'Absolute' inter-observer classifications agreement for proximal humeral fractures with a single shoulder anteroposterior X-ray.

PURPOSE: Several studies have been carried out, and there is no classification for proximal humeral fractures (PHF) exempted from variability in interpretation and with questioned reliability. In the present study, we investigated the 'absolute diagnostic reliability' of the most currently used classifications for PHFs on a single anterior-posterior X-ray shoulder image. METHODS: Six orthopaedic surgeons, with varying levels of experience in shoulder pathology, evaluated radiographs from 30 proximal humeral fractures, according to the 'absolute reliability' criteria. Each of the observers rated each fracture according to Neer, Müller/AO and Codman-Hertel's classification systems. RESULTS: The overall inter-observer agreement (κ) has been 0.297 (CI95% 0.280 to 0.314) for the Neer's classification system, 0.206 (CI95% 0.193 to 0.218) for the Müller/AO classification system, and 0.315 (CI95% 0.334 to 0.368) for the Codman-Hertel classification system. We found loss of agreement in Neer's classification as the study progressed, low agreement in the AO classification, and stable values in the different evaluations with the best degree of agreement for Codman-Hertel classification, with a moderate agreement in the second evaluation among the six evaluators. CONCLUSION: The Neer, AO, and Hertel-Codman classification systems for PHF with a single radiographic projection have a difficult interpretation for orthopaedic surgeons of varying levels of experience, and therefore substantial agreements are not obtained.

Clinical outcomes in elderly patients with Morquio a syndrome receiving enzyme replacement therapy - experience in a Colombian center.

INTRODUCTION: Mucopolysaccharidosis type IV A (MPS IVA) or Morquio A syndrome is an autosomal recessive lysosomal storage disease caused by GALNS gene mutations that lead to a deficiency of the N-acetylgalactosamine-6-sulfate sulfatase enzyme and the accumulation of two glycosaminoglycans in cell lysosomes, namely, chondroitin and keratan sulfate. OBJECTIVE: To present two female patients with Morquio A syndrome in their late adult years (over 50 years of age) with a classical phenotype, treated with enzyme replacement therapy; and to present a summary of the natural history and the characteristics of the disease, and the benefit of comprehensive management. MATERIALS AND METHODS: Descriptive clinical study before and after the treatment with enzyme replacement therapy as part of the comprehensive management of MPS IVA. RESULTS: Enzyme replacement therapy with elosulfase alfa was effective, with an adequate safety profile in these two patients, showing evidence of sustained improvement in terms of endurance and gait patterns. CONCLUSION: We present two cases of MPS IVA, with longer survival than reported previously in classical phenotypes associated with this disease condition. There is a paucity of reports of similar cases in the literature. We believe that the clinical heterogeneity of the disease manifesting with the classical phenotype, together with comprehensive management, have played a role in the survival of these two patients. Therapy with elosulfase alfa as part of comprehensive management has been crucial; we suspect a clinical response and infer a better quality of life and reduced burden for the caregiver, supporting its use in older patients.

Condrodisplasia metafisaria tipo Schmid: un caso de presentación familiar / Schmid type metaphyseal chondrodysplasia: a case of familial presentation

Resumen: dentro de las displasias óseas hay cuadros clínicos que hacen parte de las denominadas condrodisplasias metafisarias, conocidas también como disostosis metafisarias, las cuales presentan mínimas diferencias entre sí, lo que las hace susceptibles de ser confundidas con otros cuadros clínicos como la acondroplasia y el raquitismo. En este artículo se presenta un caso clínico de condrodisplasiametafisaria tipo Schmid de un paciente de Popayán, Colombia, al igual que algunas consideracionessobre las principales características clínicas, radiológicas, de diagnóstico y tipo de herencia de esta enfermedad. El caso clínico corresponde a un paciente de género masculino de 23 meses de edad, en quien se inician estudios por la presencia de talla baja desproporcionada. Los resultados mostraron coxa vara, genu varo y extremidades cortas, con un fenotipo similar en la madre y el abuelo materno. Las radiografías evidencian la presencia de irregularidad con “deshilachamiento” de las metáfisis de huesos largos; además, ensanchamiento y esclerosis en las metáfisis proximales de ambos fémur. La meta final es ser confirmado por medio de pruebas genéticas. En conclusión, las condrodisplasias metafisarias, especialmente la tipo Schmid, son enfermedades caracterizadas por talla baja y hallazgos radiológicos especiales, dados principalmente por el compromiso metafisario a nivel de los huesos largos, que en conjunto con las características fenotípicas pueden conducir a la sospecha e identificación de este tipo de patología.

