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PubMedDate not supplied

Digital "V" arthrodesis.

Numerous procedures have been proposed through the years for the treatment of digital hammertoes and other related structural deformities. Experience has determined that many of these procedures are structurally incorrect and result in secondary deformity, which are often worse than the pathology initially addressed. Phalangeal base resections were found to destroy the structural stability provided by the intrinsic musculature. Phalangeal head resections make the toe impressionable to the extrinsic forces of the adjacent toes often leading to structural mal-alignment, or additional contracture. Similarly, indiscriminate tendon lengthenings have led to flail toes and a lack of purchase and propulsion. The advent of proximal interphalangeal joint arthrodesis demonstrated that structural stability and propulsion could be maintained while enhancing alignment. The "V" arthrodesis, as introduced by the authors, provides an alternative surgical approach to digital fusions. This approach is technically less difficult compared with a peg-in-hole fusion and provides enhanced stability when compared with an end-to-end arthrodesis. The preliminary results of this procedure appear to be most promising and are provided to the readership for review and examination.

Arthrodesis↗
PubMed1993

[Brachydactyly type A4 (brachymesophalangia II and V, Temtamy type). A rare type of brachydactyly].

Brachydactyly is classified into 5 main types. In type A brachydactylies the shortening is mainly confined to the middle phalanges of the digits and toes. Brachydactyly type A4 is characterised by brachymesophalangy II and V. We found this rare type of brachydactyly in a child and his mother. We examined the hands and feet of these individuals radiologically. The reported malformation had affected members of 2 respectively 3 generations. There is no doubt about the autosomal dominant mode of inheritance.

Bone Diseases, Developmental↗
PubMed1998

Two sisters with growth failure, microcephaly, peculiar facies and apical dystrophy: the presentation of brachymorphism-onychodysplasia-dysphalangism syndrome?

We report two sisters with growth failure, relative microcephaly, peculiar facies and apical dystrophy (brachydactyly type B). They had shortness and clinodactyly of the 5th fingers, aplasia or hypoplasia of the distal phalanges of 5th fingers, short medial phalanges of the 2nd and 5th fingers, hypoplasia or aplasia of distal phalanges of 2-5th toes, with tiny toenails, and aplasia or nails of 5th fingers and right 5th toe in the younger sister. Dysmorphic facial features included high forehead, sparse hair, blepharophimosis, telecanthus, epicanthic folds, a low nasal bridge, a broad nasal tip and micrognathia. Their ears were low-set and malformed. The older sister additionally had a high-pitched voice and eczema on the face and limbs. In the younger sister a cardiac defect was diagnosed--ventricular and atrial septal defect, patent ductus arteriosus. They had some clinical features of Coffin-Siris syndrome, but with a milder phenotype and much less severe mental handicap. Their clinical picture resembles more the brachymorphism-onychodysplasia-dysphalangism (BOD) syndrome.

Abnormalities, Multiple↗
PubMed1976

[Hereditary symphalangism: observations on this syndrome in a newborn infant and his mother (author's transl)].

Symphalangism is the partial or complete bony fusion of phalanges in the fingers and toes due to an autosomal dominant gene, with inherited failure of development of one or more interphalangeal joints. Two patients--a newborn baby and his mother--both revealing this rare malformation, are presented in this paper and the clinical and radiological features are described and discussed.

Adult↗
PubMed1992

Macrodystrophia lipomatosa: radiographic observations.

23 cases of macrodystrophia lipomatosa (MDL) are reported showing a wide spectrum of radiographic findings. Typical findings were hypertrophy of all the mesodermal tissues of the affected digits with dramatic overgrowth of fat. Phalanges were enlarged both in length and transverse diameter, but the trabecular pattern was maintained. In one patient, the phalanges and metatarsals were elongated but thinned. In another case, all the phalanges and metatarsals of the great toe were small. The little toe was also involved in two cases. Articular surfaces were slanting. There was a high incidence of palmar and plantar involvement. In a few cases the forearm and leg were also involved. Other uncommon features observed were early maturation of epiphyseal centres of ossification of phalanges and metatarsals, syndactyly, polydactyly, brachydactyly and symphalangism. Angiography was uncharacteristic.

Adolescent↗
PubMed2003

Fgf signaling controls the number of phalanges and tip formation in developing digits.

