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Hand and foot remains from the Gran Dolina Early Pleistocene site (Sierra de Atapuerca, Spain).

We report here the study of the 22 hand and foot remains from the Early Pleistocene level TD6 of the Gran Dolina site at Sierra de Atapuerca (Burgos, Spain) recovered from 1994 to 1996. These remains are paratypes of Homo antecessor. All of the elements are briefly described and compared with other fossil hominids. The capitate has a constricted neck, well developed head, strong attachment for the ligamentum interosseum trapezoid-capitate, a palmarly placed trapezoid facet with a distinctive small dorsal trapezoid facet, a highly curved and oblique orientation of the second metacarpal facet, and a transversally oriented dorsodistal border. A hamate with a moderately projecting and lightly built hamulus; an inferred reduced styloid process on the third metacarpal base; a wide second metacarpal head; and middle phalanges with well marked insertions for the flexor digitorum superficialis muscle and wide heads. The morphology and dimensions of the pedal remains from TD6 are very similar to modern humans; but the base, proximal articular surface and shafts of the proximal hallucal phalanges are more rounded and the midshaft of the proximal toe phalanx is wider.

Age Determination by Skeleton↗

Olmsted syndrome: report of a case with study of the cellular proliferation in keratoderma.

Olmsted syndrome is a rare disorder that consists of sharply marginated keratoderma of the palms and soles, constriction of digits and toes that may result in spontaneous amputation of the distal phalanges, hyperkeratotic plaques around the body orifices, onychodystrophy, and other less common cutaneous and extracutaneous anomalies. Although some patients had other affected family members, most cases of Olmsted syndrome seem to be of sporadic occurrence. We describe a patient with the characteristic features of Olmsted syndrome. The symptoms consisted of diffuse transgrediens palmoplantar keratoderma and keratotic plaques around the mouth and nose. Our patient also had the associated anomalies of hyperhidrosis of the palms and soles and congenital deaf-mutism. Histopathologic study of the keratoderma demonstrated epidermal hyperplasia with acanthosis, papillomatosis, and orthokeratotic hyperkeratosis. Immunohistochemical study showed more basal and suprabasal keratinocytes of the epidermis with immunoreactivity for Ki-67 marker when compared with the keratinocytes of the epidermis of the adjacent non-involved skin. These results support the notion that Olmsted syndrome is a hyperproliferative disorder of the epidermis.

Adult↗

An autopsy case of Adams-Oliver syndrome.

We report an autopsy case of a male fetus with Adams-Oliver syndrome. His mother was a healthy, 31-year-old woman and her family and past histories were unremarkable. Therapeutic termination was done at 28(+6) weeks gestational age due to oligohydramnios detected by antenatal ultrasonography. Chromosomal study revealed normal karyotype. On autopsy, characteristic transverse terminal defect of four extremities was found. Both feet were short and broad. All toes were rudimentary with no nails and fingers were irregularly short. On infantogram, all toe-bones were stubby and rudimentary. The middle and terminal phalanges of 2nd, 3rd & 5th fingers and the terminal phalange of 4th finger on the right hand were absent. The middle and terminal phalanges of 2nd & 5th fingers and terminal phalange of 3rd finger were defected on the left hand. His abnormalities were consistent with features of Adams-Oliver syndrome, which has not been reported in Korea.

Abnormalities, Multiple↗

Brachydactyly type B: case report and further evidence for clinical heterogeneity.

We present a male child with digital and radiographic findings consistent with brachydactyly type B. His left hand had 2-4 syndactyly, shortened first and fifth digits and single palmar and fifth finger flexion creases. The nail on the second finger was hypoplastic. The right hand had a small thumb and curving of the second finger towards the midline. The left foot had a short left hallux with an absent nail, 2-4 syndactyly and an absent toe. His right foot had a short, dorsiflexed hallux with a hypoplastic nail, 2-3 syndactyly and an overlapping toe. Radiographs showed asymmetrical hypoplasia of the middle and terminal phalanges and there were no extra-articular findings to suggest Sorsby syndrome. Brachydactyly type B has classically been described as hypoplasia or absence of the terminal phalanges with absent nails. Although other digital anomalies have previously been described with brachydactyly type B, the absent digit, atypical syndactyly and marked asymmetry in this child make this case a good illustration of the clinical heterogeneity that can be associated with this type of brachdactyly. We provide a brief review of syndromes in which similar digital malformations have been reported.

