The occurrence of sacrococcygeal teratoma and its treatment. The most preferred diagnostic method. Leading specialists from clinics abroad

Teratomas are mixed tumors of complex structure, most often localized in the sacrococcygeal region. According to their course and prognosis, they can be regarded as a developmental defect (a number of authors classify teratomas as tumors due to the presence of immature embryonic tissue in them). The morphology of teratomas is extremely diverse and fully justifies its name “miraculous tumors.” In them you can find tissues arising from 2-3 germ layers. Sometimes the tumor contains formed organs or the whole fetus (organismoid teratoma).

Until recently, high mortality was noted in children with teratomas of the sacrococcygeal region. Children die from severe complications associated with the presence of a tumor: compression and intestinal obstruction, suppuration of cystic cavities with the subsequent development of sepsis, bleeding and malignant degeneration. Complications can occur at any age, but more often during the neonatal period (Bairov G. A., Mankina N. S., 1977, etc.]

Many surgeons believe that children with teratomas of the sacrococcygeal region are not viable and die soon after birth. last years noted that proper care care of the child, constant monitoring by the surgeon to identify and prevent various complications allow children to develop satisfactorily for some time. At the same time, the large size of the tumor, its rapid growth and possible malignancy lead to the need for relatively early surgical intervention, the period of which most surgeons determine by age 6 months. By this time, the child will get stronger and can better endure the operation, the severity of which is well known

Based on our observations (194 children) and published data, we can distinguish four main groups of complications, the occurrence of which is an indication for urgent surgical treatment of teratomas of the sacrococcygeal region."

1) compressed by a tumor of the rectum or urethra; 2) rupture or sharp thinning of the membranes with a cystic form of the tumor;

3) ulceration or necrosis of the skin, suppuration of individual cystic cavities;

4) rapid tumor growth, suspicion of malignant degeneration.

Clinical picture. At outdoor location tumors (Fig. 86, a) the child after birth usually does not show concern and takes the breast; bowel movements and urination are normal. The tumor initially does not affect the general condition of the patient, and only subsequently can one notice a gradual decrease in the child’s nutrition, apparently associated with the growth of the tumor, which, developing at the expense of the patient, depletes his strength. It should be noted that until 6-8 months of age, teratoma usually increases slowly, in proportion to the child’s growth.

Recognizing teratomas of the sacrococcygeal region is usually not difficult due to the typical location and external appearance of the neoplasm, the size of which varies widely, sometimes exceeding the circumference of the patient’s head.

The tumor, located in the area of the sacrum and buttocks, moves to the soft part, moving it anteriorly. The base of the teratoma is wide, not clearly defined, goes under gluteal muscles. At external-internal location(Fig. 86, b) the anterior and upper borders can only be determined by examination through the rectum. The skin over the tumor may be unchanged or covered with hair, but sometimes it is sharply thinned and macerated, with a pronounced venous pattern. Palpation reveals the heterogeneity of the tumor: areas of dense, almost cartilaginous consistency are replaced by softer, sometimes fluctuating ones. This is due to the fact that the tumor tissue contains a large number of cystic cavities of various diameters. Dense inclusions in some cases represent the rudiments of organs. Sometimes a teratoma has the character of a cyst.

At internal location tumor (Fig. 86, c) its nodes are palpable in the thickness of the buttocks, usually asymmetrically. Located between the coccyx and the rectum, the tumor compresses the perineal organs, but does not grow into them. Digital examination through the rectum allows you to clarify the location and extent of the tumor. With an internal location, teratoma is discovered at an older age, usually due to malignancy.

X-ray method It has great importance for diagnosis as part of a clinical examination of a newborn with teratoma of the sacrococcygeal region. Areas of calcification or bone inclusions identified on the images make the diagnosis undoubted (Fig. 87, a). If a newborn has a cystic form of the tumor, then a soft tissue shadow is visible on radiographs (Fig. 87, b). In such cases, you should pay attention to the shape of the arches of the lumbosacral vertebrae - the presence of a defect in them allows you to suspect spina bifida.

Differential diagnosis Most often it has to be carried out with spina bifida. The latter is excluded by palpation of dense inclusions in the tumor. Normal function limbs and pelvic organs of the child speaks against spina bifida in the same way as what was found during digital examination compression of the rectum by a tumor. In addition, unlike a hernia, the tension of a teratoma does not increase when the child cries, and pressure on the tumor does not cause bulging of the fontanel. There is no rapid increase in swelling with spina bifida.

Complications manifest themselves with quite pronounced symptoms and deterioration general condition child.

Compression by a tumor of the rectum It is more often observed in the first days of a child’s life. By the time of birth, the tumor can compress or displace the rectum so much that from the first days the newborn experiences symptoms intestinal obstruction. This usually occurs in children with internal teratoma.

