From Big Medical Encyclopedia

SPINA BIFIDA (Latin spina a thorn, a rachis + bifidus divided in two, split) — the malformation of a backbone which is characterized by not fusion of vertebrae and incomplete closing of the vertebral channel, often followed by a malformation of a spinal cord. Spina Bifida is quite often combined with a dysplasia of internals and other departments of a skeleton.

Spina Bifida can be observed in various departments of a backbone, however most often in lumbosacral department. Defects in a backbone can be combined with hernial protrusions of a spinal cord and its covers (see. Spinal cord, malformations ).

In 1641 N. Tulpius for the first time described the patient with a tumor on spin on site of not fusion of vertebrae and called this disease of «spina bifida». Splitting of a backbone without hernial protrusion in 1875 was described by R. Virkhov. This pathology is called by it the hidden vertebral crevice (spina bifida occulta). It was established that split can be arches of vertebrae (spina bifida posterior) or a body of vertebrae (spina bifida anterior).

Etiology and pathogeny Spina Bifida finally are not found out. There is an opinion that in developing of this pathology play a role an infection, an injury, intoxication in the period of an antenatal life of a fruit. Refer age of mother to risk factors, especially after 30 years, ginekol. diseases, use of contraceptives, an alcohol abuse in the first months of pregnancy. Assume that hereditary factors are the cornerstone of this malformation. Concerning the mechanism of development Spina Bifida there are two points of view. One researchers consider primary a malformation of a neurotubule and hydrocephaly; others believe that in the beginning there is a malformation of a mesoblast or a backbone at later stages of development of a germ, and formation of a malformation of a spinal cord happens for the second time.

Fig. 1. The diagrammatic representation of cross section of a backbone at rakhis-hiziy: 1 — a skin zone; 2 — an epitelio-serous zone; 3 — vascular and brain area; 4 — a firm cover of a spinal cord; 5 — an arachnoid membrane; 6 — a soft cover of a spinal cord; 7 — an arch of a vertebra; 8 — a body of a vertebra.

Classifications Spina Bifida are founded on results of pathoanatomical researches. The fullest and detailed classification is given by F. Reklingkhauzen. In a crust, time depending on degree of an underdevelopment of a backbone, a spinal cord, its covers, cover fabrics distinguish the following anatomic forms of this malformation: rakhiskhizis (full or partial), spinal hernias, spina bifida complicata, spina bifida occulta, spina bifida anterior. Basic morfol. a sign of a rakhiskhizis is not fusion of soft tissues, a backbone, a meninx and a spinal cord (fig. 1). The spinal cord which is not closed in a tube lies naked; it represents the reddish velvety flat weight consisting of a large number of expanded vessels and elements of brain fabric. At a full rakhiskhizis the described rsartina is observed throughout all backbone, partial rakhiskhizis is usually localized in lumbar department of a backbone. On site defect of arches of vertebrae the germinal medullary plate which is not closed in a tube — vascular and brain area (area medullovasculosa) is, edges lies on a soft cover of a spinal cord. The site of the last from its edge to edge of skin covered with a thin coat of epidermis was called an epitelio-serous zone (zona epitheliose-rosa). Knaruzhi from this zone is located skin — a skin zone (zona dermatica). If in subarachnoid (subarachnoid) space cerebrospinal liquid accumulates, there can be spinal hernia — a myelomeningocele. Rakhiskhizis can be combined with splitting of bodies of vertebrae. At the rakhiskhizisa, especially full, malformations of a brain and other bodies often meet.

Spinal hernias represent gryzhevidny protrusions of a meninx, nervous roots or a spinal cord through a crack of a backbone. Depending on structure of hernial protrusion and an arrangement of cerebrospinal liquid (between covers of a spinal cord or in the central channel) distinguish several forms of spinal hernias: to a meningocele, a meningoradikulotsela, a myelomeningocele, a myelocystocele (see. Spinal cord, malformations ).

Spina bifida complicata — not fusion of arches of vertebrae which is combined with growths of fatty and fibrous tissue, and also a tumor like lipoma, fibroma, etc. The last are located under skin, carry out defect in arches of vertebrae, a meninx can burgeon and grow together with roots of spinal nerves and a spinal cord.

Spina bifida occulta — the hidden crack of arches of vertebrae. It is localized more often in the area I of a sacral vertebra. The ends of a nezarashchenny arch are quite often pressed into a gleam of the vertebral channel and cause a compression of its contents. At the level of not fusion of arches can come to light various patol. educations in the form of fibrous tyazhy, and also consisting of cartilaginous and fatty tissue. Find change and an intra dural bag — unusually low arrangement of a spinal cord, growth of fatty and fibrous tissue, an arachnoiditis.

