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PubMed2006

Midterm results of primary total knee arthroplasty using a dished polyethylene insert with a recessed or resected posterior cruciate ligament.

Use of a dished polyethylene insert in 114 total knee arthroplasties, all with the posterior cruciate ligament resected or recessed, was retrospectively studied. Patients were evaluated at a mean follow-up of 8.3 years. Mean range of motion increased from 92 degrees to 111 degrees . Mean Knee Society pain and function scores increased from 35.2 and 39.7 to 91.3 and 74.7, respectively. WOMAC scores improved significantly in each category evaluated, including pain, stiffness, and physical function. Kaplan-Meier survivorship was 95% at 10 years (95% confidence interval, 82%-99%). The use of a dished polyethylene insert in primary total knee arthroplasty provides good to excellent midterm results regardless of whether the posterior cruciate ligament is recessed or sacrificed.

Adult↗

PubMed2006

Large recesses associated with left ventricular noncompaction: an unusual presentation of the disease.

Left ventricular noncompaction (LVNC) represents an arrest of myocardial morphogenesis, resulting in persistence of multiple prominent ventricular trabeculations and deep intertrabecular recesses. We report a case of a 30-year-old patient who was referred to our hospital due to familial history of cardiomyopathy. The patient underwent transthoracic echocardiography, cardiac catheterization and contrast ventriculography and cardiac magnetic resonance imaging. All imaging techniques showed the characteristic pattern of noncompaction together with two unusually large recesses in the inferior wall of myocardium. This unusual form of LVNC may have an impact on the clinical course of the disease and may require anticoagulation therapy.

Adult↗

PubMed2004

Connexin 26 mutations in nonsyndromic autosomal recessive hearing loss: speech and hearing rehabilitation.

OBJECTIVE: Hearing loss is the most common form of sensory impairment, with approximately one infant/1000 born with profound congenital deafness. A pre-lingual bilateral sensorineural hearing impairment poses a substantial problem as it negatively impacts on the subject's ability to conduct a normal social life. The aim of the study was to observe, in a group of children affected by pre-lingual non-syndromic autosomal recessive hearing impairment: (1) the role of the possible mutation of connexin 26 in the pathogenesis of the hearing loss; (2) the audiological and clinical aspects of the hearing impairment; (3) therapy to be adopted for the different patients. METHODS: The study was carried out on 39 patients, 16 males and 23 females, aged between six and 17 years (mean 12 years), affected by non syndromic congenital deafness, presumably hereditary, referred to the out-patients audiology clinic for children of the Department of Otolaryngology of the Federico II University of Naples. RESULTS: Our study conducted on 39 children with pre-lingual bilateral sensorineural autosomal recessive deafness showed as follows: (I) from a molecular perspective: an incidence of 41% in the cases studied of mutations in the encoding of the connexin 26 gene; a prevalence in our case study of the 35delG mutation (69%). (II) The characteristics of the hearing impairments in the children studied were homogeneous, regardless of the presence or absence of a connexin 26 mutation: the hearing impairment was pre-lingual bilateral sensorineural, the impairment often involved mainly the high frequencies, but, especially in the severe forms an involvement of all the frequencies was not rare; the hearing impairments were symmetrical and non progressive in time. (III) The results of the application of prosthesis and thereafter rehabilitative language therapy are generally satisfactory but correlated of course to the severity of the hearing loss. CONCLUSION: In conclusion, we hope that further developments in the research on genetic hearing impairments will promptly result in advances in clinical practice.

Adolescent↗

PubMed2006

The role of botulinum toxin A in augmentation of the effect of recession and/or resection surgery.

PURPOSE: The aim of this study is to assess the effectiveness of intraoperative botulinum toxin A (BTA) injection as an adjunct to the surgical treatment of large-angle esotropia or exotropia. METHODS: Ten patients were included in the study. Mean age of the patients was 27+/-20 years. Of these 10 patients, 7 were esotropic and 3 exotropic. The average preoperative esodeviation was 73.6+/-16.5 prism diopters and exodeviation was 76.7+/-5.8 PD. Five units of BTA were injected intraoperatively into one of the recessed horizontal rectus muscles in all of the patients. RESULTS: The average follow-up was 14+/-10 months (range, 8 to 40 months). The average final deviation in the esotropia group was 13+/-9.6 PD of esotropia. The average final deviation in the exotropia group was 4.7+/-5 PD of exotropia. The final deviation of the 70% patients was within 10 PD of esotropia or exotropia. CONCLUSION: The results of this study suggest that the combination of BTA injection with recession may increase the expected correction of a conventional horizontal rectus muscle surgery.

