Congenital bilateral abseneeof the, asdeferen (CBAVD) is an important cause of azoospermia and thus male infertility, and is closely associated with cystic fibrosis transmembrane transduction factor (CF) mutations. CBAVD patients account for 1%-2% of the male population. In China, Zhen (1988) analyzed the etiology of azoospermia in 1800 infertile men, and CBAVD patients accounted for 24% of the azoospermia patients. Therefore, CBAVD is a common clinical cause of male infertility, and with the development of male science and assisted reproductive technology, the clinical understanding of this disease has gradually become more comprehensive, and the emergence of ICSI technology has made this type of patients have the hope of fertility, and ICSI (single sperm oocyte plasma microinjection) technology for fertility in 57 patients with CBAVD at the Institute of Reproductive Medicine of Gansu Provincial Maternal and Child Health Hospital has achieved The results were good. 1. Data and methods 1.1 General data Among the infertility patients who visited the outpatient clinic of the Institute of Reproductive Medicine of our hospital from January 2009 to February 2011, there were 278 cases with more than 3 times of centrifugal semen analysis, all of which suggested azoospermia, including 57 patients, aged 25~40 years old, with an average of 31.2 years old. The duration of infertility ranged from 1 to 9 years, with an average of 3.2 years. All specimens were thin and milky white in appearance, with a volume of 0.2-1.0 ml and a pH value of <7.0. The seminal plasma fructose characterization test was negative, and the physical examination was normal for external genital development, with normal male distribution of body hair, testicular size of 13-25 ml, and bilateral epididymal heads could be found. In 42 cases, the caudal epididymis was not retrieved, in 5 cases, the caudal epididymis was not retrieved unilaterally, the vas deferens was not retrieved bilaterally, the blood follicle stimulating hormone (FSH) 4.74±2.17, luteinizing hormone (LH) 3.95±2.63 and testosterone (T) 697.96±125.80 were measured, the spermatophore was small in 3 cases, the echogenic vesicle was not seen in 54 patients, the head of the epididymis was detectable in 57 cases. The epididymal head was detectable in 57 cases, the caudal epididymis was absent bilaterally in 39 cases, unilateral caudal epididymis was absent in 7 patients, and dilated epididymal ducts up to 3.6 mm wide were seen in 43 patients. 1.2 Fine needle epididymal puncture was performed in 57 patients: the patients were placed in supine position, the skin was disinfected, the scrotal skin and spermatic cord were anesthetized with 2% lidocaine, the epididymis was fixed, a small amount of culture fluid was aspirated for puncture with a 5 ml syringe attached to a pediatric scalp needle, and the culture fluid was injected into a sterile culture dish after pressure aspiration and observed under an inverted microscope. If more active spermatozoa are seen, no more puncture will be performed. If no spermatozoa are seen in both epididymis, testicular puncture or biopsy is performed. 1.3 If active spermatozoa are identified by puncture, the couple will undergo all ICSI preoperative tests and then undergo ICSI for fertility. The ICSI cycle protocol was selected according to the age of the female partner, her basal endocrine secretion, and the number of sinus follicles. 42 cases were treated with the long protocol and 15 with the short protocol. 2. The third treatment was proposed. The cumulative pregnancy rate reached 89.47%. 3. Discussion: Congenital bilateral vas deficiency accounts for 1%-2% of male infertility and 10%-20% of patients with obstructive azoospermia [1], and is caused by abnormal development of the vas deferens derived from the Wolffian duct. Its genetic characteristics only attracted attention in the early 1980s, and more and more clinical cases are being recognized due to the improvement of diagnosis. 3.1 Diagnosis of CBAVD: Physical examination, ultrasound examination, semen routine and seminal plasma biochemical assay are effective methods to diagnose CBAVD. Scrotal palpation is the basis and key to diagnose CBAVD. By palpating the scrotum, we can understand the size, texture, position of the testes, whether there are varicocele, whether the head and body of the epididymis are normal, and whether the development and direction of the vas deferens are normal. Ultrasound can determine the size of the testes and epididymis, the development of the kidneys and the seminal vesicle gland. Ultrasound examination commonly shows normal testicular volume bilaterally, partial development of the epididymis, abnormal development of the epididymis unilaterally or bilaterally, and the absence of the epididymal body and tail can be seen. Some patients can be complicated by cysts of the kidneys or unilateral renal insufficiency, and there are also reports of complicated pancreatic cysts and pulmonary dysplasia. Semen routine parameters and seminal plasma biochemical measurements are of great value in the diagnosis of CBAVD. Patients with CBACD often present with low semen volume, less than 1 ml, thin and non-coagulable, milky white, acidic pH value, and low or absent seminal plasma fructose content. 3.2 Etiology and clinical manifestations of CBAVD: Zhou Huigeng reported that the occurrence of this disease is related to the quality of the parents' fertility, and the possibility of the disease occurring in the offspring of mothers who give birth too early, too late or too much, especially if their childbearing age is >35 years old, is greatly increased. CF is a common autosomal recessive disorder in Caucasians with a prevalence of up to 1/2500 and a very low prevalence of about l/80,000 in Caucasians. CF has a variety of clinical manifestations, including obstructive lung disease, pancreatic cysts, unilateral renal agenesis and male vasoductal development. The most common developmental abnormality of the reproductive system in Asians is a mutation in the CFTR gene that causes abnormalities in the Mullerian ducts, and in the mid-embryonic development the renal ducts stop developing or are defective, leading to malformations and defects in the development of the vas deferens, and often accompanied by defects or fibrosis in the seminal vesicle glands, often manifesting as hypoplasia or dysplasia of the epididymal vas deferens and seminal vesicles. Pulmonary disease is more common in Caucasians and often occurs prematurely in the neonatal period. 3.3 Treatment of CBAVD: CBAVD is a kind of congenital sperm duct malformation that cannot be reconstructed, and in the past, the patient’s spouse was usually made pregnant by AID. With the development of assisted reproductive technology, fertility in CAVD patients has become possible. Studies have shown that the testicular spermatogenesis of CAVD patients is mostly normal, therefore, sperm can be extracted from the testes or epididymis of patients for assisted reproductive treatment. The pregnancy rate and high quality embryo rate were higher in our group of 57 patients treated with ICSI than in other patients of the same age group at the same time, which may be related to the fact that the vast majority of this group of patients have purely male factors and the female partner has no other factors affecting pregnancy, in addition, this indicates that for CBAVD patients epididymal sperm has normal function, and compared with ejaculated sperm, sperm in the testis treated with ICSI has higher fertilization rate, There is no statistical difference in the rate of egg cleavage and the rate of available embryos; therefore, ICSI is a good method to solve fertility problems in this group of patients. We can also try to perform diagnostic puncture on the male partner during the female partner’s ovulation, and if the number of sperm in the puncture is high, IUI treatment can be performed first, which will significantly reduce the cost of treatment and avoid the possible damage caused by the ICSI technique if the pregnancy is successful, regardless of the fertility assistance. Due to the high mutation rate of CFTR gene in patients with congenital bilateral vas deferens, the pathogenic mutation gene can be passed on to offspring, so it is necessary to perform genetic diagnosis to avoid passing on the pathogenic gene to the next generation, to do genetic counseling, for genetic risk assessment before intracytoplasmic single sperm injection and embryo implantation, and even to develop gene therapy.