Köhler's Disease

KÖHLER'S DISEASE (A. Köhler). Köhler's disease I. In 1908, Köhler first published 3 cases of a peculiar disease of the navicular bone of the foot. The disease is not common (about 150 cases have been published in the literature); it appears in children aged 3-10 years (most often 5-6 years) and manifests as pain on the dorsal side of the foot corresponding to the navicular bone. The pains are usually not very severe, but still interfere with walking; they sharply increase with pressure. Some swelling of the dorsal side of the foot is often observed. Cases of bilateral disease are not uncommon. Boys are most frequently affected. The disease is clinically difficult but radiologically very easy to recognize; the picture is so typical that it cannot be confused with any other disease. On the X-ray picture, a sharp decrease in the bone nucleus is visible, it takes an elongated, narrow shape, its contours are sometimes uneven. No structure of the bone nucleus is visible, it is sharply compacted. This sharp compaction of the nucleus should be considered the main sign of this disease. The spaces between the navicular bone and neighboring bones are widened. Sometimes two sharply compacted nuclei are visible. The disease is not prolonged, the pains and other clinical phenomena usually pass within several weeks or months, and on the X-ray film there is almost always a completely normal picture after 1-2 years. Only one case is known in the literature in which a stable deformity remained after recovery. The nature of the pathological-anatomical process in this disease has not yet been finally clarified, since surgery is performed only very rarely. Most authors assume that the basis of the disease is bone necrosis, although not all microscopic studies confirm this view. Subsequently, fibrous proliferation, resorption of dead tissue and complete bone regeneration occur. Therefore, most authors classify this disease among the large group of necrotic osteopathies appearing in adolescence (Perthes' disease, Köhler II, Kienböck-Paiser's disease, etc.), and this opinion is confirmed by the very similar clinical and X-ray pictures of all these diseases. As for the etiology of the disease, it is not fully clarified. The assumption of traumatic origin has not been confirmed by microscopic examination: no traces of trauma in the form of bone fractures or individual trabeculae were found. In the anamnesis, trauma is present only in a relatively small number of cases. One must therefore assume that trauma can play only a role as a precipitating factor. Rather, overload of the foot and in particular of the navicular bone can be given importance. The latter is located between other bones in such a way that overload should particularly adversely affect it. In addition, the blood supply to the navicular bone is incomparably worse than the nutrition of neighboring bones. And finally, if one takes into account that the ossification nucleus in the navicular bone appears later than in the other bones of the foot, then it really suggests that overload in this easily vulnerable bone can lead to irregularities in ossification or even necrosis. This is further confirmed by the fact that this disease is often observed in weak, poorly developed children. The possibility of genotypic conditioning of K.d. cannot be excluded. This is evidenced at least by the case of Nieden, who radiologically found in the brother of a patient suffering from K.d. the same anomaly of the navicular bone, although no clinical manifestations were present in him. Both sisters of the patient were healthy. Treatment should be conservative: if there are any deformities of the foot (flat foot, etc.), they should be eliminated by appropriate methods; in addition, "rest and heat in all its forms are applied. Köhler's disease II. This disease was first described by Freiberg in America as a fracture of the head of the 2nd metatarsal bone and then at the end of 1914 by Köhler, who drew attention to certain peculiarities of the disease that aroused general interest, why this disease was named Köhler's disease II. The disease appears almost always at the age of 10-20 years, 3 times more often in women than in men. The disease is not very rare, up to 200 cases have been published in the literature. Patients complain of pain in the front part of the foot, increasing during walking. Objectively, limited tenderness is found in the area of the head of the 2nd, less often the 3rd and very rarely the 4th metatarsal bone. Occasionally both feet are affected. If the disease is still in the acute stage, there is some swelling in the area of the diseased bone, sometimes extending further to the entire dorsum of the foot. Redness of the skin is noted much less frequently. Usually, especially in the late stages, it is possible to feel an enlarged, often deformed head. The painful process lasts 2-2½ years and usually ends favorably, although with more or less marked deformation of the diseased head. The pathological-anatomical picture of this disease was clarified by Axhausen (6 operated cases), whose opinion was subsequently confirmed by a number of works by other authors [Heitzmann, Engel (4 operated cases); Holst and Handrikov (7 operated cases) and others]. Axhausen distinguishes 5 stages of the disease. In the first stage (figs. 6 and 7) he found complete necrosis of the entire epiphysis and its bone marrow. The cartilage is unchanged. In addition, proliferation of connective tissue originating from the periosteum of the metaphysis and breaking through the epiphyseal cartilage into the epiphysis was found, where resorption and regeneration of the dead bone occurs. No indentations or fractures of the bone or individual trabeculae are noted anywhere. The first stage lasts no more than 10-12 weeks, usually less. In the 2nd stage the same histological picture is found and in addition an impression fracture of the head. There are many fragments of trabeculae, sometimes crushed to bone meal and filling the bone marrow canals. This impression fracture is usually located on the lower surface of the head, and sometimes also on the dorsal surface (3 cases of Holst and Handrikov). In the 3rd stage only small necrotic areas remain; the bone marrow cavities are partly filled with dead marrow, partly with bone fragments. The dead part of the epiphysis is delimited from the rest by proliferating connective tissue along the plane. In other parts of the epiphysis there is living bone tissue, but the reticular structure indicates its recent regeneration. In the 4th stage (fig. 8) almost the entire bone has regenerated; only occasionally small necrotic foci are found. The cartilage is alive, deformed in places. Finally in the 5th stage