Abstract: between the dysplastic bone pathologies there are some medical conditions that belong to so-called metaphyseal chondrodysplasias, also known as metaphyseal dysostosis. These differ slightly from each other, making them capable of being confused with other medical conditions such as achondroplasia and rickets. This article presents a case of Schmid type metaphyseal chondrodysplasiafrom Popayan, Colombia, as well as some considerations about the main clinical characteristics, radiological, diagnosis, and type of inheritance of this disease. The clinical case corresponds to a male patient, 23 months old, who was studied by the presence of disproportionate short stature. Findings showed coxa vara, genu varus, and short limbs, with similar phenotype to the mother and maternal grandfather. The radiological images showed the presence of irregularity with ®fraying¼ of the metaphysis of long bones, in addition to widening and sclerosis in the proximal metaphysis of both femurs. The ultimate goal is to be confirmed by genetic testing. In conclusion, the metaphyseal chondrodysplasias, especially Schmid type, are diseases characterized by short stature and by special radiological findings, mainly given by the metaphyseal affectation of long bones, which together with the phenotypic characteristics may lead to the suspicion and identification of this disease.Keywords: Schmid type metaphyseal chondrodysplasia, osteochondrodysplasias, collagen type.

Asymmetric inferior oblique anterior transposition for incomitant asymmetric dissociated vertical deviation.

BACKGROUND: Inferior oblique anterior transposition (IOAT) is indicated in patients with incomitant dissociated vertical deviation (DVD) larger in adduction. In general, bilateral surgery is recommended in patients with DVD unless there is deep monocular amblyopia. The purpose of this study is to evaluate the results of asymmetric IOAT in patients with asymmetric incomitant DVD larger in adduction. METHODS: Retrospective chart review of the records of all patients with incomitant asymmetric DVD associated with inferior oblique (IO) overaction who underwent asymmetric IO weakening procedure. In all patients, the eye with more DVD in adduction underwent IOAT to the temporal corner of the insertion of the inferior rectus (IR) muscle, and the eye with less DVD underwent IOAT to a position 3-4 mm posterior to the insertion of the IR. No other muscles were operated simultaneously. No patient had previous surgery on any cyclovertical extracular muscle. RESULTS: Fourteen patients were included. Mean age at surgery was 10.3 ± 8.8 years (range 4-33). Primary position DVD preoperatively was 18 ± 2 PD in the eye with the larger DVD compared to 1.1 ± 1.0 PD postoperatively (p < 0.0001). DVD asymmetry between the lateral gaze with the largest DVD and the lateral gaze with the smallest DVD was 9.8 ± 3.1 PD (range 5-14 PD) preoperatively vs 1.1 ± 1.0 PD (range 0-2 PD), (p < 0.0001). Ten patients had preoperative V-pattern >10 PD (24.7 ± 8.7 PD, range 12-50 PD) preoperatively vs no patients postoperatively (mean V-pattern 4.4 ± 2.0 PD), (p < 0.0001). Postoperative follow up was 1.6 ± 0.7 years (range 1-3 years). CONCLUSION: In patients with asymmetric incomitant DVD, asymmetric IOAT improves lateral incomitance without increasing the risk of antielevation, limitation in upgaze rotation, or hypertropia, or worsening the DVD in the eye with less deviation preoperatively.

Does inferior oblique recession cause overcorrections in laterally incomitant small hypertropias due to superior oblique palsy?

AIM: To evaluate the effects of inferior oblique muscle recession (IOR) in cases of laterally incomitant hypertropia <10 prism dioptres (PD) in central gaze thact 2t are clinically consistent with superior oblique palsy (SOP). METHODS: We retrospectively reviewed patients with SOP and hypertropias <10 PD in central gaze who underwent graded IOR. Primary outcomes were reduction of lateral incomitance and number of overcorrections in central gaze. RESULTS: Twenty-five patients were included. Mean follow-up was 13.8 months (range 1.4-66). Mean central gaze hypertropia decreased from 5.6±2.1 to 0.2±1.6 PD (p<0.001). Contralateral gaze hypertropia decreased from 15.9±7.6 to 2.3±3.3 PD (p<0.001). Lateral incomitance (central vs contralateral gaze) was 10.3±6.9 PD preoperatively and 2.0±3.0 PD postoperatively (p<0.001). There were two patients overcorrected in central gaze, and one patient overcorrected in downgaze. One patient necessitated further surgery for overcorrection. CONCLUSIONS: Although small hypertropias can be treated with prisms or small, adjustable inferior rectus recessions, IOR collapses incomitance without causing much overcorrection. IOR is a reasonable treatment for small, laterally incomitant hypertropia due to SOP.