Compression of the rectum can also occur at an older age. We observed 3 children in whom the tumor was not difficult at first.

la functions of the pelvic organs. Fast growth teratoma led to obstruction of the rectum - the act of defecation became difficult, anxiety and bloating appeared. The general condition of such newborns progressively worsens, appetite is reduced, regurgitation and vomiting occur. The phenomena of complete obstruction gradually develop.

Compression of the urethra occurs relatively rarely - with a high internal location of the tumor. The complication is urinary retention; the child is worried when urinating, straining. Catheterization is difficult. Soon the urine begins to separate drop by drop, bladder stretched. In advanced cases, complete urinary retention occurs. Compression of the urethra by a large tumor is often combined with obstruction of the rectum.

Sharp thinning of the membranes or rupture of the wall of a cystic teratoma at birth we observed in 6 children. In newborns with a multilocular teratoma, when a rupture occurs, one or more cavities are emptied, but a tumor-like formation remains. In one girl we operated on, the teratoid single-chamber cyst was so large that the birth ended safely only after the membranes ruptured and the contents leaked out. Upon admission to the clinic, a skin “bag” measuring 20 x 30 cm was identified in the sacrococcygeal region.

In some cases, the thinning of the teratoma membranes reaches such an extent that the wall becomes translucent and shiny. It seems that a careless touch will cause the cyst to open; this sometimes happens when swaddling a baby.

Tumor ulceration and necrosis skin lesions are indications for urgent hospitalization of the child in the surgical department and early surgical treatment. Availability infected wound or suppuration of a cyst without active special assistance can quickly lead a newborn to sepsis and death, which we observed in 2 out of 8 newborns with a similar complication.

In children with necrosis and ulceration of teratoma, spontaneous bleeding occurs in some cases, which leads to death without immediate surgery.

Malignization We observed in 6 children aged 1 to 3 years: in 3 there was primary degeneration of teratoma, in 3 the tumor area left during surgery underwent malignant degeneration.

Teratomas, which contain immature tissue elements, are prone to early malignant degeneration. Rapid tumor growth, the appearance of a venous pattern, and deterioration in the child’s general condition are the main signs of teratoma malignancy. According to our observations and published data, malignant degeneration is observed in children older than 10-12 months, more often after non-radical operations.

Treatment. Surgical placement according to emergency indications carried out in case of complications in newborns. The prognosis for sacrococcygeal teratomas largely depends on the type of complications, timely initiation of treatment, tumor size and intervention technique. Equally important is the correct management of the preoperative and postoperative period.

Preoperative preparation. The nature and duration of preoperative preparation depend on the type of complications.

Children admitted with obstruction of the rectum or urethra, as well as with ruptures of the tumor membranes, are taken to the operating room after the usual drug preparation to anesthesia.

When thinning the membranes, special preparation is also not required. If the patient has anemia, then surgery is prescribed after several blood transfusions.

Longer preoperative preparation performed for children with suppurative and inflammatory changes in the tumor. In such cases, antibacterial treatment is prescribed (antibiotics, bandages with antiseptics), existing purulent leaks are opened and active restorative treatment(transfusions of blood, plasma, vitamin therapy, etc.). The operation is postponed until the general condition improves and local symptoms subside inflammatory processes, normalization of body temperature and blood test parameters.

Surgery in newborns is performed under endotracheal anesthesia. During the operation, constant blood transfusion is necessary to fully compensate for the massive blood loss.

Hence the need arises for catheterization of the superior vena cava to measure central venous pressure during anesthesia and thorough blood replacement.

Operation technique. The child's position is on his stomach with his knees bent and legs apart. An arcuate skin incision is made in such a way that its ends reach the greater trochanters, and the middle of the arc is located 5-7 cm behind the anus (Fig. 88, a). The width of the flap should approximately correspond to the base of the tumor. After dissecting the skin and subcutaneous tissue, the tumor is first isolated from the front, trying not to open the membranes. Using hydraulic preparation with a 0.25% novocaine solution, it is possible to perform the most difficult part of the operation - separation of the tumor from the posterior surface of the rectum (Fig. 88, b). To prevent damage and facilitate mobilization of the rectum, a thick gas outlet tube is inserted into the rectum.

For the widest access to the part of the tumor that is located in the pelvis, some authors recommend resecting the coccyx and part of the sacrum (Fig. 88, c). We use a similar approach in children with tumor recurrence or internal layout teratomas (usually in older children).

