The Anatomy of a Human Foot with Missing Toes and Reduplication of the Hallux*
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J. Anat. (1991), 174, pp. 1-17 1 With 10 figures Printed in Great Britain The anatomy of a human foot with missing toes and reduplication of the hallux* DAVID R. HOOTNICKtf, DAVID S. PACKARD, JRt, E. MARK LEVINSOHN§ AND DAVID A. FACTORII t Departments of Orthopedic Surgery, t Anatomy and Cell Biology, § Radiology, SUNY Health Science Center, Syracuse, New York and 11 Department of Medical Illustration, Mayo Clinic, Rochester, Minnesota, USA (Accepted 15 May 1990) INTRODUCTION Recent research has demonstrated a consistent association between a wide variety of congenital bony dysplasias of the human lower limb with the absence or reduction of the anterior tibial artery and its derivatives (Hootnick, Levinsohn, Crider & Packard, 1982; Hootnick, Levinsohn, Randall & Packard, 1980; Hootnick, Packard & Levinsohn, 1983a, b; Hootnick, Packard & Levinsohn, 1990; Packard, Levinsohn & Hootnick, 1990; Sodre et al. 1987; Sodre et al. 1990; Williams et al. 1983). Reduction or absence of the anterior tibial artery may be a risk factor for the development of bony dysplasias by reducing the number of vessels available for collateral circulation. Some event, such as extravasation of blood or embolisation, may subsequently or concurrently compromise blood flow in the remaining vessels, leading to tissue damage (Hootnick et al. 1984). We believe that the timing of the teratogenic event with respect to the specification and differentiation of limb structures determines the final morphology of the limb (Hootnick et al. 1990). The analysis of the anatomy of amputated limbs supports this view of limb teratogenesis (Packard et al. 1990). The arterial anomalies in these limbs differed from the other tissue abnormalities in that they were consistent and independent of the bony anomalies. The remainder of the soft tissues exhibited anomalies interpreted to be secondary to the bony anomalies. The present report is part of a series of limb dissections representing a variety of congenital bony malformations (Hootnick et al. 1983b; Hootnick, Packard, Levinsohn & Cady, 1987; Hootnick et al. 1984). The specimen described here came from a patient whose limb featured a short femur and tibia, an absent fibula, and an anomalous foot. After amputation of the foot, all of its soft tissues and bony elements were studied. To compare and contrast the abnormal anatomy in different birth defects, a brief description of the abnormalities found in this limb has already been presented along with the anomalies found in our previous dissections (Packard et al. 1990). The detailed anatomy of the specimen is presented in this paper. Clinical presentation Though there was no record of any previous congenital anomalies in the family, the patient had an abnormal right leg at birth (Fig. 1). He had a hip flexion contracture * Reprint requests to Dr David R. Hootnick, Department of Anatomy and Cell Biology, SUNY Health Science Center at Syracuse, Syracuse, New York 13210, USA. 2 D. R. HOOTNICK AND OTHERS t: 1:: N,t" .l..."' Fig. 1. Clinical photograph taken prior to surgery shows the foot with a cleft in the midline, two fused medial toes and two normal lateral toes. with an extremely short femur and a proximal femoral focal deficiency. A shortened tibia was anteriorly bowed in the midshaft, and the foot was cleft in the midline from the metatarsal heads distally. There appeared to be two fused medial toes and two normal lateral toes. Radiographs revealed that the right femur and tibia were only about one half of the length of the contralateral femur and tibia. The upper femur was more dense and Anatomy of a malformedfoot 3 Fig. 2. Neonatal radiograph of the leg and foot. This lateral radiograph shows an abnormally shaped tibia with an anteriorly angulated apex. The fibula is not evident. An ossified structure probably representing the calcaneus is present. A broader than normal medial metatarsal articulating with two medially directed toes is seen. These toes each demonstrate two phalanges. Two laterally positioned metatarsals are noted, each articulating with toes having three phalanges. rounded than usual. The fibula was radiographically absent. There were four toes in the foot (Figs. 2, 3). The patient underwent an ankle disarticulation (Symes amputation) at the age of 15 months. He is currently fully ambulatory with a prosthesis. 