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Biomedical subjects

R Aldegheri

Publications and source records attributed to R Aldegheri.

At least 19 recordsLinked to original sources
PubMed2001

Limb lengthening in short stature patients.

A series of 140 patients with short stature operated on for limb lengthening (80 had achondroplasia, 20 had hypochondroplasia, 20 had Turner syndrome, 10 had idiopathic short stature due to an undemonstrated cause, 5 regarded their stature as too short, and 5 had a psychopathic personality due to dysmorphophobia that had developed because of their short stature) was reviewed. All patients underwent symmetric lengthening of both femora and tibiae; 10 of these achondroplastic patients underwent lengthening of the humeri. We carried out the 580 lengthening procedures by means of three different surgical techniques: 440 callotasis, 120 chondrodiatasis and 20 mid-shaft osteotomy. In the 130 patients with a disproportionate short stature, the average gain in length was 18.2 +/- 3.93 cm: 43.8% had complications and 3.8% had sequelae; the average treatment time was 31 months. In the 10 patients with proportionate short stature, the average gain in length was 10.8 +/- 1.00 cm: 4 experienced complications and none had sequelae; the average treatment time was 21 months. Patients who underwent lengthening of the upper limbs experienced an average gain in length of 10.2 +/- 1.25 cm: the average treatment time was 9 months and none of them experienced any complications or sequelae. The authors discuss how difficult it is to achieve the benefits of this surgery: they underline the strong commitment on the part of the patients and their families, the time in the hospital, the number of operations and, above all, the severity of those permanent sequelae that occurred.

Achondroplasia↗
PubMed2000

Painless fractures and thermoregulation disturbances in sensory-autonomic neuropathy: electrophysiological abnormalities and sural nerve biopsy.

We describe the case of a young girl suffering from thermoregulation disturbances, painless fractures and arthropathy since early childhood. The patient was diagnosed as having a hereditary sensory autonomic neuropathy. Although needle EMG, conventional nerve conduction studies and somatosensory evoked potentials gave normal results, sympathetic skin responses (SSRs) were absent. Sural nerve biopsy showed a substantial reduction in the number of small myelinated and unmyelinated fibers. We emphasize the importance of SSR testing in revealing a condition which is otherwise difficult to identify by electrophysiological techniques. The combined evidence of functional and morphological findings is strongly suggestive of selective peripheral nerve involvement.

Adolescent↗
PubMed1999

Distraction osteogenesis for lengthening of the tibia in patients who have limb-length discrepancy or short stature.

BACKGROUND: This study was performed to determine the safety and effectiveness of lengthening of the tibia, in patients who have a limb-length discrepancy or a short stature, with use of distraction osteogenesis, a technique based on the principle of distracting the callus that is formed after a subperiosteal osteotomy of the proximal portion of the diaphysis of a long bone. METHODS: A total of 230 tibial lengthening procedures were done in 150 patients. Seventy procedures were performed because of a limb-length discrepancy, which was secondary to trauma (thirty limbs), congenital fibular hemimelia (twenty-six), poliomyelitis (ten), or infection (four). The remaining 160 procedures were performed because of a short stature, which was secondary to achondroplasia (fifty-eight limbs), Turner syndrome (thirty-four), an idiopathic etiology (twenty-two), hypochondroplasia (twenty), achondroplasia (ten), Ellis-van Creveld syndrome (six), rickets (four), or adrenogenital syndrome, Laron syndrome, or pseudoachondroplasia (two limbs each). The age of the patients at the time of the operation was 18.4+/-6.2 years (average and standard deviation), with a range of six to forty-one years. The procedures were performed according to one of three protocols. In Group A (ninety procedures), an Orthofix telescopic fixator and a variable number of screws were used and the tibiofibular syndesmosis was not stabilized; in Group B (ninety-six procedures), an Orthofix reconstruction system was used, the syndesmosis was stabilized, and a tenotomy of the Achilles tendon was performed; and in Group C (forty-four procedures), an Orthofix Garches lengthening device was used, the syndesmosis was stabilized, and a tenotomy of the Achilles tendon was performed. RESULTS: At the time of the latest follow-up (average, five years; range, two to seven years), the average gain in length after the seventy procedures performed because of a limb-length discrepancy was 4.0+/-1.98 centimeters (range, 2.5 to 9.5 centimeters), or 14 percent (range, 7 to 45 percent). The average gain in length after the 160 procedures that were performed because of a short stature was 7.8+/-2.28 centimeters (range, 2.5 to fifteen centimeters), or 33 percent (range, 10 to 78 percent). Ten (14 percent) of the seventy procedures performed because of a limb-length discrepancy and forty-six (29 percent) of the 160 performed because of a short stature were associated with a complication. There was only one permanent sequela in the entire series. CONCLUSIONS: Although the three operative protocols resulted in similar healing indices, the rates of complications differed significantly among the groups (p<0.0001). Group C (the Garches device) had the lowest rate of complications (7 percent). It is important to be aware of potential complications as well as the need for additional procedures in order to avoid predictable problems. These procedures include percutaneous tenotomy of the Achilles tendon and fixation of the distal segment of the fibula to the tibia to maintain the integrity of the tibiotalar articulation and the alignment of the foot.

