ABSTRACT
Objective:To observe the pathological changes of levator palpebrae superiors muscle in patients with different severities of simple congenital ptosis (SCP).Methods:Levator palpebrae superiors muscle specimens from 102 eyes of 68 patients with SCP who received levator palpebrae superiors muscle shortening surgery at Wuhan Aier Hankou Eye Hospital from August 2018 to October 2019 were collected as the observation group.According to the severity of ptosis, the specimens were divided into three groups, coverage ≤4 mm group (n=35), coverage >4 mm to ≤6 mm group (n=30), and coverage >6 mm group (n=37). Fresh levator palpebrae superiors muscle tissues from 8 normal donors in Aier Eye Bank of Wuhan Red Cross were selected as the control group.All specimens were performed with Masson trichrome staining and immunohistochemical staining for α-smooth muscle actin (α-SMA), and ImageJ software was used to measure the collagen fiber area ratio, skeletal muscle fiber area ratio and the integrated absorbance (IA) value of α-SMA.Seventeen specimens (2 from the control group, 5 from coverage ≤4 mm group/coverage >4 mm to ≤6 mm group/coverage >6 mm group) were observed with a transmission electron microscope (TEM). This study protocol adhered to the Declaration of Helsinki and was approved by an Ethics Committee of Wuhan Aier Hankou Eye Hospital (No.HKAIER2018IRB-005-01). All patients and their legal guardians were well informed about the treatment method and the purpose of sampling and voluntarily signed informed consent.Results:Compared with the control group, the skeletal muscle fiber was reduced in number and was in disordered arrangement, and the striation of some muscle fibers disappeared, and hyperplastic fibrous connective tissue was found in intercellular substances in the observation group.The collagen fiber area ratio of the coverage ≤4 mm group, coverage >4 mm to ≤6 mm group, coverage >6 mm group were significantly higher than that of the control group, and the skeletal muscle fiber area ratio of the three groups was significantly lower than that of the control group (all at P<0.008 3). There were more smooth muscle fibers and positive expression of α-SMA found in the specimens of the observation group.The IA value of α-SMA of the coverage ≤4 mm group, coverage >4 mm to ≤6 mm group, coverage >6 mm group was 7 195.28(5 935.69, 14 058.29), 55 584.18(33 861.88, 80 419.32), 166 507.76(119 121.95, 187 890.86), respectively, which were all higher than 5 543.03(4 867.67, 8 312.02) of the control group, among which, there were statistically significant differences between the control group and the coverage >4 mm to ≤6 mm group, coverage >6 mm group (both at P<0.008 3). Abundant organelles and some damaged mitochondria were found in smooth muscle cytoplasm in the observation group with a TEM.But no characteristic structure of smooth muscle cells such as dense patch and dense body was detected.Conclusions:There are abnormal smooth muscle cells in the levator palpebrae superiors muscle of SCP patients, and the dysgenesis of the levator palpebrae superiors muscle may be related to this abnormal muscle cell.
ABSTRACT
Background The simplex congenital blepharoptosis is the common blepharon motor dysfunction disease.Some researches have shown that congenital blepharoptosis is related to the hypoplasia of levator.Objective This study was to investigate the thickness and pathological features of levator palpebrae superioris aponeurosis in congenital blepharoptosis patients.Methods A prospective cohort study was carried out in Anyang Eye Hospital from March 2012 to April 2014.Eighty-five eyes of 56 patients with congenital blepharoptosis were divided into mild (15 eyes), moderate (25 eyes) and severe blepharoptosis (19 eyes) groups, and the fellow eyes of monocular blepharoptosis was used as fellow eye group (26 eyes).Twenty-six eyes of 13 normal subjects were recruited for the normal control group.The thickness of levator aponeurosis was measured by ultrasound biomicroscope (UBM) , and the shifting range of levator aponeurosis was detected by using measuring scale.Levator aponeurosis specimens were collected during the levator palpebrae superioris shortening surgery for the pathological examination.The study was approved by the medical ethics committee of Anyang Eye Hospital, and the patients or their guardian signed the informed consent.Results The thickness of levator aponeurosis was (0.331±0.018), (0.373±0.026), (0.539± 0.023) , (0.557 ± 0.024) and (0.547 ± 0.028) mm in the severe blepharoptosis group, moderate blepharoptosis group,mild blepharoptosis group, normal control group and fellow eye group, respectively, showing a significant difference among them (F =1.681, P =0.043).The thickness values of levator aponeurosis were considerably lower in the severe blepharoptosis group and moderate blepharoptosis group than those in the mild blepharoptosis group,fellow eye group and normal control group (all at P＜0.05) , and the thickness value of levator aponeurosis was significantly reduced in the severe blepharoptosis group compared with the moderate blepharoptosis group (P＜0.05).Pathological examination showed arranging disorder of muscle fibers,hyaline-like degeneration, connective tissue hyperplasia and interruption of endomysium.The number of eyes with severe hyaline-like degeneration and connective tissue hyperplasia was significantly increased in the severe blepharoptosis group than that in the moderate blepharoptosis group or the mild blepharoptosis group, as well as in the moderate blepharoptosis group than that in the mild blepharoptosis group(all at P＜0.01).The adipose cells in muscle in the mild blepharoptosis group, moderate blepharoptosis group and severe blepharoptosis group were (12.35±4.62), (17.58±7.46) and (26.19±10.81)/field,and adipose cells in the severe blepharoptosis group were significantly more than those in the mild and moderate blepharoptosis groups (t =5.60, P =0.00;t =2.71, P =0.01).A significant increase in the adipose cells also was seen in the moderate blepharoptosis group compared with the mild blepharoptosis group (t =2.44, P =0.02).Conclusions UBM can offer accurate thickness data of levator aponeurosis.The combination of thickness data and shifting range measurement of levator aponeurosis is helpful for the evaluation of muscle strength.The development of levator aponeurosis appears to be abnormal in congenital blepharoptosis patients.The histopathological change parallels to the severity of the disease.