Tetrapods have two pairs of limbs, each typically with five digits, each of which has a defined number of phalanges derived from an archetypal formula. Much progress has been made in understanding vertebrate limb initiation and the patterning processes that determine digit number in developing limb buds, but little is known about how phalange number is controlled. We and others previously showed that an additional phalange can be induced in a chick toe if sonic hedgehog protein is applied in between developing digit primordia. Here we show that formation of an additional phalange is associated with prolonged Fgf8 expression in the overlying apical ridge and that an Fgf Receptor inhibitor blocks its formation. The additional phalange is produced by elongation and segmentation of the penultimate phalange, suggesting that the digit tip forms when Fgf signaling ceases by a special mechanism, possibly involving Wnt signaling. Consistent with this, Fgfs inhibit tip formation whereas attenuation of Fgf signaling induces tip formation prematurely. We propose that duration of Fgf signaling from the ridge, responsible for elongation of digit primordia, coupled with a characteristic periodicity of joint formation, generates the appropriate number of phalanges in each digit. We also propose that the process that generates the digit tips is independent of that which generates more proximal phalanges. This has implications for understanding human limb congenital malformations and evolution of digit diversity.

Animals↗
PubMed1995

Anatomic considerations of the human nail.

The nail unit is distinctive for human beings. Developmentally, the fetus demonstrates cranial and caudal paired anlage of the upper and lower extremities. The leg bud develops a foot plate and eventually toe rays that are first notched, later webbed, and then develop into separate toes. Distal on the toe, the primitive nail unit appears and eventually moves dorsal. When developed fully, the nail unit consists of the nail plate, nail bed, ungualabia, eponychium, hyponychium, lunula, and nail matrix. These structures are associated intimately with the terminal or ungual phalanges of the foot's five toes.

Adult↗
PubMed2005

[Value of the radiographic criteria "fingers and toes" in the early diagnosis of psoriatic arthritis].

PURPOSE: To assess the value of Fournie's radiological criteria "fingers and toes" in the early diagnosis of psoriatic arthritis. MATERIALS AND METHODS: 47 patients with psoriatic arthritis were studied retrospectively. Clinical and radiological features at the hands and fingers were assessed. Radiographic analysis was performed using Fournie's criteria. RESULTS: Of 47 patients studied, 25 patients developed hand and finger involvement. Asymmetrical joint distribution was observed in 76% of cases. Distal interphalangeal arthritis was noted in 60% of cases. Nail lesions were present in 11 cases and dactylitis in 3 cases. Eight patients had erosions of the distal interphalangeal joints. Osteolysis with pencil-in-cup deformity was noted in 3 cases. Ankylosis of interphalangeal joints was found in 7 cases and acro-osteolysis in 3 cases. CONCLUSION: Psoriatic arthritis frequently affects the small joints of the hands and fingers. Asymmetrical and distal interphalangeal joint involvement are a characteristic feature of this arthropathy. Some radiological lesions are suggestive and contribute to the early diagnosis of psoriatic arthritis.

Adult↗
PubMed1994

Idiopathic adult hallux varus.

Adult hallux varus is an uncommon clinical entity usually caused by an inflammatory arthropathy or overcorrection during bunion reconstruction. We present five cases of unexplained spontaneous hallux varus. Clinically, all patients initially were found to have flexible deformities, and no evidence of underlying inflammatory disease or history of trauma. Symptoms were easily relieved with shoe wear modifications. Two of the patients developed more rigid deformities and subsequent medial callusing of the first toe and varus deformities of the lesser toes. These two patients eventually required surgical intervention. A Keller arthroplasty was performed on the first metatarsophalangeal joint and valgus proximal phalangeal osteotomies were done on the lateral toes when indicated. The outcome for these patients was good. Possible etiologic mechanisms are discussed. It is postulated that in these cases, the abductor hallucis tendon inserts more medially on the phalanx and overpowers the adductor. This produces a medially directed moment and, with time, a varus deformity.

Female↗
PubMed1996

Case report: hypoglossia-hypodactylia syndrome.

A case of hypoglossia-hypodactylia syndrome is described. The infant was characterized by micrognathia, accompanied by a defect of the gingiva, and hypoplasia of the tongue. There was defect in the right arm below the elbow. The right foot manifested syndactyly of the great, second and third toes and osseous fusion of the first and second metatarsi and proximal phalanges. In the left foot brachydactyly of the great and fifth toes, syndactyly of the second, third and fourth toes, hypoplasia of the first and second metatarsi and defect in the first phalanx were evident.

Abnormalities, Multiple↗
PubMed1996

Variant of Coffin-Siris syndrome or previously undescribed syndrome?

We describe a 23-year-old woman with growth and mental retardation, hypoplasia of the nails and distal phalanges, particularly of the fifth fingers and toes, hirsutism, and a "coarse" face with large mouth and large tongue, and bushy eyebrows. Follow-up from birth to adulthood showed that developmental delay and hypoplasia of nails and distal phalanges are permanent signs. Sparse scalp hair, hypotonia, and feeding difficulties were present in early infancy. Later, growth retardation, hirsutism, and a "coarse" face with midface hypoplasia, broad nose, and large mouth became more impressive. Differential diagnosis includes a number of conditions, particularly Coffin-Siris syndrome, which is the most likely but not completely convincing diagnosis. Therefore, this woman might represent a variant of Coffin-Siris syndrome or a new entity.

