Sacral-coccygeal teratoma in children, treatment

May 31, 2018 00:15 | Proctology
The sacrococcygeal teratoma has a mixed structure and is classified as as the organismoid teratoma , or of the embryo. It consists of various tissues that are derived from all three primary embryonic sheets - ectoderm, mesoderm and endoderm. In 100% teratomas, ectodermal elements are found, in 90% - mesodermal and in 70% - endodermal. A tumor can contain skin, hair, cartilage, bones and even the rudiments of individual organs. The sacrococcygeal teratomas are divided into benign and malignant. However, even in benign teratomas there are immature tissues of the embryonic type, which threatens their malignancy.

Clinic and diagnosis of teratoma .Signs of a tumor are detected immediately after birth. Most often they are observed in girls and are located in the perineum, reaching sometimes enormous sizes, hanging between the hips and pulling down and shifting the anus anteriorly.

At palpation, the teratoma is painless and heterogeneous in consistency: it is probed that dense inclusions( bones, cartilage), then softer areas( cystic).The skin above the tumor is usually unchanged, but vasodilatation can occur as branched angioma, as well as hairiness, embryonic scars. The upper pole of the tumor goes into the presacral space, where it is well felt in digital rectal examination. But sometimes the upper boundary reaches the promontory and even higher and palpation is unavailable. Behind the teratoma goes under the gluteus muscles and its boundaries are not always clearly defined.

Benign teratoma grows slowly in the first months of a child's life. Its increase depends mainly on the accumulation of contents in the cystic cavities. From the 6th to the 10th month there is a danger of malignant degeneration, which is characterized by rapid growth and metastasis to the nearest lymph nodes( in particular inguinal) and other organs. Malignant transformation of sacrococcygeal teratomas is observed in 10% of cases.

Primary malignant forms develop rapidly, showing blastomatous properties in the first weeks of life. Often, the tumor squeezes the anus and the neck of the bladder, so there is early delay in stool and urination, which sometimes requires the urgent imposition of a fecal or suprapubic urinary fistula.

The diagnosis of the teratoma is mostly unobtrusive. A characteristic and, it may be said, pathognomonic sign of a teratoma is the combination of cystic fluctuating areas and dense nodes. X-ray images of the tumor show dense shadows from bone and lime inclusions, which, together with the clinical picture, gives enough grounds for establishing a diagnosis. In a number of cases it is necessary to differentiate teratoma and spinal hernia.

Treatment of teratoma .The only correct method is surgical removal of the tumor. The duration of the operation is determined by the clinical course of the tumor process. With an obviously benign course, the operation can be postponed to 6-8 months of age. With primary malignant teratoma( rapid growth), as well as complications( tumor rupture, bleeding), urgent intervention is necessary. However, at the present stage, in all cases, it is necessary to consider the intervention as principally shown as possible earlier, as the potential malignancy of the teratoma reduces the chances of a favorable outcome, even if the operation is postponed for several weeks. Therefore, if the medical institution has the appropriate conditions, the patient with a teratoma of the sacrococcygeal region should be sent to the clinic as soon as possible, where such an operation can be performed.

Technique for the removal of teratoma .An arcuate cut of the skin is applied so that its ends reach the large trochanteres of the thigh, and the middle of the arc is located posteriorly from the anus at 3-5 cm. Before surgery, a thick vent tube is introduced into the rectum, which facilitates the excretion of the intestine,with a tumor. After dissection of the skin and cellulose, the excretion of the tumor begins in the front. Then the tumor is separated from the intestine - this is the most difficult stage of the operation. Isolation of the tumor from the muscles of the buttocks is usually not difficult. To access the part of the tumor that is located in the pelvis, the coccyx is resected. After careful hemostasis, the pelvic floor and skin are sewn up, the excess of which is excised from behind.

In the postoperative period, drainage is left for 1-2 days between the sutures. The child is laid in bed on his stomach or on his side. The first days of intensive infusion therapy.

One of the frequent postoperative complications is paresis or paralysis of the sphincters of the rectum and bladder, which subsequently causes the need for rehabilitation treatment.
Tips for the treatment of the sacrococcygeal teratoma The results of treatment with benign teratite are good. The forecast is favorable. Primary malignant and malignant tumors are prognostically very unfavorable.