4 D. R. HOOTNICK AND OTHERS Fig. 3. Specimen radiograph following amputation. The anteroposterior radiograph shows hind foot bones corresponding to the calcaneus, cuboid and lateral cuneiform. A misshapen talus and an ossified structure consistent with the medial cuneiform is noted. A large, abnormally shaped medial metatarsal is present. Two toes articulate with that metatarsal; each demonstrates two phalanges. Two lateral metatarsals each articulate with toes demonstrating three phalanges. RESULTS The specimen consisted of a foot, without the heelpad, which had been amputated through the ankle at the level of the talus on its dorsal surface. There were four toes present. These were designated A for the medialmost toe, A' for the toe syndactylised with A, B for the next toe laterally and C for the lateralmost toe (Fig. 4). Anatomy of a malformedfoot 5 PT B A' Fig. 4. Drawing of a dorsal view of the amputated foot following removal of the skin. The distribution of the superficial peroneal and the sural nerves is shown. DL, deltoid ligament; EDL, extensor digitorum longus muscle; EHL, extensor hallucis longus muscle; PT, peroneus tertius muscle; SN, sural nerve; SPN, superficial peroneal nerve; TA, tibialis anterior tendon. Bones The talus, calcaneus, navicular, and cuboid bones (Figs. 7, 10) were fused into a single cartilaginous mass (a synchondrosis). The calcaneus appeared everted while the remainder of the foot appeared to be normally aligned. Two cuneiform bones of approximately equal size were present, one on the medial border of the foot and the other immediately lateral to it in the midline of the foot. The medial cuneiform articulated proximally with the medial aspect of the fused navicular in a normal fashion and distally with an oblique facet on the proximomedial aspect of the thickened medialmost metatarsal (Metatarsal A). The more lateral cuneiform articulated proximally with both the medial cuneiform and the navicular portion of the synchondrosis. Distally, the lateral cuneiform articulated with a lateral facet of Metatarsal A and more laterally with the central metatarsal (Metatarsal B). A single enlarged metatarsal (Metatarsal A) articulated with the medial cuneiform and the medial portion of the lateral cuneiform. The two more lateral metatarsals were designated B and C, the most lateral metatarsal being C. Metatarsal B articulated with the lateral aspect of the lateral cuneiform. Metatarsal C articulated with the distal aspect of the cuboid portion of the synchondrosis. The proximal phalanges of the two syndactylised toes, A and A', articulated with the distal aspect of Metatarsal A. Each of these toes had two phalanges. Toes B and 6 D. R. HOOTNICK AND OTHERS B Fig. 5. Drawing of a dorsal view of the amputated foot. The extensor muscles are shown. AbDM, abductor digiti minimi muscle; DPN, deep peroneal nerve; EDB, extensor digitorum brevis muscle; EDL, extensor digitorum longus muscle; EHL, extensor hallucis longus muscle. C each contained three phalanges that articulated with Metatarsals B and C in a fashion typical of Toes 2-5. Nerves The superficial peroneal nerve was identified at the level of the ankle as it entered the dorsum of the foot where it immediately formed medial and lateral divisions (Fig. 4). As the lateral division of the superficial peroneal nerve passed distally, it received a communication from the sural nerve. Near the midpoint of Metatarsal B, it divided into three branches. The lateralmost two branches supplied the skin of Toe B and the skin of the medial surface of Toe C. The most medial branch communicated with a branch of the medial division of the superficial peroneal nerve. Near the proximal end of Metatarsal A, the medial division of the superficial peroneal nerve divided into two branches; the first and more medial branch then subdivided into several small branches that appeared to supply the skin of the medial surface of Toe A (not shown). The lateral branch of the medial division of the superficial peroneal nerve united with the medialmost branch of the lateral division to run to the region of the cleft. The sural nerve was located on the dorsolateral border of the foot. It passed to the lateral aspect of Toe C, after having sent a communicating branch to the lateral division of the superficial peroneal nerve. The deep peroneal nerve was located dorsal to the talar neck and deep to the more Anatomy of a malformedfoot 7 B A' Fig. 6. Drawing of the specimen following deep dissection of the extensor muscles on the dorsum of the amputated foot. AbDM, abductor digiti minimi muscle; DL, deltoid ligament; DPN, deep peroneal nerve; EDB, extensor digitorum brevis muscle; TA, tibialis anterior tendon. medial long extensor tendons in the foot. The nerve divided immediately distal to the level of the amputation. A motor branch passed laterally to terminate in the proximal part of the extensor digitorum brevis muscle (Figs. 5, 6). A cutaneous branch ran distally dorsal to Metatarsal A to divide into two branches that supplied the skin of both sides of the cleft between toes A' and B. On the plantar surface of the foot, the medial plantar nerve was identified distal to the level of the heel pad amputation in the first layer of muscles (Fig. 8). At the distal tarsal level, it divided into three branches. The medialmost branch ran to the distal end of Metatarsal A and divided into multiple branches to the skin of Toe A and to the skin of the medial surface of Toe A'. The small central branch of the medial plantar nerve terminated ventral to the proximal end of Metatarsal A.