Hand Deformities, Congenital↗

[Congenital onychodysplasia: Iso-Kikuchi syndrome].

We report the first German case of Iso-Kikuchi syndrome, which is characterized by a congenital onychodysplasia commonly involving the index fingers, but not infrequently also the neighbouring fingers, and typical bony alterations of the corresponding terminal phalanges. This is also the first report of associated great toe anomalies.

Adult↗

[Morphogenesis of the chick embryo foot as studied by Janus green B-induced malformations].

Janus green was injected into the amniotic sac of 6-5-day-old chick embryos at a single dose of 8-5 or 15 mug; the dye causes respectively 55 and 82% malformations of the feet. Toe are affected by partial or total soft tissue syndactyly, hypophalangy and infrequently by hyperphalangy. Lateral toes are more severely and more frequently affected by syndactyly and hypophalangy than medium ones. Hyperphalangy is found exclusively in toes I and II and causes the formation of one excess phalanx at most. Syndactylous toes are joined by an overdeveloped digital membrane. Hypophalangic toes have a truncated (non-pointed) distal tip. Two phalanges are lacking at most. These malformations are due to two distinct phases of the Janus green action. The first one, which is early and fast, inhibits interdigital programmed cell death, causing in term the non-regression of the interdigital membranes and thus syndactyly. The second one, which is late and slow, blocks the apical growth; this leads to hypophalangy.

Abnormalities, Drug-Induced↗

[Current treatment of static deformities of the great toe].

The modern techniques of treatment of the hallux valgus are founded on the biomechanic and permit to give again a good function of the big toe. The authors prefer the osseous methods and specially the phalangeal and metatarsal osteotomies. It is necessary also to do a lateral release of the metatarsophalangeal joint and an exostosectomy. The late results are good.

Adult↗

Atrial septal defect associated with Albright's hereditary osteodystrophy and other anomalies: a clinical case.

A 36 year-old woman with a history of asthenia and palpitations was admitted to the Cardiac Surgery Department of Tor Vergata University, in Rome. Physical examination revealed short stature, depressed nasal bridge, hypertelorism, hypoacusia, pectus excavatum, diffuse brachydactyly, clinodactyly of the second digit of both the right hand and left foot. A 3/6 holosystolic increasing-decreasing murmur on the pulmonary focus was present at cardiac auscultation. Echocardiogram and cardiac catheterization revealed an ostium secundum atrial septal defect. X-ray examination of the hands exhibited shortening of the third, fourth and fifth metacarpals, shortening of the distal phalanges, shortening of the proximal and middle phalanges of the fifth digits and cone epiphysis of the middle phalanx of the second digits. Radiograph of the feet revealed shortening of the third and fourth and metatarsals on the left side, bilateral shortening of the first metatarsals and of the distal phalanges, cone epiphyses at the proximal base of the first toes. Additional radiographic findings included pectus excavatum and narrowing of the spinal canal. Laboratory investigations disclosed increased plasma levels of parathormone and hypocalcemia. The patient underwent primary closure of the atrial septal defect on cardiopulmonary bypass. Radiographic findings supported the diagnosis of Albright's hereditary osteodystrophy. This is a skeletal malformation involving type I-A pseudohypoparathyroidism and so-called pseudo-pseudohypoparathyroidism. Coexistence of hypocalcemia and high levels of parathormone indicated that our patient was affected with type I-A. About one-fourth of congenital heart diseases are associated with extracardiac anomalies. Although skeletal malformations appear to be the most frequent, the association of a congenital heart defect with Albright's hereditary osteodystrophy has never been described before.

Adult↗

Thumb reconstruction with a big toe microvascular wrap-around flap: a report of fifteen cases.

Fifteen thumb reconstructions were performed using the wrap-around procedure. A free wrap-around flap was taken from the big toe, and included the nail, and a part of the distal phalangeal skeleton. An iliac bone graft was added as the core of the reconstruction. One patient had an abnormal metatarsal arterial pattern which made the microsurgical reconstruction impossible, and another patient had early venous failure. Thirteen cases with successful thumb reconstruction were evaluated at an average of 27 months. Sensibility based on moving two-point discrimination ranged from 6 mm to 10 mm, and key pinch strength ranged from 0.5 kg to 3 kg. None of the patients had the problem of bone graft resorption, cold intolerance or social unacceptance after surgery. A postoperative study of the feet by Harris mat revealed an area of increased pressure under the second metatarsal head and the big toe stump. None of the patients experienced difficulty when walking or running on level ground.

