The term arthrogryposis suggested by Stern in 1923 means curved joints and has been used most commonly to describe a syndrome characterised by multiple deformities and rigid joints already present at birth. This condition has also been named arthrogryposis multiplex congenita, myodystrophia foetalis deformans, and amyoplasia congenita. Current reports classify arthro- gryposis into two forms, neuropathic and myopathic, based on pathogenesis. The characteristic deformities of the limb have been described in the neurogenic form in association with spina bifida and other defects of spinal development. In those cases not obviously linked with a spinal malformation, the basic neurological defect was found to be the absence of segmental anterior horn cells ( Adams, Denny-Brown and Pearson 1953; Brown, Robson and Sharrard 1980). The patient described here was born with two pairs of legs, one pair of which appeared arthrogrypotic and were removed by operation, together with part of the pelvis. The opportunity was taken to dissect the excised specimen and examine the tissues histologically. J. 0. Matthews, FRCS, Lecturer in Orthopaedic Surgery ) University Department of Orthopaedics, P. V. Lawford, BSc, Scientific Officer Floor I, Royal Hallamshire Hospital, T. Duckworth, FRCS, Professor of Orthopaedic Surgery J Glossop Road, Sheffield 510 2JF, England. Requests for reprints should be sent to Mr J. G. Matthews. © 1982 British Editorial Society of Bone and Joint Surgery 0301-620X182/1008-0088 $2.00 88 THE JOURNAL OF BONE AND JOINT SURGERY ARTHROGRYPOSIS IN CAUDAL DUPLICATION AN ANATOMICAL AND HISTOLOGICAL STUDY OF ARTHROGRYPOTIC LIMBS J. G. MATTHEWS, P. V. LAWFORD, T. DUCKWORTH From the University Department of Orthopaedics, Royal Hallamshire Hospital, Sheffield A case of caudal duplication with arthrogrypotic features in the parasitic limbs is reported. Anatomical and histological studies showed that development ofthe limbs had proceeded in the absence of persisting motor innervation. It is suggested that the characteristic deformities of neurogenic arthrogryposis are the product of an imbalance of the embryonic muscles, moukling in the uterus and unmodified patterns of growth. ‘ - ; . . .. ,: . ,fr Fig. 1 The appearance of the child after a temporary colostomy. MATERIAL AND METHODS The child had four legs (Fig. 1) of which the lateral pair appeared to be normally developed and innervated and displayed a full range of movements. The medial pair, which faced backwards, arose from a rudimentary parasitic pelvis that was attached face to face with the host pelvis; although well formed, they were smaller and immature and showed no clinical evidence of innervation. All the joints were stiff, with webbing of the skin which was tight, shiny, and lacked the normal skin creases. There was no anal orifice or meconium staining of either host or parasitic perineum. The genitalia of the host, which were displaced to the left, consisted of a penis that displayed a complete epispadias and a well developed scrotum containing a solitary left testis. A rudimentary penis arose from the right posterolateral aspect of the parasite. An oval-shaped mass was present anteriorly at the junction between two pelvic structures. The host umbilicus was displaced inferiorly and was represented by an omphalocele which had ruptured antenatally. No other visceral abnormality was present. The inital radiographs at the age of 10 hours showed wide separation of the symphysis pubis of the host with two normally articulating hips. The pelvis of the parasitic twin was represented by several pieces of bone and articulated with the smaller pair of legs (Figs 2 and 3). The legs and the rudimentary pelvis were removed en bloc. Two major sets of vessels supplying the specimen were ligated but no major nerve trunks were found. The parasitic hips were held in flexion, abduction and slight lateral rotation with regard to the rudimentary pelvis, the knees were flexed at right angles, and the feet held in moderate equinovarus. The thickness of the skin appeared normal and the nails were normally developed. Dimples were present over the extensor aspects of the knees. The flexion creases of the knees were obliterated by large skin webs. The hips appeared to be in joint and the knees were not obviously subluxated or dislocated. An arteriogram showed that the entire parasitic limb complex was supplied from the right internal iliac artery of the host. Dissection. The specimen was fixed in I0 per cent formol saline before dissection. Taking the rudimentary pelvis as a midline marker, the specimen was bisected sagittally. The half representing an anatomical right leg was carefully stripped of skin and dissected in segments, proximally to distally, and from the superficial layers to the bone or joint capsule, before finally dissecting the joints. The left leg was preserved, decalcified with Gooding and Stewart’s decalcifying agent and subjected to serial transverse sectioning from the proximal end to the distal end, omitting the knee and ankle, which were saved for separate orientation and sectioning. Wax sections were cut at 10 micrometres and stained using routine haematoxylin and eosin, Holme’s stain and solochrome cyanine.
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