Isolated y-splitting and recession of the lateral rectus muscle in patients with exo-duane syndrome.

PURPOSE: Weakening of both horizontal rectus muscles is performed for patients with Duane syndrome and significant misinnervation of the lateral rectus (LR) muscle resulting in strabismus, limitation to ocular rotations, and globe retraction. In patients with severe up-/downshoots, a Y-splitting of the LR is often recommended. The purpose of this study was to evaluate the efficacy of isolated unilateral LR recession-Y splitting in exo-Duane patients with limitation to adduction and up-/downshoots. METHODS: etrospective review of the records of consecutive patients with exo-Duane syndrome and up/down-shoots who underwent isolated Y-splitting-recession of the affected LR. RESULTS: The records of 10 patients were reviewed (mean age at surgery 23 ± 21 years). The Y-split was performed 10 mm posterior to the insertion and was combined with a mean LR recession of 8.7 ± 2.9 mm. Torticollis decreased from 12.7 ± 4.4° to 4.8 ± 5.3° (P = 0.003). Exotropia improved from 18.4 ± 7.3 to 6.2 ± 5.9 PD postoperatively (P < 0.001). Exotropia in contralateral gaze improved from 33.7 ± 11.8 to 18.7 ± 18.1 PD postoperatively (P = 0.09). No significant postoperative changes in esotropia in ipsilateral gaze, vertical deviations, or ocular rotations in adduction or abduction were observed. Downshoots were significantly decreased (P = 0.01), and there was a trend toward improvement of upshoots (P = 0.07). There were no overcorrections, although 3 patients required additional LR weakening and transposition. CONCLUSIONS: LR Y-splitting-recession improves ocular alignment, torticollis, and up-/downshoots. LR recession improves ocular alignment and torticollis, while the addition of a Y-split procedure improves up-/downshoots.

Surgical management of dissociated vertical deviation associated with A-pattern strabismus.

INTRODUCTION: Dissociated vertical deviation (DVD), pattern strabismus, and oblique muscle dysfunction frequently coexist, and the recognition of bilaterality, symmetry, and lateral incomitance is important in selecting appropriate surgical management. In this study, we compare 3 different surgical approaches in patients with DVD associated with A-pattern strabismus. METHODS: This was a retrospective review of 40 consecutive patients with DVD and A-pattern strabismus who underwent strabismus surgery. Bilateral superior rectus muscle recession was performed in 9 patients, bilateral superior rectus recession and superior oblique posterior tenectomy were performed in 14 patients, and bilateral superior oblique temporal tenotomy and inferior oblique recession were performed in 17 patients. RESULTS: Bilateral superior rectus muscle recession corrected 7(Delta) +/- 2(Delta) of A pattern, 10(Delta) +/- 3(Delta) of vertical deviation and 4(Delta) +/- 2(Delta) of DVD asymmetry. Bilateral superior rectus muscle recession combined with superior oblique posterior tenectomy corrected 17(Delta) +/- 3(Delta) of A pattern, 10(Delta) +/- 2(Delta) of vertical deviation, and 4(Delta) +/- 2(Delta) of asymmetry. Bilateral superior oblique muscle temporal tenotomy combined with inferior oblique recession corrected 30(Delta) +/- 4(Delta) of A pattern, 9(Delta) +/- 3(Delta) of vertical deviation, and 2(Delta) +/- 2(Delta) of asymmetry. CONCLUSIONS: In patients with DVD and A patterns, the size of the A pattern and the symmetry of the DVD are of major importance for surgeons determining appropriate procedures. Bilateral superior rectus muscle recession corrects small amounts of A pattern. Larger amounts of A pattern require additional superior oblique weakening or weakening of all four oblique muscles. superior rectus muscle recession is warranted if the asymmetry is larger than 5(Delta).

Maximum motor fusion combined with one-hour preoperative prism adaptation test in patients with acquired esotropia.