Adolescent↗

PubMed2004

Gastrocnemius and soleus lengths in cerebral palsy equinus gait--differences between children with and without static contracture and effects of gastrocnemius recession.

Equinus gait is one of the most common abnormalities in children with cerebral palsy. Although it is generally assumed that the calf muscles are abnormally short in equinus gait, no studies have been done to confirm that the muscles are short and that this shortness contributes to the equinus. This study used musculoskeletal modeling combined with computerized gait analysis to examine medial gastrocnemius (MGAS), lateral gastrocnemius (LGAS), and soleus (SOL) musculotendinous lengths during equinus gait in children with cerebral palsy. All three muscles were abnormally short during equinus gait whether or not the children had equinus contractures (P < or = 0.005). Children with static contractures had shorter maximum static MGAS and LGAS lengths than children with dynamic equinus (P < or = 0.002). The children with static contractures had ratios of peak dynamic length to maximum static length close to 1.0 for MGAS and LGAS (1.005 +/- 0.015) but lower ratios for SOL (0.984 +/- 0.024). For the children with static contracture, these ratios did not change significantly after gastrocnemius recession (P > or = 0.14) because both static and dynamic lengths increased postoperatively (P < or = 0.04). These results support the current clinical understanding of the role of calf "tightness" in equinus gait, including the appropriateness and effectiveness of gastrocnemius recession for children with equinus contracture.

Ankle Joint↗

PubMed2006

Intra-articular synovial lipoma of the knee joint (located in the lateral recess): a case report and review of the literature.

This report describes a rare intra-articular synovial lipoma of the knee joint which developed in a 66-year-old female. The patient suffered from sudden knee pain and a catching or slight interruption of normal motion of the left knee and then noticed the tumor. The tumor was located in the lateral recess of the knee joint and showed a signal intensity similar to subcutaneous fat on T1 and T2()-weighted magnetic resonance images. The arthroscopy revealed a smooth, globular, yellowish, encapsulated tumor extending into the lateral recess from the surface of the lateral condyle and the tumor was totally excised under arthroscopic guidance. Histologic examination of the specimen revealed a tumor composed of mature adipose cells covered by a thin fibrous layer of varying thickness and normal synovial lining cells. The diagnosis was intra-articular synovial lipoma. Intra-articular synovial lipomas should be distinguished from other similar lipomatoid conditions such as Hoffa disease and villous lipomatous proliferation of the synovial membrane (lipoma arborescens). Intra-articular synovial lipoma should be considered in the differential diagnoses when examining a patient with sudden knee pain, and a catching or locking knee.

Adult↗

PubMed2005

Endoscopic transpterygoid approach to the lateral sphenoid recess: surgical approach and clinical experience.

OBJECTIVE: Pathology within a lateral recess of a widely pneumatized sphenoid sinus is difficult to access with the use of traditional open and current endoscopic surgical approaches. A new surgical procedure, the endoscopic transpterygoid approach, directly accesses this region. A clinical experience over several year with this approach is reported as well as a refined and updated description of the technique. SETTING: Tertiary care center. METHODS: Retrospective review of medical records of patients treated with the endoscopic transpterygoid approach to the lateral sphenoid recess. RESULTS: An endoscopic transpterygoid approach was performed in 9 patients, 6 to resect a middle fossa meningoencephalocele and repair the CSF leak and associated skull base defect, 2 for possible invasive fungal sinusitis, and 1 to marsupialize a symptomatic epidermoid cyst. Patients tolerated the approach well and no significant complications occurred. CONCLUSIONS AND SIGNIFICANCE: In selected cases, the endoscopic transpterygoid approach enables the otolaryngologist to meet modern demands to treat conditions in the lateral sphenoid using minimally invasive techniques that are well-tolerated by patients. The surgical approach and the initial experience described herein is presented in the hopes that it can aid fellow otolaryngologists to help patients with challenging conditions in this region of the body.

Cerebrospinal Fluid Rhinorrhea↗

PubMed2005

The effect of multicolor playground markings on children's physical activity level during recess.

