There are two types of testicular syringomyelia: primary and secondary. In primary cases, the etiology is unclear, the course of the disease is slow, and chronic inflammatory reaction of the sphincter is common on pathological examination. Secondary cases are associated with primary diseases such as orchitis, epididymitis, trauma or systemic diseases such as hyperthermia and heart failure. In chronic cases, there is no obvious cause, sometimes it can be seen after chronic injury to the scrotum or local surgery such as lymph or vein removal in the inguinal region, or it can be complicated by certain diseases in the scrotum, such as tumor, tuberculosis, syphilis, etc. In the tropics and in southern China, filariasis and schistosomiasis can also cause syringomyelia. Infantile syringomyelia is associated with delayed development of the lymphatic system.
Classification
Testicular syringomyelia: The sphincter closes normally and fluid is formed in the intrinsic sheath of the testis, which is the most common type.
Spermatic cord sheath effusion: the two ends of the sphincter are closed, but the middle part is not closed and there is fluid accumulation. The fluid accumulation in the sac does not communicate with the abdominal cavity and the testicular sheath cavity, also called spermatic cord cyst. The cyst that occurs in girls is called Nuck cyst or round ligament cyst.
Mixed type: Testicular and spermatic sphincter effusions are present at the same time, but do not communicate with each other.
Traffic syringomyelia: Due to the late closure of the sphincter, the fluid in the testicular sphincter cavity may connect to the abdominal cavity through a small tube. If the channel between the sphincter and the abdominal cavity is large, the intestinal tube and omentum may also enter the sphincter cavity, which is known as congenital inguinal hernia.
Testicular and spermatic cord sheath effusion (infantile type): the sheath is only closed at the inner ring, the spermatic cord is not closed, and the effusion communicates with the testicular sheath cavity.
Pathology
Primary syringomyelia is mostly a pale yellow, clear fluid, which is exudate. Secondary acute syringomyelia is cloudy and may be celiac, light red or brownish red, and may be purulent when inflammation is severe. The sphincter wall is often fibrous and thickened and calcified. The testes may atrophy due to prolonged compression.
Epidemiology
Under normal conditions, the testicular sheath contains a small amount of fluid, which can be absorbed at a constant rate through the internal spermatic veins and lymphatic system. When the sheath itself or the testes or epididymis become diseased, the secretion of fluid increases or absorption decreases, and the sheath sac accumulates more than the normal amount of fluid and forms a cyst, it is called syringomyelia.
Syringomyelia can occur in all age groups, and the 2009 European Urology Guidelines report that neonatal syringomyelia accounts for 80% – 94% of full-term male infants. As the syringomyelia matures with age, 90% of congenital syringomyelia is often absorbed within 12-24 months; in adults 1% have syringomyelia. In a survey analysis involving 2782 children aged O-7 years with birth defects in China, syringomyelia ranked third. Syringomyelia is usually unilateral, with bilateral syringomyelia accounting for 7% – 10% of cases.
Clinical manifestations
Symptoms: The main manifestation is a cystic mass in the scrotum or groin area. A small amount of syringomyelia has no uncomfortable symptoms and is often discovered by chance during physical examination; those with a larger amount of fluid often feel scrotal sagging, swelling, and spermatic cord traction pain. In cases of large testicular syringomyelia, the penis shrinks into the foreskin, affecting urination and sexual life, and making walking and labor inconvenient. Traffic syringomyelia, scrotal enlargement when standing. When the scrotum is held up after lying down, the fluid gradually flows into the abdominal cavity and the cyst shrinks or disappears.
Physical signs.
1.Visual: the swelling of testicular sphincter is located in the scrotum, oval or pear-shaped, and the skin may be blue; spermatic sphincter is located in the groin or above the testes, with obvious demarcation from the testes; in case of traffic sphincter, the effusion sac may shrink or disappear when lying down.
2. Touch: testicular syringomyelia is soft, elastic and cystic, and the testes and epididymis cannot be touched. Spermatic sphincter effusion can be moved and the testes and epididymis can be touched underneath. Traffic syringomyelia can shrink or disappear by squeezing the effusion sac.
Examination.
1.Transillumination test is positive, but may be negative in case of secondary inflammatory bleeding.
2. Ultrasound examination can further clarify the diagnosis, and is important for secondary testicular syringomyelia suspected to be caused by testicular tumor, etc.
Non-surgical treatment.
1.Follow-up observation: It is suitable for those who have a slow course of disease, little fluid, little tension and no growth for a long time, and no obvious symptoms. Children with syringomyelia before 2 years old can often absorb it on their own and do not need surgery.
2.Conservative treatment: After successful treatment for the primary disease, syringomyelia can often subside on its own without surgery.
Surgical treatment
1.Surgical indications
①Syringomyelia in infants under 2 years of age can generally be self-absorbed, but when the volume of fluid is large and there is no obvious self-absorption, surgery is required.
②Patients over 2 years old with traffic syringomyelia or larger testicular syringomyelia with clinical symptoms affecting the quality of life should be treated surgically. However, syringomyelia caused by epididymitis and testicular torsion should be excluded.
2.The main surgical methods for testicular syringomyelia.
Surgery is the most safe and reliable method to treat testicular syringomyelia. The surgical methods are
① Testicular syringomyelia reversal: the most commonly used surgical method, easy to operate, good results. Especially for patients with small amount of testicular sphingomyelia and no significant thickening of the sphincter.
② testicular sheath folding: it is suitable for those with thin sheath and no complications. The advantages are simple operation and few complications.
③Sphincterotomy: a common clinical procedure, mainly for patients with significant thickening of the sheath. Because almost all of the sphincter is removed, there is little chance of recurrence.
④Transportation syringomyelia: an oblique inguinal incision is often used to cut and suture the sphincter at a high level at the inner ring, while extruding the testis and the sheath from the incision and performing syringotomy or syringotomy. In recent years, with the development of laparoscopic techniques, the use of laparoscopy for the treatment of traffic syringomyelia has become more and more sophisticated. Due to the local magnification of laparoscopy, the blood vessels of the internal loop can be clearly identified, and damage to the spermatic vessels and vas deferens can be avoided during suturing; postoperative complications are few, pain is mild, hospital stay is short, and there are no obvious scars.
The spermatic cord syringomyelia should be peeled off and the cyst removed completely.
Complications of surgery: mainly bleeding, infection, edema, vas deferens injury and testicular atrophy and infertility caused by damage to the spermatic artery.
Follow-up: The main purpose of follow-up is to check for recurrence and to check sperm quality if there is infertility and to exclude intraoperative damage to the spermatic cord.