Adolescent↗

[A new method of thumb reconstruction using big and second toe elements].

The author's modification of thumb reconstruction using the big and second toe elements is presented. Two separate parts were raised on one common pedicle to form future thumb. The main element including bones, tendons and soft tissues were taken from the second toe. The flap comprising nail radix, matrix and lateral fragment of the second toe pulp was prepared and left in situ. Similar flap was raised on the hallux. Winding of the hallux flap round the second toe part allowed to form nearly normal thumb. The nail radix and matrix were harvested subperiosteally without any bone fragments of distal phalanges. Harvesting of minimum of tissues from the hallux and covering the wound by similar flap from the second toe markedly limited the big toe deformation, typical for this type of procedure. The nail plate of the second toe looked acceptable on the slightly thinner hallux. Scares were localized out of the weight bearing surfaces. Best indications for this procedure include cases when the thumb is amputated on the proximal phalanx level. Rare anatomical variation of the toe vascularity may restrict application of this method. Individual length differences between the hallux and the second toe sometimes make it difficult to put the hallux flap in the proper position on the thumb. A small hallux valgity may appear when the second toe is too short.

Humans↗

Idiopathic acro-osteolysis in an elderly woman. A 10-year follow-up.

We report a case of idiopathic acro-osteolysis in an 80-year-old, otherwise normal woman. The patient first presented at the age of 70 years with a 3-year history of progressive shortening of the left toes. There was no history of trauma or bone disorders. She had osteolysis of the left second to fourth distal phalanges. Angiography was normal. During follow-up over 10 years the osteolysis gradually progressed and spread to affect the right toes.

Age Factors↗

Osteochondral disruption of the second metatarsal: a variant of Freiberg's infraction?

A case of Freiberg's Infraction of the second metatarsal is presented. The metatarsal head was resected and its pathology proved very informative. Necrotic bone was not found, but a shearing type of separation was identified at the level of "tidewater mark" in the articular cartilage and was the major observation. This suggested the possibility that in adults Freiberg's Infraction rather than true avascular necrosis may be in fact the result of a shearing-compression type of injury occurring at the interface between mineralized and nonmineralized articular cartilage (tidewater mark). An anatomical examination of metatarsophalangeal joints in cadavers showed that during walking at the "toe off" position, the toes dorsiflex to force the metatarsal head into plantar flexion. The proximal phalanges ride dorsally over the metatarsal heads producing a shearing force that would support our interpretation of the observations made in the case presented.

Ankle Injuries↗

Salter Harris type II injury of the proximal phalanx of the fifth toe: case report.

Fractures of the growth plate are unique to childhood. The risk of growth disturbances depends on the type of fracture, its location, the age of the patient, the vascularization to the epiphysis, the state of the surrounding soft tissues, and whether the injury is open or closed. Epiphyseal fractures of the foot are uncommon, and only rarely are the phalanges involved. We report a Salter-Harris type II injury in the fifth toe. The patient, a 3-year-old girl, was successfully managed conservatively with no sequelae.

Child, Preschool↗

Madura foot in the U.K.: fungal osteomyelitis after renal transplantation.

We report the case of an ethnic Asian patient who attended the renal transplant follow-up clinic complaining of pain in the right great toe. He had undergone transplantation nine months earlier and was maintained on triple immunosuppression. Initially, a clinical diagnosis of gout was made and the patient treated with analgesia. Two weeks later he remained symptomatic and developed a discharging sinus on his toe. A plain X-ray revealed a lytic lesion with minimal periosteal reaction. Aspiration of his first right metatarsal phalangeal joint was performed and fungal hyphae were observed in the fluid. Subsequently, despite surgical debridement and treatment with Itraconozaole amputation of the toe was required. Microbiological analysis revealed the organism to be Madurella grisea,which was resistant to both Itraconazole and Amphotericin B. He has remained well since amputation. We believe this to be the first case of Madurella infection to be described in a transplant patient.

Amputation, Surgical↗