PURPOSE: Preoperative prism adaptation and the maximum motor fusion test reduce the risk of postoperative undercorrection, overcorrection, and bifocal requirements in acquired esotropia. The purpose of our study is to determine the efficacy of the maximum motor fusion test followed by a short prism adaptation test in patients with acquired esotropia undergoing bilateral medial rectus muscle recession. METHODS: We undertook a retrospective review of 29 subjects with acquired esotropia without history of amblyopia, previous strabismus surgery, use of bifocals, pattern anisotropia, dissociated vertical deviation, or oblique muscle dysfunction. All subjects underwent bilateral medial rectus muscle recessions based on the distance angle of deviation measured with the maximum motor fusion test followed by prism adaptation test. Alternate prism and cover testing was performed after 1 hour of prism adaptation to determine the angle of deviation for surgical correction. RESULTS: The age at surgery was 4.5 +/- 1 years. The preoperative angle of deviation at distance was 20.4 +/- 4.2(delta). It increased to 36.2 +/- 4.2(delta) after the maximum motor fusion test followed by 1 hour of prism adaptation testing. The final preoperative angle of deviation post motor fusion test-prism adaptation test was significantly larger than the initial angle of deviation (p < 0.0001). The final postoperative deviation at distance was 1.3 +/- 3.3(delta), and 5.2 +/- 1.5(delta) at near. Postoperative follow up was 18 +/- 2.6 months (range, 13-24 months). CONCLUSIONS: The combination of maximum motor fusion and preoperative prism adaptation allowed increased amounts of medial rectus muscle muscle recession, decreasing the risk of postoperative undercorrection without increasing the risk of overcorrection.

Superior oblique posterior tenectomy in patients with Brown syndrome with small deviations in the primary position.

INTRODUCTION: Procedures used to weaken the superior oblique muscle (SO) tendon in the treatment of patients with Brown syndrome, may result in severe complications, including complete SO palsy, overcorrections, foreign body extrusion, and scarring with limitation to ocular rotations. SO posterior tenectomy moderately weakens abduction and depression while preserving most of the torsional action of the SO muscle. PURPOSE: We sought to evaluate motor and sensorial results after SO posterior tenectomy in patients with Brown syndrome who had a small vertical deviation (less than 7 prism diopters [PD]) in primary position but severe limitation to elevation in adduction. METHODOLOGY: We retrospectively analyzed 12 consecutive patients with unilateral Brown syndrome who underwent a 15 mm tenectomy of the posterior four-fifths fibers of the ipsilateral SO tendon. Intraoperative forced duction showed restriction to elevation in adduction in all subjects. No patient had concomitant surgery on any other extraocular muscle. RESULTS: The mean patient age at diagnosis was 6.9 +/- 1.7 years. Preoperative vertical deviation measured 4 +/- 1 PD in the primary position and 12 +/- 2 PD in elevation in adduction. Postoperative follow-up was 24.7 +/- 9.2 months. Postoperatively, all patients had less than 2 PD of orthotropia in the primary position, and the deviation in elevation in adduction was significantly improved at 3 +/- 2 PD (P < 0.05). Elevation in adduction improved from -4.0 preoperatively to -1.9 +/- 1 postoperatively (P = 0.0000003) and no patient experienced underaction of the SO. Postoperatively, all patients had stereopsis in primary position. CONCLUSION: The use of SO posterior tenectomy improves alignment and ocular rotations in patients with Brown syndrome, resulting in fusion, small vertical deviation in primary position, and minimal-to-no anomalous head posture, in whom the most important finding is a disfiguring downshoot on attempted adduction. Other advantages include minimal-to-no postoperative SO muscle underaction and no risk of foreign body extrusion, fibrosis, and scarring.

Vertical rectus muscle transposition for bilateral Duane syndrome.

