necessary amount of rib cartilage and the quality of rib cartilage has not been reinforced However, there are 5 patients over 20 because they did not have the economic conditions for surgery at a young age Therefore, we recommend that the formation of the ear cartilage with rib cartilage should be done best around the age of 10 According to the 4th International Ear Reconstruction Congress with the participation of 31 surgeons, the age is agreed for surgery is from 810 years old According to Im, 72% of surgeons believe that surgery at a later age will have better aesthetic results According to Li (2018), the standard that patients can have surgery is > 120cm height, chest circumference > 55 cm 4.1.2. Sex All studies of microtia is male predomiant disease except for one study by Zhu (2000) that found no difference between the sexes, it is not explained in detail 4.1.4. Location Microtia is mainly on the right, accounting for 62.5%, only 34.4% has left ear and 1 patient has on both sides. This is also consistent with 19 the clinical characteristics of microtia more commonly on the right than on the left. According to Ly Xuan Quang, among 38 patients with 50% had right ear, 47.4% had left ear and there was 1 patient with both ears, according to Brent, a total of 1200 cases was 58.2% had right ear , 32.4% had left ear and 9.4% had both ears, according to Kawanabe this rate is 65.2% and 34.8 respectively. However, the reason why microtia is more common on the right is that no studies have mentioned it 4.1.6. The malformation and accompanied syndrome There were 19 patients with hemifacial microsomia, accounting for 57.6%, higher than Brent's study of 36.5%, Zhang's study was 44%. Thus, the majority of patients with hemifacial microsomia. This problem poses the need plastic surgegy so that the face is symmetric so that the reconstructed ear can become more balanced. The surgery helps to symmetry the face for better results. This is the expectation of many parents as well as patients, it also affects the level of satisfaction of the patient 4.1.7. Normal ear charateristics Our results are relatively consistent with Vietnamese results in terms of length and width. There are currently no studies evaluating the angle auricular mastoid, the distance between helix lateral canthus, the distance auricular mastoid 4.2. Reconstructed ear results 4.2.1. Number of surgeries The average number of surgeries per patient is 2.75. Most of the following surgeries are corrected for ear details or fistula surgery Although the Nagata technique consists of only 2 stages of surgery, in fact it is only 2 major surgeries. In addition, after 2 surgeries, there will be a number of small corrective surgeries According to Constatine, the average number of surgeries is 4.88 times, higher than our study According to the summary at the International Ear Reconstruction Congress in 2007, 61% of surgeon operated 3 times, 29% of surgeon operated twice for ear reconstruction 4.2.3. The time between 2 stages Over time we have mastered the technique and the first patients to be completely operated by Vietnamese surgeons who have had stage 2 surgery are 6 months 20 According to Ly Xuan Quang, 71.8% of patients had the 2 nd stage after 612 months. According to the 4 th International Ear Reconstruction Congress, 71% of patients had the 2nd stage after 612 months 4.2.4. Complications of surgery 4.2.4.1. Complications at the chest: Early complications: There are 6 cases of pleural perforation, of which most are holes avoids hematoma and congestion so it is necessary to close the skin incision. Three out of 32 patients (9.4%) had a mild infection, which was characterized by a red, inflamed ear ring. Patients who are exchanging or adding antibiotics are stable. According to Firmin (2010) the infection rate was 6/930 cases (0.65%), the cause is usually from the outer ear canal caused by Pseudomonas bacteria. According to Long (2013) this rate is 0.9% However, in our study, only patient had skin flap necrosis (3.0%), According to Firmin (1998), the rate was higher, 13.9%. According to Long (2013), the rate is lower than 0.16% Learning from this complication, we see an important role in creating skin pockets at the location of the microtia Skin pockets should be removed not too thick to avoid seeing the anatomical details but not too thin, which will lead to malnutrition and skin necrosis Late complications: After 2 stages, 2 patients had hypertropic scars and 2 patients with keloid scars. According to Cho (2007), 3 out of 125 patients had keloids in the reconstructed ear. So we can see the bad scarring rate of our study is much higher 4.2.4.3. Complications at the inguinal skin site Early complications: No patients Late complication: the rate of bad scars is relatively high although patients do not have keloid scars. Causes: the inguinal skin is the movement part of the body that makes it difficult for the scar to stabilize during the healing process and can easily lead to bad scars. In addition, this may be related to racial factors, such as Wolfram and Yotsuyanagi's assessment of the risk of bad scars and keloids in which colored people are at higher risk than whites 4.2.5. Reconstructed ear characteristics The average length and width of the reconstructed ear are smaller than the normal side, but the difference is negligible, about 12mm The average angle of the auricular mastoid is 16.7º, which is about 34º smaller than the average angle of the normal ear. In addition, there are patients with different angles from 1020º The cause of the reduction of the auricular mastoid angle over time may be due to: 22 The semilunar cartilage is not thick enough to elevate the ear The high rate of bad scars also causes the skin flap shrink over time To solve this problem we have 2 options: If the right side of the ear is large, the shape of the ear is not too small, you can proceed to adjust the shape of the good side to create a balance Enhance the angle of the ear by strengthening the extra cartilage placed behind the cartilage framework 4.2.6. Compare these indicators with healthy ears through examinations 4.2.6.1. Difference in ear length compared to healthy side: In our study, 60.6% of cases had the difference