Abnormalities, Multiple↗
PubMedDate not supplied

Diaphyseal lengthening for shortness of the toe.

A method of bone lengthening for bilateral shortness of the fourth toe in a 23-year-old woman is described. The symptomatic short fourth toes were successfully treated by metatarsal and phalangeal diaphyseal lengthenings with metatarsophalangeal joint preservation.

Adult↗
PubMed1990

[Current status of amputation surgery].

Limb salvage by revascularisation of occluded arterial pathways is main goal of vascular surgical activities. However, if dying or gangrenous tissue has to be removed, the question of: where to amputate--and the potential rehabilitation has to be answered. Septic amputation being an emergency operation has to be compared with elective one with definitive shaping of the stump. In major amputations knee disarticulation is preferred nowadays as being mostly "atraumatic" and well able for rehabilitation. In minor amputations bunions of the great and the little toes should be preserved especially in case the whole phalangeal and metatarsal forefoot amputation seems to have better results than removal of the first of fifth toe together with their metatarsia. A distal borderline amputation can only be performed in diabetics and after revascularisation of the feeding arterial tree.

Amputation, Surgical↗
PubMed1999

Robinow (fetal face) syndrome: report of a boy with dominant type and an infant with recessive type.

The cases of two patients with Robinow fetal face syndrome, an 11-year-old Thai boy and a newborn Caucasian girl, are described. The Thai boy had the characteristics typical of the dominant type of the syndrome with a few newly recognized signs, including communicating hydrocephalus, underdeveloped sinuses, short roots of the teeth, narrow and thick-floored pulp chambers, hypoplastic nipples, absent middle phalanges of the second to fifth toes, cone-shaped epiphyses of the second and fourth fingers and fifth toes, single creases of the fourth and fifth fingers, clinodactyly of the third fingers, dysmorphic umbilicus, and shawl scrotum. The girl had anomalies typical of the recessive type of the syndrome. She also had capillary hemangioma at the tip of her nose and hypoplastic fourth metatarsal bones, which are the newly recognized features of the recessive type. Infrequently reported clinical manifestations of the syndrome are discussed.

Abnormalities, Multiple↗
PubMed1987

Dominant inheritance of a syndrome similar to Rubinstein-Taybi.

Two sibs and their mother were affected with a syndrome like the Rubinstein-Taybi syndrome. All three individuals had broad terminal phalanges of the thumbs and great toes, antimongoloid slant of the palpebral fissures, and characteristic facial appearance with beaked noses. Four sibs of the mother had broad thumbs, suggesting a familial pattern. So far there has been no report of dominant transmission of the Rubinstein-Taybi syndrome. This family bears close resemblance to the Rubinstein-Taybi syndrome, which is transmitted as an autosomal or X-linked dominant trait. Inasmuch as the expressivity of the syndrome may be variable, we propose that a thorough family investigation is indicated in all apparently sporadic cases of Rubinstein-Taybi syndrome. Familial cases may be more common than realized and require appropriate genetic counseling.

Abnormalities, Multiple↗
PubMed2006

Diffuse and severe ischemic injury of the extremities: a complication of umbilical vein catheterization.

We report a case of severe, peripheral, and diffuse tissue ischemia after umbilical vein catheterization (UVC) in a preterm newborn born to a preeclamptic mother. Nitroglycerin ointment was used to treat lesions. The recovery was good but partial loss of the distal phalange of one finger and one toe occurred. This is the first report of peripheral vasospasm occurring after UVC. Topical nitroglycerin, traditionally used to treat peripheral artery catheter-induced ischemic injury, may be useful to treat the same lesions occurring after UVC.

Adult↗
PubMed1996

Anatomy, physiology, and functional restoration of the thumb.

The essential prehensile nature of the human hand rests on the presence of a mobile, sensate thumb with adequate stability and length. The true significance of the thumb-to-hand function is variable and dependent on a person's vocation, expectations, and needs. The frequently stated opinion that the thumb represents 40% of hand function is too exacting and does not allow for flexibility in evaluating a patient's requirements after thumb injury or loss. It is our approach to consider each patient's specific needs for individualized planning of thumb reconstruction. The patient can often offer useful information regarding need for strength vs. precision, width of hand vs. requirements for fine motor function, and concern for the aesthetic nature of an abnormal thumb vs. the variable deformities resultant from thumb reconstruction. We offer a review of the basic anatomy and physiology of the human thumb, with emphasis on hand-and-thumb function. We present the alternatives for thumb reconstruction, the advantages and disadvantages, and the relationships to a specific patient's needs. Clinical examples of various methods of thumb reconstruction, including metacarpal lengthening, phalangization, osteoplastic reconstruction, pollicization, and toe-to-hand transfer are provided.

Amputation, Traumatic↗