After removing the tumor, carefully stop the bleeding. The pelvic floor is sutured with catgut sutures after powdering the wound with dry antibiotics. Excess skin is excised, and interrupted nylon sutures are placed on the wound. For 24 hours, a thin rubber drainage is inserted into the corner of the wound between the sutures. In cases where it is technically not possible to completely remove the tumor, the operation is performed in two stages. We believe that stage II - radical excision of the remaining areas of the teratoma - should be carried out no more than 1-2 months after the first operation.

Postoperative treatment. After the operation, the child is placed on his stomach, thereby preventing contamination of the wound. The patient quickly gets used to this position and lies completely calm for 7-9 days. If the dressing gets wet, change it immediately.

On the first day, the child is left with an intravenous drip system; the amount of fluid is calculated according to body weight and age. Feeding is allowed 6-10 hours after surgery. Feeding newborns begins with 10 ml of expressed milk, the amount of which is gradually increased, after a day switching to regular milk. age norm. Older children receive a gentle postoperative norm within 3-4 days.

During the first week, the child is prescribed antibiotics wide range actions, conduct restorative treatment. Repeated blood transfusions are necessary 2-3 times a week until the general condition improves. The sutures are removed on the 9-11th day. The child is discharged home after complete healing of the wound. In the future, systematic observation of the surgeon is necessary for early detection of possible tumor recurrence.

(CCT) is the most common congenital tumor in newborns and occurs in approximately one in 35,000 to 40,000 live newborns. The ratio of female to male children is 4:1. Teratoma includes solid formations and cysts filled with serous fluid, mucoid or sebaceous substance. Among other elements, the most common tissues are neuroglia, skin, smooth and striated muscles, respiratory and intestinal epithelium, cartilage; In some cases, parts of an immature twin were found. In rare cases, the tumor is malignant. The size of the formation ranges from 1 to 30 cm, averaging 8 cm in diameter.

Among concomitant pathology Renal lesions (hydronephrosis, dysplasia), urethral atresia, undescended testicles, rectal atresia, hip dislocation have been described.

Need for blood supply a large mass of teratoma tissue leads to the formation of abundant arteriovenous anastomoses, large shunting of blood, increased cardiac output and the development of heart failure. The vascular network is significantly pronounced when the tumor is solid and less so when it is cystic.

Sacrococcygeal teratoma can grow posteriorly (outward) or anteriorly from the sacrum, deforming adjacent organs (rectum, vagina, bladder). In accordance with this, four types of tumors are distinguished:
Type I - predominantly external location of the teratoma with a minimal presacral component;
Type II - external and internal location of teratoma;
Type III - the predominant location of the teratoma in the abdominal cavity with a visible external component;
Type IV - presacral location of the teratoma without an external component.

Natural history of sacrococcygeal teratomas. In fetuses with this pathology, dropsy, intratumoral hemorrhage, and polyhydramnios are detected, which aggravate the course of pregnancy. Mortality among fetuses is high, on average 50%. The causes of death are heart failure associated with volume overload of the heart, premature birth, teratoma rupture, anemia. When obstructed by a tumor urinary tract On the contrary, oligohydramnios and pulmonary hypoplasia occur. Among newborns who have survived the intrauterine period, mortality is reduced to 5%. Delivery must be carried out in specialized center, which has specialists in the field of neonatal surgery.

Prenatal diagnosis sacrococcygeal teratomas. Most teratomas are detected between 22 and 34 weeks of pregnancy. During ultrasound examination, the main symptom that attracts the attention of researchers is an increase in the size of the uterus due to the teratoma itself or polyhydramnios. In the area of the fetal sacrum, a heterogeneous mass consisting of soft tissues is determined, cystic formations and numerous vessels. Often the inferior vena cava is significantly dilated compared to the aorta. An important point is to study the intrapelvic position of the teratoma and its relationship with adjacent organs. In this aspect, it is essential diagnostic benefits has magnetic resonance imaging. When a teratoma is detected, dynamic monitoring of its growth, the degree of vascularization of the tumor, and symptoms of heart failure is necessary. Teratoma can reach a significant size and even exceed the size of the fetus.

Additionally, a thorough examination of the fetal spine is necessary to exclude myelomeningocele, as well as a search for other congenital anomalies.

Clinical symptoms of sacrococcygeal teratomas. Pregnancy, as a rule, is complicated by polyhydramnios, hydrops fetalis, preeclampsia, and premature birth. When the fetus passes through natural birth canal Possible bleeding into the teratoma or its rupture. The volume of hemorrhage may be comparable to the volume of blood in a newborn. After birth, it is necessary to ensure that there is no damage to the surface of the tumor or erosions that could contribute to bleeding.

Baby's blood circulation characterized by high cardiac output; Systolic and diastolic murmurs are heard above the tumor.
Electrocardiography. Electrocardiogram changes are nonspecific and may reflect cardiac volume overload.

Radiography. X-ray required chest, abdomen and pelvis to determine the extent of the tumor, identify concomitant pathology or metastases. CTI is usually increased.