BACKGROUND: British children do not meet daily physical activity recommendations, and as yet no sustainable intervention has been reported to address their sedentary lifestyle. The purpose of this investigation was twofold: First, to examine whether painting playgrounds with multicolored markings would increase the percent of recess time spent in moderate to vigorous physical activity (MVPA) and vigorous physical activity (VPA) in girls and boys and second to quantify the contribution recess makes to national recommendations for young people's physical activity. METHODS: MVPA and vigorous physical activity (VPA) were measured using short wave heart rate telemetry. Physical activity levels were compared before and after playgrounds were painted in 4 intervention schools and compared to 4 matched schools with unmarked playgrounds. Schools were situated in areas of deprivation. Ninety-nine children completed the study. RESULTS: A significant interaction (group x time) was evident for MVPA and VPA. Time spent in MVPA and VPA increased significantly in intervention schools as a result of playground painting. Boys were more active than girls, and activity decreased with age, but neither difference reached significance. CONCLUSION: Multicolor playground markings can be a low-cost method of increasing children's daily physical activity levels in the short term. If these increases were sustained, then school playgrounds with multicolor markings would make a valuable contribution to physical activity recommendations for young people.

Child↗

PubMed1992

Recession of both horizontal recti muscles in Duane's retraction syndrome with elevation and depression of the adducted eye.

Upshoot and downshoot of the adducted eye in Duane's retraction syndrome is thought to be caused by a bridle effect of the co-contracting horizontal recti muscles. A recession of both of these muscles transposes their insertions posteriorly in relation to the center of rotation of the globe, which reduces the bridle effect and decreases the upshoot and downshoot. An 11-year-old girl had Duane's retraction syndrome type III and upshoot and downshoot of the left eye. The left lateral rectus muscle was recessed 8 mm and the left medial rectus muscle 6 mm. At last examination 10 1/2 months postoperatively, the upshoot and downshoot on attempted adduction was no longer present.

Child, Preschool↗

PubMed1992

X-linked recessive familial exudative vitreoretinopathy.

Familial exudative vitreoretinopathy is an inherited disorder characterized by retinal traction, peripheral vitreous opacities, and subretinal and intraretinal exudates. We observed a family in which four boys (the children of three sisters) were affected with this disorder and an X-linked recessive inheritance was apparent. The differential diagnosis includes retinopathy of prematurity, primary hyperplastic primary vitreous, Coats' disease, peripheral uveitis, retinoblastoma, and Norrie's disease, but this differentiation can usually be made on the basis of clinical findings alone. Knowledge of X-linked recessive transmission is important for correct diagnosis and for genetic counseling.

Child, Preschool↗

PubMed1999

A novel mutation in the ABCR gene in four patients with autosomal recessive Stargardt disease.

PURPOSE: To identify additional mutations in the ABCR gene and describe the clinical features of four affected siblings with autosomal recessive Stargardt disease. METHODS: A cohort of eight siblings was identified for study. Four of these individuals were diagnosed with Stargardt disease based on clinical evaluation and fluorescein angiography. Blood samples were obtained from seven of eight siblings, including all those affected. All 50 exons of the ABCR gene were analyzed by single-stranded confirmation polymorphism analysis, followed by direct sequencing of observed variants, to identify mutations in the ABCR gene. RESULTS: We identified a previously unreported kindred of eight siblings, four of whom had mutations in both of their ABCR alleles. A previously described G-to-C transversion of nucleotide 2588, predicting a Gly863Ala amino acid substitution, and a novel G-to-A transition of nucleotide 161, resulting in a Cys54Tyr substitution, were identified. These mutations co-segregated with the affected members of this family. Three of the siblings demonstrated clinical features characteristic of classic Stargardt disease, with bilateral regions of macular atrophy associated with yellow-white "flavimaculatus" flecks in the posterior pole at the level of the retinal pigment epithelium. The fourth affected sibling showed features of early Stargardt disease, with a beaten-bronze appearance to both maculas, as well as perimacular flecks. In all four affected patients, fluorescein angiography showed a characteristic peripheral dark choroid. CONCLUSIONS: We have identified both a previously described and a novel mutation in the ABCR gene in four patients with autosomal recessive Stargardt disease. In-depth knowledge of the ABCR mutation spectrum in patients with Stargardt disease will provide for more efficient screening and may provide potential therapies for Stargardt disease and other retinal diseases.