Achilles Tendon↗
PubMed1997

Femoral callotasis.

The procedure for femoral callotasis is explained, and a series of 160 lengthenings is reviewed, 60 for limb length discrepancy and 100 for short stature. Callotasis was performed in 106 subjects with a mean age of 19 years. The mean healing index (HI) of 36 days per centimeter is related more to etiology than to age or extent of lengthening. There were nine complications (15%) among subjects treated for limb discrepancies and 39 (39%) in those treated for short stature. Three permanent sequelae were recorded: necrosis of the head of the femur (two cases) and permanent extension deficit of the knee (one case). The present review assesses the type of results that can be achieved with callotasis in straightforward cases of femoral lengthening when the guidelines proposed by the author are followed. It does not attempt to compare this technique with other methods of limb lengthening.

Adolescent↗
PubMed1997

Lower-limb lengthening in short stature. An electrophysiological and clinical assessment of peripheral nerve function.

We assessed peripheral nerve function during and after lower-limb lengthening by callotasis in 14 patients with short stature, using motor conduction studies. Four patients with short stature of varying aetiology showed unilateral and one showed bilateral weakness of foot dorsiflexion. Both clinical and electrophysiological abnormalities consistent with involvement of the peroneal nerve were observed early after starting tibial callotasis. There was some progressive electrophysiological improvement despite continued bone distraction, but two patients with Turner's syndrome had incomplete recovery. A greater percentage increase in tibial length did not correspond to a higher rate of peroneal nerve palsy. The function of the posterior leg muscles and the conduction velocity of the posterior tibial nerve were normal throughout the monitoring period. The F-wave response showed a longer latency at the end of the bone distraction than in basal conditions; this is probably related to the slowing of conduction throughout the entire length of the nerve.

Achondroplasia↗
PubMed1996

Leg lengthening in Turner dwarfism.

We have reviewed 16 patients treated by leg lengthening for various forms of Turner dwarfism with regard to the long period of healing and the complications. We consider that Turner dwarfism is a suitable indication for leg lengthening because of the moderate length deficit and the morphological appearance of the patients, and have introduced an improved programme of management to deal with the problems encountered.

Adolescent↗
PubMed1994

Articulated distraction of the hip. Conservative surgery for arthritis in young patients.

Between 1979 and 1982, 80 patients with a variety of hip diseases were treated with articulated distraction of the hip. The patients ranged from nine to 69 years of age (mean, 34 years). The primary diagnoses were avascular necrosis, osteoarthrosis, and chondrolysis. A standard dynamic axial fixator with a single axis articulating unit was used to create a 5-mm joint space. The fixator allowed flexion and extension motion and remained in place for six to ten weeks. The follow-up period ranged from five to eight years. Assessment was performed by questionnaire, clinical, and radiographic review. The results were poor in 24 patients who were either older than 45 years of age or had a diagnosis of inflammatory arthropathy. Forty-two good results were found in the 59 patients younger than 45 years with osteoarthrosis, hip dysplasia, avascular necrosis, and chondrolysis. Only four patients older than 45 years of age had a good result. No serious complications occurred.

Adolescent↗
PubMed1993

A chart of anthropometric values.

A chart is presented to assist with the assessment and treatment of patients with growth-related deformities. It is based on anthropometric values from five published sources and relates sitting height to stature, limb length, the radiographic lengths of the leg bones and the lengths of the feet and hands. It has proved useful in the prediction of leg-length discrepancies, in the diagnosis of cases of short stature, and in the assessment of spinal shortening from scoliosis.

Adolescent↗
PubMed1989

Epiphyseal distraction. Chondrodiatasis.

Chondrodiatasis is a limb-lengthening technique involving slow, controlled, symmetric epiphyseal distraction. The clinical and histological differences between this technique and distraction epiphysiolysis are described. Results are given for the elongation of 170 bone segments in 75 children (41 with limb-length discrepancies and 34 with achondroplasia). All patients were operated upon while the growth plate was still open with maximum bone maturation corresponding to Risser 2.

Achondroplasia↗
PubMed1989

Epiphyseal distraction. Hemichondrodiatasis.

Hemichondrodiatasis is a technique of closed, gradual, asymmetric distraction of the growth plate to correct angular deformities in growing children. This report describes the technique and the results achieved in 35 operated lower extremity segments, 14 involving the femur and 21 involving the tibia. The best results were achieved in posttraumatic deformities when the bone bridge occupied less than 20%-30% of the epiphyseal plate. The procedure is best performed toward the end of growth, or earlier if a progressive deformity exceeds 15 degrees - 20 degrees.

Adolescent↗
PubMed1989

The callotasis method of limb lengthening.