Echocardiography. This study usually does not detect any specific changes in the heart. In most cases, the hyperdynamic nature of its work is determined; cardiac output increased. The size of the heart cavities is usually enlarged, and disturbances in systolic and diastolic functions vary widely.

Ultrasound examination of teratoma aimed at assessing the nature of the formation (cystic or solid), its prevalence, degree of vascularization, and involvement of adjacent organs.

Angiography. This study is useful for determining the sources of blood supply to the teratoma (usually the middle and lateral sacral arteries, gluteal branches of the internal iliac artery, branches of the deep femoral artery). The angiogram shows a pronounced vascular network with arteriovenous shunts intertwined with dilated veins.

Treatment of sacrococcygeal teratomas.

In the case of predominantly cystic tumor structure possible prenatal puncture and controlled emptying ultrasound examination. This allows you to reduce uterine irritation, prevent premature birth, as well as teratoma rupture during childbirth. For solid tumor treatment options are more limited. It is necessary to strive for a speedy delivery, as soon as the fetal lungs are sufficiently mature. Usually they resort to caesarean section to exclude hemorrhage into the teratoma, its rupture and other pathology during childbirth.

There are rare reports of intrauterine radiofrequency teratoma ablation. In these cases, heart failure is eliminated, but the fetus may die from the effects of tumor necrosis. After birth, surgery is aimed at complete removal teratomas along with the coccyx; If malignant cells are detected, radiation and chemotherapy are additionally administered. During surgery Special attention must be devoted to maintaining normal temperature baby's body, because rich in blood vessels the tumor leads to intense heat loss.

More common in girls than boys (3:1); Malignant teratomas are more common in boys.
The most common congenital tumor, usually diagnosed before birth in pregnant women, hydrops fetalis
Non-immune hydrops fetalis is benign in 80-85% of cases
Localization: the tumor is usually located intradural and extramedullary, rarely - intramedullary (2% of cases)
Mortality: 15-35%
Mixed tumor consisting of derivatives of all three germ layers
Formed from pluripotent cells of Hensen's node
The tissue component has varying degrees maturity (mature and immature teratoma are distinguished).

Classification of teratoma

Type I: Spreads beyond the fetus.
P type: Extends outward and presacral.
Type III: Small external component, predominantly intrapelvic and intra-abdominal spread.
Type IV: Completely presacral tumor.

What is the Currarino triad?

Malformations of the sacrum.
Atresia or stenosis anus.
Presacral tumor (benign teratoma in 40% of cases, anterior meningocele in 47% of cases).

Which diagnostic method to choose: MRI, CT, ultrasound

Most preferred diagnostic method

Sagittal projection: T1- and T2-weighted images, STIR sequence.
Frontal projection: T2-weighted image.
Horizontal projection: T2-weighted image.
Abdominal examination (CT, MRI): assessment of ventral extension (in abdominal cavity).

What happens with a teratoma

Heterogeneous solid-cystic formation in the sacrum and distal part lumbar region spine, sometimes extending into the pelvic cavity and abdominal cavity
Secondary hydronephrosis due to compression of the urinary tract
Polyhydramnios.

What will an MRI of the spine show for sacrococcygeal teratoma?

Cysts:

give a uniformly enhanced signal (on T1- and T2-weighted images) with high content squirrel; weak signal (on T1-weighted image) and increased signal (on T2-weighted image) with serous content, sometimes septations are present

Solid nodules:

accumulate contrast

Fat component:

STIR sequence with signal suppression from adipose tissue

Areas of calcification:

marked decrease in signal intensity on T1- and T2-weighted images.

A newborn with a sacrococcygeal teratoma identified by prenatal ultrasound. MRI of the pelvis (sagittal projection, T2-weighted image). A formation, most of which is located anterior to the spine, giving an inhomogeneous signal; the caudal parts of the spine are fully represented.

MRI of the sacrum (frontal view, T2-weighted image). The spread of the tumor into the sacrum is visualized.

What can be seen on an ultrasound

Hyperechoic (solid) areas with inclusions or a peripheral anechoic (cystic) component
Calcifications: hyperechoic foci with acoustic shadowing.

Clinical manifestations

Typical symptoms:

Hydrops fetalis in pregnant women: a consequence of intrauterine complications such as polyhydramnios and hemorrhage into the tumor
The prognosis is extremely unfavorable for the development of hydrops fetalis in pregnant women before 30 weeks. pregnancy (mortality rate 93%)
Complications in postpartum period due to concomitant malformations (10% of cases), compression neighboring organs(eg, urinary tract), tumor recurrence, intraoperative and postoperative complications.