Spina bifida anterior — nezarashche-ny bodies of vertebrae. Meets seldom, is generally rentgenol. find. It is localized more often in the lower cervical and upper chest vertebrae. Not fusion of bodies of vertebrae can be followed by splitting of a spinal cord, gryzhevidny protrusion of a meninx, roots and a spinal cord in a chest or abdominal cavity.

Clinical picture at Spina Bifida it consists of local changes and nevrol. frustration. At the level of not fusion of arches of vertebrae there is a gryzhevidny protrusion, a cut with age of the child can increase in sizes. At the same time its covers become thinner, ulcerate, there are likvorny fistulas. At the latent form of Spina Bifida skin at the level of not fusion of arches of vertebrae can be absolutely normal, or are available hypertrichosis (see), cicatricial changes, tail-shaped educations, etc. Nevrol. disturbances are most often connected with damage of a lower part of a spinal cord and horse tail, can meet in various combinations. Sluggish paresis or paralyzes of the lower extremities with an atrophy of muscles, disorders of sensitivity in a zone of an innervation sacral are noted, is more rare — lumbar roots. Dysfunctions of pelvic bodies are most often noted: incontience of urine and calla, paralytic locks, delay of an urination. Trophic and vasculomotor disturbances of the lower extremities can come to light. Decrease or lack of reflexes (knee, an akhillova, bottom), and also kre-masterny and proctal is noted. The latent form of Spina Bifida proceeds asymptomatically more often, however at it both symptoms of loss, and symptoms of irritation of a nervous system in the form of lumbosacral pains, hyperesthesias, paresthesias on the lower extremities, bed wetting, imperative desires to an urination can be observed. At Spina Bifida various deformations of feet are quite often observed, the clubfoot is more often. Cerebrospinal liquid at the latent form of Spina Bifida has normal structure.

Diagnosis. The hidden vertebral crevice (spina bifida occulta), as well as splitting of bodies of vertebrae (spina bifida anterior), hl reveal. obr. with the help rentgenol. researches. The big defects which are followed by loss of contents of the vertebral channel can be suspected at a wedge, inspection of the patient.

Fig. 2. Diagrammatic representation of different types of inborn crevices of a vertebra: 1,2 — back; 3,4 — side; 5, 6, 7 — lobbies.
Fig. 3. The tomogram of a part of lumbar department of a backbone at spina bifida anterior (a direct projection): bodies III and IV lumbar vertebrae have wide front crevices (are specified by shooters).

Localization of a crevice in a body or an arch of a vertebra can be the most various (fig. 2). Radiological the crevices oriented in the sagittal plane are easier distinguished to-rye are better visible on the roentgenogram in a direct projection. Crevices in side departments of arches and bodies are visible on roentgenograms in a side projection, and sometimes only on tomograms. At localization in bodies of vertebrae (spina bifida anterior) the crack divides a body of a vertebra into two parts, each of to-rykh has on the roentgenogram in a direct projection the wedge-shaped form. More often both parts of a body of a vertebra are symmetrized, remind by the form wings of a butterfly — a so-called babochkovidny vertebra. However wedge-shaped parts of a body of a vertebra can be also asymmetric (fig. 3). In the course of growth of a body of the next vertebrae adapt to a shape of abnormal vertebras.

Fig. 4. The roentgenogram of a part of chest department of a backbone at not fusion of the back site of an arch with lack of an acantha (a direct projection): the zone of not fusion of the back site of an arch is specified by an arrow.
Fig. 5. The roentgenogram of a part of lumbar department of a backbone (a direct projection) with symmetric crevices (are specified by shooters) in side sites of arches of the IV lumbar vertebra.

Defects in arches of vertebrae, especially lower lumbar and sacral meet more often. Not fusion of an arch on the centerline with splitting and an underdevelopment, and sometimes and lack of an acantha is the most typical. This localization of Spina Bifida easily comes to light on the roentgenogram in a direct projection (fig. 4). Less often the crevice is localized in an arch between joint shoots with one or on both sides — a spondylosis (fig. 5). Diagnosis of Spina Bifida of this localization is difficult if it is not combined with spondylolisthesis (see). At suspicion on a spondylosis the X-ray analysis in two projections is obligatory, and at insufficient informational content of roentgenograms the tomographic research in a side projection is necessary (see. Tomography ).