Callotasis is a lengthening technique that involves slow, controlled distraction after subperiosteal-submetaphyseal osteotomy. The technique and its advantages over other methods are described. Results of lengthenings involving 270 operated bone segments (146 femurs and 124 tibias) in 140 patients are reviewed. Ninety-five patients had limb-length inequality and 45 had hypochondroplasia or achondroplasia. On average, 6.6 cm, or 24.6% of initial length, was gained. The mean healing index was 39; the complication rate was 13.3%.

Achondroplasia↗
PubMed1988

Limb lengthening by diaphyseal corticotomy, callus distraction, and dynamic axial fixation. An experimental study in the ovine femur.

Limb lengthening of the left femur was performed in 12 sheep, 7-8 months of age. After weakening of the cortex by drill holes, a controlled fracture was created in the diaphysis. The bone segments were fixed in reduced position using a unilateral external fixation device. The fixator was left in neutral position for 10 days postoperatively when lengthening started. By means of daily distraction at a rate of 1.0 mm once a day for 3 weeks, an average lengthening of 1.9 cm (11.9%) was obtained. Two weeks after distraction had been discontinued, distinct callus bridging of the lengthening zone was confirmed radiographically in all animals. At this time the rigid state of fixation was converted to a system allowing axial compression by loading in half the animals. The other half of the animals served as controls with the fixator remaining unchanged. The external device was left on until 5 weeks after the end of distraction. At this time the animals were killed. Both femora of all animals were tested mechanically in torsion. The average ultimate torsional strength of the elongated femur compared with control was 71% in the group with dynamic axial fixation and 65% in the control group. No significant difference in relative strength of the tested bones in the two groups was found. We conclude that intermittent axial compression by loading during consolidation had no significant effect on the bone-healing process evaluated by radiography and mechanical testing.

Animals↗
PubMed1988

Surgical correction of short stature.

The aim of surgical orthopaedics in short stature is to increase overall height and improve body proportions. It is particularly applicable to lower limb lengthening in achondroplasia, hypochondroplasia, Turner's syndrome and Ellis-van Creveld syndrome. Two methods are routinely used in Verona: chondrodiatasis and callotasis. Although simultaneous lengthening of tibia and femur of the same limb have generally been employed, recent efforts have concentrated on simultaneous lengthening of one femur and the contralateral tibia. Lengthening of up to 20-25 cm can now be achieved, with a complication rate below 20%.

Achondroplasia↗
PubMed1986

Limb lengthening by distraction of the epiphyseal plate. A comparison of two techniques in the rabbit.

We have compared, in rabbits, two techniques of limb lengthening by distraction of the epiphyseal plate using a unilateral external fixation frame. In all cases, 14 mm of symmetrical lengthening without deviation was achieved. With rapid distraction at rates of 1 mm per day (distractional epiphyseolysis) separation of the epiphysis from the metaphysis occurred by day 7, and by day 70 almost complete ossification of the cartilage and the elongated segment was evident. In contrast, slow distraction at 0.25 mm every 12 hours (chondrodiatasis) produced hyperplasia of growth cartilage without any evidence of detachment at 28 days, the end of the distraction period. By day 70 the epiphyseal plate had returned to normal thickness with normal cellular morphology, while the lengthened segment was occupied by ossified tissue. The significance of these findings is discussed.

Animals↗
PubMed1986

Chondrodiatasis-controlled symmetrical distraction of the epiphyseal plate. Limb lengthening in children.

We describe a technique for slow, progressive, symmetrical distraction of the growth plate using a lightweight dynamic axial fixation system. Results are given for the elongation of 40 bony segments in children with limb-length discrepancies and 60 segments in children with achondroplasia or hypochondroplasia. Increases in limb length of up to 36% were obtained in non-achondroplastic and up to 64.5% in achondroplastic patients. There were no nerve or vascular lesions or bony infections and no case required a bone graft. Pin-track complications occurred in only 1.5%.

Achondroplasia↗
PubMed1986

Dynamic axial fixation. A rational alternative for the external fixation of fractures.

Conventional external fixation systems neither inhibit motion at the fracture site sufficiently to permit primary bone healing, nor do they allow sufficient motion to encourage adequate external callus formation. Healing with such systems is therefore prolonged. These methods are usually reserved for the most severe fractures when internal fixation may be contraindicated. A unilateral, dynamic axial fixation system (Orthofix - registered trademark) is described which allows for simple conversion from a rigid to a dynamic mode, and so can be readily adapted to the changing physiological patterns of fracture repair. In 288 fresh fractures a success rate of 94% was achieved, with an average time to healing of 4.4 months. The incidence of pin-track infection was only 0.6%. The contribution which the mechanical and design features of the apparatus make to the results obtained is discussed. It is suggested that the system is capable of extending the range of indications for an externally mounted system to include many cases which would formerly have been treated by internal fixation, plaster cast or traction.

Adult↗