Treatment

In cases of non-immune hydrops fetalis, labor should be induced as soon as the fetal lungs have reached sufficient maturity.
In utero or in the 1st week of life, radical resection of the tumor (together with the coccyx) is indicated - removal of the sacrococcygeal teratoma
Fetal interventions: tumor resection, aspiration of cyst contents, amnioreduction.

What can be confused with spinal teratoma?

Myelomeningocele

Spina bifida is located higher and more dorsally

Tumors in the pelvic cavity

Ovarian cysts, mesenteries
Dilatation or duplication of the intestine
Cystic neuroblastoma

Solid tumors

Chordoma
Neurogenic tumors
Lipoma
Hemangioma
Melanoma

Pilonidal teratoma - rare view The tumor, which occurs with a frequency of 1:40,000 newborns, is much more common in girls than in boys (3:1). It usually originates from the caudal end of the spine, spreading to the perineum and displacing the anus anteriorly. It is generally accepted that sacrococcygeal teratoma occurs as a result of the continued proliferation of totipotent cells of the primary Hensen's node, which lose the ability to reverse development at the end of embryonic life.

Pilonidal teratomas are true tumors. Willis gave them the following definition: “a teratoma is a true tumor or neoplasm consisting of a variety of tissues foreign to the area where it develops.” A teratoma is composed of several tissue types, usually derived from two or three germ layers. Within a single tumor, cells can range from completely benign (even in the form of well-formed teeth, hair, or other organs) to clearly malignant. However, many teratomas contain "malignant-like" cells (usually described as "immature"), and if such a teratoma is completely excised, it does not recur. The risk of malignancy depends on the location and size of the tumor, as well as the timing of diagnosis. Tumors diagnosed later than the second month of life have high risk malignancy. Teratomas that have “exophytic” growth (i.e., the tumor is located primarily externally with a small intra-abdominal component) are usually benign. However, tumors with a large intra-abdominal component have a high risk of malignancy. If the teratoma is completely removed, the risk of recurrence is low.

Symptoms

Pilonidal teratoma - photo

Typically, coccygeal teratomas are diagnosed immediately at birth as great education in the sacral region. However, nowadays the diagnosis is most often made by antenatal ultrasound. A number of publications report that if the size of a teratoma in a fetus exceeds the size of the fetus itself, then in most cases such children die in utero from heart failure, since the fetal heart is not able to sufficiently supply blood (“pump blood”) to both the tumor and itself. fetus. In addition, most studies report that with teratoma, fetal hydrops (non-immune) is often observed - in this case there is also a high risk of fetal death.

Pilonidal teratoma is usually a large tumor covered with skin that bulges in the coccygeal region and displaces the anus and vagina anteriorly. Dilated veins are sometimes visible on its surface. Large teratomas may be complicated by rupture of the membranes with profuse bleeding or ulceration of the surface. Newborns with a teratoma that is close to the size of the baby may be born prematurely and often have signs of nonimmune hydrops. In all cases, the tumor is tightly connected to the coccyx, one might say “emanates” from its anterior surface. The coccyx can be displaced posteriorly, and the sacrum is almost always normal.

Video: Ovarian teratoma. Monster tumor

Diagnostics

Ultrasound allows you to determine the size and “consistency” of both the pelvic and abdominal parts of the teratoma. Sometimes it is necessary to fill the bladder to improve visualization of the teratoma on ultrasound. Magnetic resonance imaging allows one to clearly differentiate a teratoma from an anterior meningocele, as well as identify the spread of the tumor ( rare cases) through the sacral foramen into the spinal canal.

Treatment

Treatment at home, Traditional treatment

It is better to remove a coccygeal teratoma in the first day after birth, when the newborn’s intestines are not yet colonized, and therefore the risk of infection is reduced both during and in the postoperative period. Antibiotics are administered perioperatively before surgery and for 24-48 hours after surgery. If the child was feeding before surgery or is already several days old, then bowel preparation before surgery is advisable. It is also necessary to determine the blood type and ensure adequate venous access. It is important to determine the level of a-fetoprotein in the blood before surgery, so that after the intervention it is possible, by repeating the study, to ensure that the level of a-fetoprotein has decreased to normal.

Operation

After induction of anesthesia, the patient is placed face down with a bolster under the hips. The cushion is positioned so that the child's weight rests primarily on the anterior superior iliac spines. It is important that the abdomen “hangs” freely in order to avoid breathing problems associated with compression of the abdomen. In this regard, the child’s shoulders should be supported by a small bolster placed under the upper chest, or by two bolsters located parallel to the spine under the glenohumeral joints. A catheter is inserted into the bladder to measure urine output during surgery. Many surgeons believe that the surgical field should be separated from the anus. However, according to the author, this approach is inconvenient and impractical, since access to the anus is often necessary during surgery. It is important to keep the child warm on the operating table (plastic covering) to prevent hypothermia.