ATP-Binding Cassette Transporters↗

PubMed2004

Autosomal recessive hyperimmunoglobulin E syndrome: a distinct disease entity.

OBJECTIVE: The autosomal-dominant form of the hyperimmunoglobulin E syndrome (AD-HIES) has been described as a multisystem disorder including immune, skeletal, and dental abnormalities. Variants of AD-HIES are known but not well defined. METHODS: We evaluated 13 human immunodeficiency virus-seronegative patients from six consanguineous families with an autosomal-recessive form of hyperimmunoglobulin E syndrome (AR-HIES) and 68 of their relatives. RESULTS: Persons affected with AR-HIES presented with the classical immunologic findings of hyperimmunoglobulin E syndrome, including recurrent staphylococcal infections of the skin and respiratory tract, eczema, elevated serum immunoglobulin E, and hypereosinophilia. In addition, severe recurrent fungal and viral infections with molluscum contagiosum, herpes zoster, and herpes simplex were noted. Autoimmunity was seen in two patients. Central nervous system sequelae, including hemiplegia, ischemic infarction, and subarachnoid hemorrhages, were common and contributed to high mortality. Notably, patients with AR-HIES did not have skeletal or dental abnormalities and did not develop pneumatoceles, as seen in AD-HIES. In lymphocyte proliferation assays, patients' cells responded poorly to mitogens and failed to proliferate in response to antigens, despite the presence of normal numbers of lymphocyte subpopulations. CONCLUSION: The autosomal-recessive form of hyperimmunoglobulin E syndrome is a primary immunodeficiency with elevated immunoglobulin E, eosinophilia, vasculitis, autoimmunity, central nervous system symptoms, and high mortality. AR-HIES lacks several of the key findings of AD-HIES and therefore represents a different, previously unrecognized disease entity.

Autoimmunity↗

PubMed1994

Bone marrow transplantation for autosomal recessive osteopetrosis. A report from the Working Party on Inborn Errors of the European Bone Marrow Transplantation Group.

The outcomes of 69 patients who received allogeneic bone marrow grafts for autosomal recessive osteopetrosis in the period between 1976 and 1994 were analyzed retrospectively. Four patients received bone marrow transplants (BMT) without prior myeloablative conditioning; transient osteoclast function was demonstrated in one of them. Sixty-five patients received myeloablative pretreatment. Recipients of a genotypically human leukocyte antigen (HLA)-identical BMT had an actuarial probability for 5-year survival, with osteoclast function, of 79%; recipients of a phenotypically HLA-identical bone marrow graft from a related or unrelated donor, or one HLA-mismatched graft from a related donor, had an actuarial probability for 5-year survival, with osteoclast function, of 38%; patients who received a graft from an HLA-haplotype mismatched related donor had a probability for 5-year survival of only 13%. The main problems in haplotype-nonidentical BMT were graft failure and BMT-related complications such as sepsis, bleeding, and interstitial pneumonia. Osteoclast function developed in all patients with full engraftment. Recovery of osteoclast function was associated with severe hypercalcemia in 24% of the patients with engraftment, especially those older than 2 years of age. At the time of BMT, severe visual impairment was present in 35% of the patients; of the 15 patients who had visual impairment at the time that a successful BMT was performed, two had improvement after BMT (13%). Within the total group, one patient had neurodegeneration. Engraftment of healthy donor cells had no influence on the progression of that abnormality and BMT thus had no beneficial effect on this phenotype of osteopetrosis. In general, however, early BMT remains the only curative treatment for autosomal recessive osteopetrosis.

Actuarial Analysis↗

PubMed1982

Superior rectus recessions for dissociated vertical deviation.

Thirty-two eyes of 31 patients received superior rectus recessions for the treatment of dissociated vertical deviation (DVD). All patients were followed for a postoperative period of at least six months, and 50% were followed for a period of three years or more. The differential diagnosis of DVD and inferior oblique overaction was important, and the distinguishing features of each are mentioned. A distinction was made between occlusion hyperphoria, a latent deviation in the preferred fixating eye, and DVD, a manifest deviation in the nonpreferred eye. In the vast majority of cases, a unilateral superior rectus recession of graduated amounts was performed in the eye with a cosmetically unacceptable DVD. The surgical "hang loose" technique permitted avoiding technically difficult situations. After operation 82% of the eyes were aligned, within the good to excellent categories (0-9 prism diopters).