there is a typical picture of deforming arthritis, which also involves the head of the phalanx. The X-ray picture appears as follows: in the 1st stage (fig. 1) no changes are yet visible on the film. In the 2nd stage (fig. 2) the articular surface of the head is flattened, sometimes even concave, the joint space is widened, the parts of the head near the articular surface are usually compacted. In the 3rd stage (fig. 3) focal compactifications are noted, apparently caused by sequestra in the epiphysis, which appears significantly flattened and widened. Sometimes freely lying sequestra are visible. In this stage, bone protrusions (exostoses) form in the lateral parts of the epiphysis. The metaphysis, sometimes also part of the diaphysis, are thickened. The 4th stage (fig. 4) is characterized by a sharp deformation of the head, which is even more pronounced in the 5th stage (fig. 5), when there is a typical picture of deforming arthritis, i.e. the process also involves the base of the phalanx. The thickening of the metaphysis decreases in this stage. Opinions regarding the origin of this process differ considerably. Thus, Kappis and others assumed a primary fracture with subsequent necrosis. Microscopic examinations of cases in the 1st stage proved the incorrectness of this opinion. Other authors assumed fibrous osteitis (Klett and Engelke), late rickets (Fromme), primary deforming arthritis (Hühne) and even tuberculosis (Greenwood). All these assumptions are incorrect and caused by the fact that the authors had no more than 1-2 specimens at their disposal. All authors who have examined a large number of cases agree with Axhausen's opinion. There is primary necrosis; the necrotized and therefore little stable tissue easily yields to external mechanical influences, which explains the impression fractures. From the side of the periosteum of the metaphysis, proliferation of connective tissue begins (which explains the thickening of the metaphysis); from here the regeneration of the bone tissue of the epiphysis begins. But due to the overcrowding of the bone canals with fragments, regeneration is delayed; therefore the head undergoes further indentations and deformation. When the connective tissue breaks through the epiphyseal cartilage, a bone ridge is formed. As for the mechanism of necrosis occurrence, this question is far from being clarified. Microscopic studies give the right to assume that necrosis is most likely a consequence of impaired bone nutrition. Axhausen assumes that this impaired nutrition is caused by mycotic non-infectious or slightly infectious thrombosis of the terminal vessels. Most authors do not agree with this last opinion.

Many authors attach great importance to trauma, especially in the sense of prolonged pressure, overloading or improper loading of the foot (long marches, high heels, flat foot, etc.). According to Pirr (Raug), mechanical moments can cause stretching (high heels, claw-like position of the toes) and compression of blood vessels until their complete closure with subsequent ischemia and necrosis. Some authors [Holst and Handrikov, König and Rauch] found in their specimens obliterating endarteritis and believe that this endarteritis is a consequence of stretching of the vessels (according to Pirr) and in turn causes necrosis of the bone. According to the nature of the course of the process, the X-ray and path-anat. picture, this disease undoubtedly belongs to the group of bone diseases recently discovered with the help of X-rays in adolescence: head of the femur (Perthes), navicular bone of the foot (Köhler I), navicular and lunate bones of the wrist (Kienböck-Peiser), etc. Sonntag calls the entire group of diseases osteochondritis juvenilis deformans (incorrectly, since there is no inflammatory process), Burckhardt - epiphysitis juvenilis; Reinberg - osteochondropathia juvenilis, and Holst - osteopathia juvenilis necroticans. Treatment, bearing in mind that the outcome of the disease is usually favorable, is preferably conservative. First of all, those harmful factors that can cause the disease should be eliminated, e.g. high heels. In case of flat foot - appropriate supinators. In addition, rest is prescribed. For pain, warmth, massage, etc. are recommended. Usually such treatment leads to a favorable result. As for surgery, it must be remembered that resection of the head of the 2nd metatarsal deprives the foot of one of the main points of support; therefore such an operation can lead to disease of the head of the 3rd or 1st metatarsal bones, onto which the point of support is transferred (such cases have been observed). Thus, resection should generally be considered not indicated. But there are severe cases where severe pain persistently does not pass for a long time. This usually concerns cases of the 5th stage, and in these cases surgery is permissible. But in this case, the bones must be spared as much as possible and only severely deformed parts should be removed, and the entire head should not be resected if possible. A partially removed head can regenerate. 18 Figure 1. Köhler's Disease II. 1st stage. No changes in the bones are noted on the X-ray. Figure 2. Köhler's Disease II. 2nd stage. The head is flattened; the joint space is widened; the structure of the head in the distal part is densified. Figure 3. Köhler's Disease II. 3rd stage. The head is sharply flattened, deformed; focal densifications of the head. Figure 4. Köhler's Disease II. 4th stage. Severe deformation of the head; its flattening; focal densification; thickening of the metaphysis. Figure 5. Köhler's Disease II. 5th stage. Picture of deforming arthritis; severe deformation of the head and base of the phalanx; exostosis; thickening of the metaphysis is less pronounced. Figure 6. Sagittal section through the head of the metatarsal bone. 1-2nd stages of Köhler's disease (enlarged by a magnifying glass). The cartilage is unchanged. Most of the epiphyseal bone is destroyed, replaced by connective tissue, which grows into the epiphysis from the periosteum of the metaphysis. Figure 7. 1st stage of Köhler's disease. Fragments of bone trabeculae, bone cavities are empty, giant cells, lacunar resorption, bone necrosis. The bone marrow is also necrotic. The invading connective tissue. Figure 8. Köhler's Disease II in the 4-5th stage. Sagittal section through the head of the metatarsal bone (enlarged by a magnifying glass). The structure of the bone is almost everywhere restored again. A large lateral ridge on the dorsal surface, further forward - a depression, in which lies a free piece of cartilage, under the depression - a densified area with connective tissue. The entire head is bent downward. (For illustration of the article Köhler's disease.)