An angular incision is made above the dorsal surface of the tumor, continuing deep into the fascial layers. At this stage of release, it is not advisable to penetrate beyond the level of the deep fascia. Often, several large veins are found on both sides of the midline in the subcutaneous tissues. They must be cut after dressing. The incision should be made in such a way as to preserve normal skin, as much as possible. Excess skin can be removed later if necessary. The top of the cut should be located above bottom sacrum.

Video: Epithelial coccygeal tract

Dissection of the coccygeal teratoma continues straight down to the sacrococcygeal joint, or even below the 4th or 5th sacral vertebra. The edges of the sacrum are identified and at this level a clamp is made between the sacrum and the underlying (rather large) middle sacral vessels supplying the bulk of the tumor. Now the sacrum (usually completely or mostly cartilaginous) can be cut with a scalpel and the tumor is moved slightly downward, allowing the middle sacral vessels to be approached. Dissection of the sacrum should be done with great care, since sometimes the main outflow venous blood from the tumor is carried out through the sacral fissure (outlet of the sacral canal) into the epidural plexus. If such an anatomical situation is not recognized, rapid and significant loss blood. Bleeding is stopped by normal pressure on the vessels until arterial vessels will not be bandaged or cut. However, even after this, bleeding from the epidural plexus may continue, requiring the use of wax to stop bleeding from the bone. Sometimes, to allow caudal displacement of the distal part of the sacrum and coccyx, it is necessary to separate (cut off) the remnants of the levators attached to the edges of the lower part of the sacrum and coccyx. The middle sacral vessels are then ligated along their length and divided. Early division of the middle sacral vessels [as suggested by Smith et al] allows entry into a layer outside the tumor capsule but deeper to the remnants of the levator and gluteus maximus muscles. The levators can be so thin as to be almost invisible, but they contract in response to stimulation as muscle stimulator, and electrocautery. Dissection should be continued laterally on either side of the midline until the muscles extend into the tumor fascia. At this point they can be incised along a line parallel to the skin incision. This will allow the tumor to be further displaced in a caudal direction.

All attention should now turn to the pelvic component of the coccygeal teratoma. Using small tampers, the tumor is isolated anteriorly towards the anterior sacral vessels, while displacing it forward until the upper border of the tumor is reached. There is usually an avascular zone anterior to the sacrum, although laterally several branches can be found extending towards the tumor from the internal iliac vessels. These branches usually coagulate. In most cases, the tumor can now be “dislocated” from the pelvis and retracted inferiorly.

After retraction of the coccygeal teratoma, it opens top part rectum, which can be easily detected by inserting a Vaseline tampon or finger into the rectum through the anus. Teratoma is separated from the rectum, combining blunt and acute discharge and moving it more and more downwards until it is completely separated from the rectum and anal canal. During the process of isolation, you should always stay as close as possible to the tumor capsule, trying to preserve all normal anatomical structures, no matter how altered, mixed and thinned they may be. As the tumor is retracted downwards, the subcutaneous tissue is reached along its lower surface, behind the anus. At this point, the isolation can be completed if the lower skin flap is of sufficient size to easily close the wound. The lower skin flap is separated from the coccygeal teratoma and the tumor is removed. The teratoma bed is carefully examined to ensure reliable hemostasis.

Then reconstruction is carried out pelvic floor and suturing the wound. The remnants of the levator loop are identified and the central part is sutured to the perichondrium of the anterior surface of the sacrum using 5/0 absorbable monofilament. All muscles and fascia are sutured with the same sutures. It is these initial fascial sutures, to a greater extent than the skin ones, that will determine the position of the anus. Therefore, this stage of reconstruction must be performed with extreme care and precision to ensure a good functional and cosmetic result.

If a drain is used, it is placed at this stage in the presacral space, leading out through the gap in the levators and through a tunnel in the subcutaneous tissues of the buttocks.

If levator remnants are identified on the sides of the midline, they are sutured with separate absorbable 5/0 monofilament sutures. The medial edges of the gluteus maximus muscle are sutured in the midline above the sacrum and the lower part of the levator ring. The skin flaps are then aligned in length. If possible, then subcutaneous tissue sutured with a continuous suture PGA5/0, and the skin with the same continuous subepithelial suture. The skin edges are strengthened with a plaster and a bandage with collodion. If the subcutaneous tissue cannot be sutured, then a subepithelial suture is not appropriate. In this case, the skin is sutured with 5/0 nylon. At the end of the operation, a Vaseline swab is inserted into the rectum to obliterate the “dead space”. It is advisable to stitch the end of the tampon with a 2/0 silk thread to facilitate its removal if the tampon “goes” inside (higher) into the rectum in early postoperative period.