Adolescent↗

PubMed1987

Simultaneous superior oblique tenotomy and inferior oblique recession in Brown's syndrome.

The technique and results of simultaneous superior oblique tenotomy and 14-mm inferior oblique recession for true Brown's syndrome are presented for 16 eyes of 13 patients. A good or excellent result was achieved in 15 of 16 eyes (94%). Reoperation for overcorrection was not necessary; however, repeat tenotomy was required in two cases. Inferior oblique underaction was present in 12 of 16 eyes (75%) in the early postoperative period; however, elevation in adduction improved over time. At most recent examination, 92% of eyes demonstrated greater than 25 degrees elevation in adduction. Underaction of the superior oblique occurred in 3 of 16 eyes (19%). A simultaneous superior oblique tenotomy and inferior oblique recession is recommended in all patients selected to undergo surgery for true Brown's syndrome to prevent reoperation for iatrogenic superior oblique palsy.

Adolescent↗

PubMed1991

Large recession of the horizontal recti for treatment of nystagmus.

Ten patients had large recession of four horizontal recti at one procedure for treatment of nystagmus. Six patients had congenital motor nystagmus, two had oculocutaneous albinism, and two had optic nerve hypoplasia. Anomalous head posture with null point was also present in five patients. The rectus muscles were placed at or behind the equator in all but one case. Three patients with both esotropia and nystagmus had the medial recti placed 1 mm behind the equator and the lateral recti or at 1 mm anterior to the equator. Visual acuity improved an average of 1 line at distance and/or near in 8 patients who cooperated for testing. Although nystagmus was not eliminated in any patient, its its amplitude decreased in eight of ten patients, and anomalous head posture improved in three of five patients. In all patients, near vision was better than distance vision both preoperatively and postoperatively. Ductions were diminished minimally after the large recession and there were no other complications from surgery.

Adolescent↗

PubMed1993

Effect of severing the check ligaments and intermuscular membranes on medial rectus recessions in infantile esotropia.

BACKGROUND: The effect of severing the check ligaments and intermuscular membranes on the dose-response curve for medial rectus recessions in patients with infantile esotropia has not been previously studied by randomized assignment techniques using contemporary control and experimental groups and masked precautions. METHODS: A prospective study of 101 patients was performed over an 8-year period. Patients were randomly assigned to either augmented or nonaugmented groups. In the former group, the medial check ligaments and intermuscular membranes were severed; in the latter group, no surgery was performed on the medial check ligaments or intermuscular membranes. Patients were postoperatively evaluated by an observer masked to group assignment. RESULTS: No difference in response to surgery was observed between the two groups at the time of evaluation, which took place, on average, 6 to 7 months after surgery. CONCLUSION: Severing the check ligaments and intermuscular membranes on patients with infantile esotropia undergoing medial rectus recessions does not appear to affect the dose-response curve.

Adolescent↗

PubMed1993

Surgical management of post-traumatic angle recession glaucoma.

PURPOSE: The purpose of this study is to compare the results of three different drainage procedures performed for uncontrolled post-traumatic angle recession glaucoma. METHODS: A retrospective analysis was undertaken of 87 drainage procedures performed on 65 patients over an 8-year period. The results of trabeculectomy (47 procedures), Molteno single-plate implantation (20 procedures), and trabeculectomy combined with antimetabolite (20 procedures) were compared. Of those treated with antimetabolite, 11 received postoperative subconjunctival injections of 5-fluorouracil and 9 received an intraoperative application of 0.02% mitomycin C to the trabeculectomy site. RESULTS: In the group undergoing trabeculectomy with antimetabolite therapy, the intraocular pressure (IOP) drop was significantly greater, the percentage of successful cases at 3 and 6 months postoperatively was significantly higher, and the number of postoperative glaucoma medications was significantly lower than the other two groups. No statistically significant differences were found between the groups undergoing trabeculectomy without antimetabolite therapy and Molteno implantation. Of concern were three cases of late bleb infection in the group that received postoperative antimetabolite therapy. CONCLUSION: In medically uncontrolled post-traumatic angle recession glaucoma, trabeculectomy with antimetabolite therapy is the most effective surgical procedure. However, late bleb infection is a significant risk.

Adolescent↗