BACKGROUND: Augmented transposition of the superior and inferior rectus muscles to the lateral rectus muscle is effective surgical treatment for esotropia in unilateral Duane syndrome. Medial rectus muscle recession in bilateral Duane syndrome may increase the risk of consecutive exotropia and cause limitation to adduction postoperatively. Vertical rectus muscle transposition may be useful in bilateral Duane syndrome with esotropia. METHODS: We undertook a retrospective review of 11 patients with bilateral Duane syndrome and esotropia in primary position. All patients had vertical rectus muscle transpositions. Six patients had unilateral vertical rectus transpositions (2 eyes with and 4 without suture augmentation). Twelve eyes from 7 children (2 unilateral and 5 bilateral) had transpositions augmented with posterior fixation sutures. Posterior fixation suture were added to large deviations in patients without prior medial rectus recessions. RESULTS: The preoperative esotropia at distance was 22.8 +/- 6.3 prism diopters (PD). It reduced to 2.0 +/- 6.7 PD postoperatively. (P < 0.001) Esotropia at near changed from 21.0 +/- 5.8 PD preoperatively to 1.2 +/- 8.1 PD postoperatively. (P < 0.001) One patient with a 10-degree face turn had complete resolution postoperatively. One patient had a small undercorrection and developed a vertical deviation requiring additional surgery. All patients had improvement in abduction. Nine of 11 patients did not develop any limitation to adduction. One patient developed a -1 adduction deficit 5 years later. Three patients achieved fusion with a mean stereovision of 67 seconds of arc (range, 80-40 seconds.). Follow-up averaged 22.2 months (range, 1-100 months). CONCLUSION: Vertical rectus muscle transposition in patients with bilateral Duane syndrome and esotropia is an effective procedure to improve ocular alignment and motility while preserving adduction.

Gunshot wounds of abdomen: evaluation of stable patients with triple-contrast helical CT.

PURPOSE: To assess helical computed tomography (CT) with contrast material administered intravenously, orally, and rectally (triple contrast helical CT)) in the prospective evaluation of stable patients with abdominal gunshot wounds in whom there is no clinical indication for immediate exploratory laparotomy. MATERIALS AND METHODS: The study was conducted for 19 months. All patients met the following inclusion criteria: age of 16 years or older, hemodynamic stability, no clinical signs of peritoneal irritation, and signed consent to participate. Patients with obvious indications for laparotomy, such as gastrointestinal bleeding or evisceration, were excluded from the study. Forty-seven patients fulfilled the criteria and underwent abdominal triple-contrast helical CT. CT findings were evaluated by one of four radiologists for evidence of peritoneal penetration and injury to solid organs or hollow viscera. Patients were followed up clinically for 13 weeks. CT findings were compared with those at surgery and/or clinical follow-up. RESULTS: CT demonstrated abnormalities in 27 (57%) patients. Laparotomy was performed in 11 (23%) patients; 10 procedures were therapeutic and one was nontherapeutic. The remaining 20 patients had a negative CT scan. These patients were treated conservatively. One injury was missed at CT. For prediction of the need for laparotomy, sensitivity of CT was 96%; specificity, 95%; positive predictive value, 96%; negative predictive value, 95%; and accuracy, 96%. CONCLUSION: In stable patients with gunshot wounds to the abdomen in whom there is no indication for immediate surgery, triple-contrast helical CT can help reduce the number of cases of unnecessary or nontherapeutic laparotomy (negative laparotomy) and can help identify patients with injuries that may be safely treated without surgery.

Tratamiento quirúrgico de la parálisis del IV par / Surgical treatment of IV nerve palsy

Para el manejo quirúrgico de la parálisis del oblicuo superior es indispensable saber si son unilaterales o bilaterales. La clasificación de Knapp con algunas modificaciones continúa siendo nuestra base para una correcta decisión quirúrgica

Tratamiento del síndrome de Brown / Treatment of Brown's syndrome

El tratamiento del síndrome de Brown verdadero está solo indicado si hay una hipotropia en posición primaria de la mirada o una posición anormal de la cabeza. Se valoraron los resultados de los tratamientos quirúrgicos con tenotomía del oblicuo superior, tenotomía del oblicuo superior con debilitamiento simultáneo del oblicuo inferior y la elongación con banda de silicona

Tratamiento de la desviación vertical disociada / Treatment of dissociated vertical deviation

Se presentan los resultados de los tratamientos de la DVD bilateral asimétrica, monocular y la DVD asociada a hiperfunción de los oblicuos inferiores. En 35 pacientes se hicieron retroinserciones bilaterales asimétricas de los rectos superiores, obteniéndose buenos resultados en 25 pacientes. En 17 pacientes con DVD monocular se hizo retroinserción solamente de un recto superior, obteniéndose buenos resultados en 12 pacientes. En 19 pacientes se hizo una transposición anterior de los oblicuos inferiores para pacientes con DVD disociada a una hiperfunción de oblicuos inferiores, obteniéndose buenos resultados en 14 pacientes