After operation

Positioning the child face down for several days after surgery for coccygeal teratoma. As the condition stabilizes and recovers respiratory functions the child is disintubated and removed urinary catheter. After disintubation, feeding begins. The Vaseline tampon is removed during the first postoperative day. If any drainage is left in place, it will be removed within the first few days after surgery. It is necessary to determine the level of a-fetoprotein immediately after surgery and before discharge. The child is examined monthly during the first three months after surgery and then once every 3 months. during a year.

The child should be observed for 5 years. At each examination, it is necessary to conduct a rectal examination (in order not to miss a relapse) and determine the level of a-fetoprotein to identify distant metastases (if they occur). The level of a-fetoprotein is often very high (on the order of 100,000 or more) and even in healthy children may be above 10,000. Such indicators usually last for a year until they return to normal. It is believed that if the level of a-fetoprotein steadily decreases, then relapse is unlikely. However, the author’s experience indicates that a constant drop in the level of a-fetoprotein does not at all exclude the possibility of both local relapse of coccygeal teratoma and relapse even with malignancy. All local relapses and relapses with malignancy developed against the background of a persistent decrease in the level of a-fetoprotein. For this reason, according to the protocol developed by the author, rectal examination and ultrasonography are performed regularly during the first 3 years of life.

If surgery for coccygeal teratoma was performed radically and there were no distant metastases at the time of intervention, children develop normally, although appearance buttocks “leaves much to be desired.” Surprisingly, children usually retain their feces. However, the relatively late development (at the age of 3 years) in one of our patients of a neurogenic bladder indicates that it is necessary to speak with confidence about normal bladder function in such patients with caution until school age. If bladder function is normal by school age, it can be argued that urinary system there are no consequences of intervention.

Video: coccygeal tract 1

Forecast

The prognosis of children with malignant pilonidal teratoma must be discussed with great caution. Modern chemotherapy has contributed to some improvement in survival, but the drugs used are extremely toxic and the tumor is quite resistant to chemotherapy. However, fortunately, malignant pilonidal teratoma is rare.

Video: Operation for removal of coccyx cyst Kleft Lift (plasty of the intergluteal groove)

Many surgeons believe that children with teratomas of the sacrococcygeal region are not viable and die soon after birth. In recent years, it has been noted that proper child care, constant observation by a surgeon to identify and prevent various complications allow children to develop satisfactorily for some time. At the same time, the large size of the tumor, its rapid growth and possible malignancy lead to the need for relatively early surgical intervention, the period of which most surgeons determine at 6 months of age. By this time, the child will get stronger and can better endure the operation, the severity of which is well known

1) compressed by a tumor of the rectum or urethra; 2) rupture or sharp thinning of the membranes with a cystic form of the tumor;

3) ulceration or necrosis of the skin, suppuration of individual cystic cavities;

4) rapid tumor growth, suspicion of malignant degeneration.

Clinical picture. At outdoor location tumors (Fig. 86, a) the child after birth usually does not show concern and takes the breast; bowel movements and urination are normal. The tumor initially does not affect the general condition of the patient, and only subsequently can one notice a gradual decrease in the child’s nutrition, apparently associated with the growth of the tumor, which, developing at the expense of the patient, depletes his strength. It should be noted that until 6-8 months of age, teratoma usually increases slowly, in proportion to the child’s growth.

The tumor, located in the area of the sacrum and buttocks, moves to the soft part, moving it anteriorly. The base of the teratoma is wide, not clearly defined, and extends under the gluteal muscles. At external-internal location(Fig. 86, b) the anterior and upper borders can only be determined by examination through the rectum. The skin over the tumor may be unchanged or covered with hair, but sometimes it is sharply thinned and macerated, with a pronounced venous pattern. Palpation reveals the heterogeneity of the tumor: areas of dense, almost cartilaginous consistency are replaced by softer, sometimes fluctuating ones. This is due to the fact that tumor tissue contains a large number of cystic cavities of various diameters. Dense inclusions in some cases represent the rudiments of organs. Sometimes a teratoma has the character of a cyst.

X-ray method is of great importance for diagnosis in the complex clinical examination of a newborn with teratoma of the sacrococcygeal region. Areas of calcification or bone inclusions identified on the images make the diagnosis undoubted (Fig. 87, a). If a newborn has a cystic form of the tumor, then a soft tissue shadow is visible on radiographs (Fig. 87, b). In such cases, you should pay attention to the shape of the arches of the lumbosacral vertebrae - the presence of a defect in them allows you to suspect spina bifida.