ABSTRACT
A blefaroptose é o posicionamento inadequado da pálpebra superior, estando abaixo de sua posição normal na posição primária do olhar, a qual seria 0,5 – 2mm abaixo do limbo superior. Pode causar bloqueio parcial ou completo do campo visual superior, além do comprometimento estético. As causas são categorizadas em congênitas ou adquiridas. É considerada congênita se presente ao nascimento ou diagnosticada no primeiro ano de vida. As principais técnicas utilizadas para o tratamento da ptose congênita são a ressecção da aponeurose do músculo levantador da pálpebra superior (MLPS) e a suspensão frontal. A medida da função do MLPS é o parâmetro mais importante na escolha da técnica cirúrgica. Quando a função é fraca, a suspensão frontal é mais indicada; a ressecção supramáxima do MLPS também pode ser empregada. Acima de 4 ou 5mm de função do MLPS, prefere-se a ressecção da aponeurose. Para a cirurgia de suspensão frontal, vários são os materiais utilizados, portanto apresentamos uma comparação entre os estudos mais relevantes. Discutiremos também particularidades em casos mais complicados, como as Síndromes da Blefarofimose e de Marcus-Gunn, além de técnicas cirúrgicas menos utilizadas e as complicações relatadas.
The blepharoptosis is the improper positioning of the upper eyelid, being below its normal position in primary gaze, which is 0.5 - 2mm below the superior corneal limbus. It may block partially or completely the upper visual field, and lead to aesthetic commitment. The causes are categorized as congenital or acquired. It is considered congenital if present at birth or diagnosed during the first year of life. The main techniques used for the treatment of congenital ptosis are the resection of the levator muscle aponeurosis and the frontalis suspension. The function of the levator muscle is the most important parameter to define the surgical technique. When the function is weak, the frontalis suspension is more appropriate; the supra-maximal resection of the levator muscle may also be employed. With function above 4 or 5mm, the resection of the aponeurosis is preferred. For the frontalis suspension surgery, various materials can be used, so we present a comparison of the most relevant studies. We also discuss some characteristics in more complicated cases, such as the Blepharophimosis syndrome and the Marcus-Gunn syndrome, and surgical techniques less performed and complications reported.
Subject(s)
Humans , Blepharoptosis/surgery , Blepharoptosis/congenital , Blepharophimosis/surgery , Blepharoplasty/methods , Eyelids/abnormalities , Eyelids/surgery , Ophthalmologic Surgical Procedures/methods , Surgical Flaps , Suture Techniques , Oculomotor Muscles/surgeryABSTRACT
Background Congenital blepharoptosis is a common disorder of eyelid movement.Overseas research showed that the most likely mechanism of congenital blepharoptosis is the hypoplasia of levator.But the study on Chinese is still lack.Objective This study was to investigate the pathological features of hypoplasia in levator aponeurosis in Chinese congenital blepharoptosis patients.Methods Twenty-one patients with congenital blepharoptosis were divided into mild group (3 cases),moderate group (14 cases) and severe group (4 cases).Samples of the levator aponeurosis were obtained during the levator palpebrae superioris muscle shortening surgery.Hematoxylin-eosin,special staining and immunohistochemistry were performed to analyze the characteristics of the samples.Normal samples of fresh levator aponeurosis were obtained from the donors in the eye bank of Beijign Tongren Hospital.Results Hematoxylin-eosin staining and special staining showed that with the increase of severity,the cases of levator fibers sparse,fibrous tissue hyperplasia and endomysium defect were gradually increased,showing significant differences among the different groups (Z =-0.702,P =0.002 ; Z =0.738,P ＜ 0.001 ; Z =0.746,P ＜ 0.001).Four samples (19％) presented with adipose in the interstitial tissue.Immunohistochemistry showed that the expression of muscle proteins myosin was weaker in the levator aponeurosis of patients with congenital blepharoptosis than that in the normal samples,and the expression intensity of collagen type Ⅲ in the samples enhanced in comparison with the normal samples.However,there were no significant differences in the expression of actin,myoglobin,fibronection,collagen type Ⅵ and laminin among various groups (all at P＞0.05).Conclusions The levator aponeurosis appears developing abnormality in Chinese patients with congenital blepharoptosis.The histopathological change degree is parallel with the severity of congenital blepharoptosis.