Fig. 6. The roentgenogram of lumbosacral department of a backbone at sharply expressed spina bifida: the enlightenment at the level of bodies of lumbar vertebrae and in a middle part of a sacrum is caused by absence of back departments of arches, acanthas and a back wall of the sacral channel.

At the expressed disturbances of development of arches of vertebrae on roentgenograms find their extensive defects with lack of the image of acanthas, back departments, and sometimes and joint shoots and even legs of arches (fig. 6). Detection of Spina Bifida shall induce to search of other manifestations of a spondilodisplaziya (local narrowings of the vertebral channel, intervertebral foramens, options of an arrangement of joint shoots, deformation of bodies of vertebrae, a thicket wedge-shaped, etc.).

Treatment — operative measure. At spina bifida complicata delete tumorous educations and allocate from them as far as possible nervous elements. After excision of a hernial bag close a crack in arches of vertebrae. For closing of defect in arches of vertebrae the modified method of muscular and fascial plastics offered for the first time by Bayer became conventional (To. Bayer, 1889). At the edges of defect in a backbone find two fascial rags of a semi-oval form with a small layer of muscles. Rags turn on 180 ° a fascia inside and sew yaad a stump of a hernial bag. This method, as a rule, interferes with a recurrence of spinal hernia. The method of plastics of big crevices in a backbone, especially in sacral department, at children of chest age by means of the homogeneous firm cover of a brain preserved by freezing deserves attention. In arches of vertebrae apply also periosteal and fascial rags, a polyethylene film, a protector from AKP-9 plastic to closing of defect. Operation at spina bifida occulta comes down to removal of the nezarashchenny and pressed into a gleam of the vertebral channel arches of vertebrae and all patol. the educations which are at this level. At suspicion on subdural changes it is recommended to open a firm cover of a spinal cord and to exempt a spinal cord from commissures, unions, growths of fatty tissue.

For recovery of the broken functions of c. N of page apply to lay down. physical culture, mud cure, fortifying therapy.

In postoperative period, especially after operations for spinal hernias, complications are possible: suppuration of a wound, a liquorrhea with the subsequent meningitis, accumulation of cerebrospinal liquid under a skin rag, pneumonia, acute development of hydrocephaly.

Forecast depends on a form and severity of defect. At a full rakhiskhizis fruits are impractical. At spinal hernias the most adverse forecast at patients with the miyelotsel and a myelomeningocele and substantially depends on localization of a cyst and a condition of a spinal cord. A timely and successful operative measure prevents development of complications, hl. obr. the ascending purulent infection, and also progressing nevrol. frustration; existing nevrol. changes usually remain. At other Spina Bifida forms, even with existence of secondary changes, operative measures give lasting positive effect, and sometimes lead to an absolute recovery.

Bibliography: Tops of River. The doctrine about tumors, the lane with it., t. 1, page 114, SPb., 1867; Gushchina JI. And., Aleksandrovich E. I. and Golovachev G. D. About the frequency of the hidden crevice of a backbone, Vestn. rentgenol. and radio-gramophones., No. 3, page 73, 1980; N. A. and Kargapoltsev G. V Calls. Plastics of crevices of a backbone a firm meninx at spinal hernias, Vestn. hir., t. 106, No. 6, page 72, 1971; To au-sinsky N. S. Disturbances of development of the bone and joint device, L., 1966; Krotova L. I., etc. The obstetric and gynecologic anamnesis of the women who gave birth to children with spinal hernia, Akush. and ginek., No. 9, page 56, 1979; Reynberg S.A. Radiodiagnosis of diseases of bones and joints, book 2, page 189, M., 1964; Solopayev A. A. and d river. About front sacral shell and brain hernias, Vopr. neyrokhir., No. 5, page 18, 1977; T and of e r I. L. and D I am a h e the Tax Code about V. A. Radiodiagnosis of diseases of a backbone, page 66, 68, M., 1971; A. M. K Clowns to a question of a patomorfologiya of brain and spinal hernias in combination with hydrocephaly, in book: Geographical patol. Uzbekistan, under the editorship of M. S. Abdullakhodzhayeva, page 73, Tashkent, 1975; By of e n n R. Moderne Meningomye-lorelen-Behandlung, Wien. med. Wschr., S. 18, 281, 1975; M errem G. Lehrbuch der Neurochirurgie, S. 330, B., 1970; W about with-j an J. Obraz kliniczny oraz wartosc leczenia operacyjnego wrodzonych rozsz-czepow kregoslupa (spina bifida) u dzieci, Warszawa, 1973.

R. I. Rostotskaya; P. L. Zharkov (rents.).