Differential diagnosis Most often it has to be carried out with spina bifida. The latter is excluded by palpation of dense inclusions in the tumor. Normal limb function and pelvic organs a child speaks against spina bifida in the same way as compression of the rectum by a tumor detected during a digital examination. In addition, unlike a hernia, the tension of a teratoma does not increase when the child cries, and pressure on the tumor does not cause bulging of the fontanel. There is no rapid increase in swelling with spina bifida.

Complications are manifested by quite vivid symptoms and a deterioration in the general condition of the child.

Compression by a tumor of the rectum It is more often observed in the first days of a child’s life. By the time of birth, the tumor can compress or displace the rectum so much that from the first days the newborn experiences intestinal obstruction. This usually occurs in children with internal teratoma.

Compression of the rectum can also occur at an older age. We observed 3 children in whom the tumor was not difficult at first.

la functions of the pelvic organs. The rapid growth of the teratoma led to disruption of the patency of the rectum - the act of defecation became difficult, anxiety and bloating appeared. The general condition of such newborns progressively worsens, appetite is reduced, regurgitation and vomiting occur. The phenomena of complete obstruction gradually develop.

Compression of the urethra occurs relatively rarely - with a high internal location of the tumor. The complication is urinary retention; the child is worried when urinating, straining. Catheterization is difficult. Soon the urine begins to separate drop by drop, the bladder is distended. In advanced cases, complete urinary retention occurs. Compression of the urethra by a large tumor is often combined with obstruction of the rectum.

Tumor ulceration and necrosis skin are indications for urgent hospitalization of a child in surgery department and early surgical treatment. The presence of an infected wound or a suppurating cyst without active special care can quickly lead a newborn to sepsis and fatal outcome, which we observed in 2 out of 8 newborns with a similar complication.

In children with necrosis and ulceration of teratoma, spontaneous bleeding occurs in some cases, which leads to death without immediate surgery.

Malignization We observed in 6 children aged 1 to 3 years: in 3 there was primary degeneration of teratoma, in 3 the tumor area left during surgery underwent malignant degeneration.

Treatment. Surgical intervention for emergency indications is carried out in case of complications in newborns. The prognosis for sacrococcygeal teratomas largely depends on the type of complications, timely initiation of treatment, tumor size and intervention technique. Equally important is the correct management of the preoperative and postoperative period.

Preoperative preparation. The nature and duration of preoperative preparation depend on the type of complications.

Children admitted with obstruction of the rectum or urethra, as well as with ruptures of the tumor membranes, are taken to the operating room after the usual drug preparation for anesthesia.

When thinning the membranes, special preparation is also not required. If the patient has anemia, then surgery is prescribed after several blood transfusions.

Longer preoperative preparation is carried out for children with suppurative and inflammatory changes in the tumor. In such cases, it is prescribed antibacterial treatment(antibiotics, bandages with antiseptics), open existing purulent leaks and conduct active restorative treatment (transfusions of blood, plasma, vitamin therapy, etc.). The operation is postponed until the general condition improves, local inflammatory processes subside, body temperature and blood test values normalize.

Surgical intervention in newborns it is performed under endotracheal anesthesia. During the operation, constant blood transfusion is necessary to fully compensate for the massive blood loss.

Hence the need arises for catheterization of the superior vena cava to measure central venous pressure during anesthesia and thorough blood replacement.

Operation technique. The child's position is on his stomach with his knees bent and legs apart. An arcuate skin incision is made in such a way that its ends reach the greater trochanters, and the middle of the arc is located 5-7 cm behind the anus (Fig. 88, a). The width of the flap should approximately correspond to the base of the tumor. After cutting the skin and subcutaneous tissue The tumor is isolated from the front first, trying not to open the membranes. Using hydraulic preparation with a 0.25% novocaine solution, it is possible to perform the most difficult part of the operation - separation of the tumor from the posterior surface of the rectum (Fig. 88, b). To prevent damage and facilitate mobilization of the rectum, a thick gas outlet tube is inserted into the rectum.

For the widest access to the part of the tumor that is located in the pelvis, some authors recommend resecting the coccyx and part of the sacrum (Fig. 88, c). We use this approach in children with tumor recurrence or internal teratoma (usually in older children).

On the first day, the child is left with an intravenous drip system; the amount of fluid is calculated according to body weight and age. Feeding is allowed 6-10 hours after surgery. Newborns begin feeding with 10 ml of expressed milk, the amount of which is gradually increased, after a day moving to the usual age norm. Older children receive a gentle postoperative norm within 3-4 days.

During the first week, the child is prescribed broad-spectrum antibiotics and undergoes general restorative treatment. Repeated blood transfusions are necessary 2-3 times a week until the general condition improves. The sutures are removed on the 9-11th day. The child is discharged home after complete healing of the wound. In the future, systematic observation of the surgeon is necessary for early detection possible relapse tumors.