Lateral hemiepiphysiodesis of the first metatarsal for juvenile hallux valgus.

BACKGROUND: Juvenile hallux valgus (JHV) frequently results in an impaired quality of life because of pain and deformity. We used a validated clinical score to investigate the efficacy of lateral hemiepiphysiodesis of the first metatarsal base as a treatment for JHV. METHODS: We conducted a prospective, nonrandomized, single center cohort study of children who underwent lateral hemiepiphysiodesis for 31-IV, with a clinical and radiologic follow-up of at least 2 years. The efficiency of the procedure was assessed with the Hallux Metatarsophalangeal Interphalangeal Scale (HMIS), the metatarsophalangeal angle (MPA), and intermetatarsal angle (IMA). RESULTS: Twelve patients (22 feet) with JHV were treated by lateral hemiepiphysiodesis between 2012 and 2015. Average age at surgery was 10 years, and average follow-up was 3.5 years. The average HMIS score increased from an average of 56 at baseline, to 92 at last follow-up ( p < 0.01). The average IMA decreased by 2°, from 13° preoperatively to 11° postoperatively. Average MPA decreased from 26° at baseline to 22° after surgery. No complications occurred in the immediate postoperative period, and no patient needed an additional procedure. CONCLUSION: The HMIS score was significantly improved after lateral hemiepiphyslodesis, with a clear improvement in pain and functional impairment at last follow-up. We noted a stabilization of the IMA and MPA, but the radiologic benefit did not reach statistical significance. Our data support the use of lateral hemiepiphysiodesis as an effective and safe treatment of JHV.

Outcomes of Gartland type III supracondylar fractures treated using Blount's method.

BACKGROUND: Supracondylar fractures of the elbow with major displacement are usually treated by surgical pinning and less often non-operatively as described by Blount. The objective of this study was to assess the clinical and radiological outcomes of Gartland type III supracondylar fractures treated at least 3 years earlier using Blount's method. HYPOTHESIS: Blount's method produces good outcomes after more than 3 years when used to treat Gartland type III supracondylar fractures of the humerus. METHODS: A single-centre retrospective study was done in paediatric patients who were seen within 24hours after sustaining a Gartland type III supracondylar fracture then re-evaluated at least 36 months after treatment. Closed reduction was performed either in the operating room under general anaesthesia or in the radiology suite under procedural sedation. The upper limb was then immobilised for 4 weeks using the cuff-and-collar method described by Blount (mean elbow flexion, 134°). The child was evaluated and radiographs obtained at the outpatient clinic on days 7 and 14. Functional outcomes were assessed using the 1962 SoFCOT criteria and Flynn's criteria and the radiological outcome using Baumann's angle, the humero-condylar angle, and distal fragment rotation. From 2009 to 2013, 22 patients met the inclusion criteria. Mean follow-up was 57 months. RESULTS: Clinical outcomes assessed using the 1962 SoFCOT criteria were very good in 15 patients and good in the remaining 7 patients. The rate of satisfactory outcomes according to Flynn's criteria was 100%. At last follow-up, mean Baumann's angle was 68°, mean humerocondylar angle was 42°, and 2 patients had residual rotation of the distal fragment. CONCLUSION: This work confirms the effectiveness of Blount's method for treating Gartland type III supracondylar fractures. We advocate routine first-line treatment of these fractures using Blount's method in the absence of vascular compromise and instability. LEVEL OF EVIDENCE: IV, retrospective study.

Cardiac sarcoidosis: a potentially fatal but treatable form of infiltrative heart disease.

Cardiac sarcoidosis is a rare but important differential diagnosis in patients who present with progressive heart failure and arrhythmia. It may be diagnosed on endomyocardial biopsy. An excellent response can be achieved with steroid therapy in the early acute inflammatory stage. Progression of the disease may lead to end-stage heart failure that requires implantation of a permanent pacemaker, implantable cardioverter-defibrillator, or mechanical circulatory support as a bridge to heart transplantation. We present three Hong Kong Chinese patients with cardiac sarcoidosis.

[Percutaneous surgical treatment of two epiphyseal plate fractures of the proximal humerus]. / Réduction et embrochage percutanés de deux décollements épiphysaires de l'humérus proximal.

We report two cases of Salter 2 displaced epiphyseal detachment of the proximal humerus treated successfully by percutaneous reduction and centromedular nailing. This surgical treatment was required because of the irreducibility of the lesion in one patient and instability in the other. This percutaneous technique can be recommended after failure of orthopedic treatment and is an attractive alternative to open surgery.

Initial treatment of congenital idiopathic clubfoot: Prognostic factors.

BACKGROUND: The initial treatment of congenital idiopathic clubfoot (CIC) is nonoperative. Either the French physiotherapy method or the Ponseti casting method may be used. Whether either method is superior over the other remains unclear. However, the method used initially is not the only determinant of the final outcome. OBJECTIVE: The primary objective was to identify determinants of the final outcome as evaluated based on the need for surgical treatment and on the Ghanem-Seringe score. HYPOTHESIS: Factors associated with the final outcome can be identified. METHODS: Between 2004 and 2011, 100 CICs in 79 patients were treated in two centres, 47 using the French method and 53 the Ponseti method. The Dimeglio grade was determined at baseline and the Ghanem-Seringe score at last follow-up. Surgical procedures (if any), splinting duration, and rehabilitation therapy duration were recorded. The two groups showed no statistically significant differences for Dimeglio grade distribution, time from birth to treatment initiation, or mean follow-up. RESULTS: Factors significantly associated with a poor outcome by univariate analysis were use of the Ponseti method (P=0.0027), older age at last follow-up (P=3×10-4), initial Dimeglio grade (P=7×10-5), and need for surgery (P=10-5); no significant effect was found for splinting duration, rehabilitation duration, bilateral involvement, or antenatal diagnosis. By multivariate analysis, factors independently associated with a poor prognosis were older age at last follow-up, Dimeglio grade, and need for surgery. CONCLUSION: This study confirms the major prognostic significance of initial severity (Dimeglio grade) on the final outcome. The data do not firmly establish that one method is superior over the other. Nevertheless, the need for percutaneous Achilles tenotomy with the Ponseti method leads us to prefer the French physiotherapy method. LEVEL OF EVIDENCE: IV, retrospective study.

Too-long calcaneal process: Results of surgical treatment and prognostic factors.

INTRODUCTION: The too-long anterior process (TLAP) can be responsible for ankle pain or repeated sprains in children or adolescents. The objective of this study was to assess the results of TLAP surgical treatment and to analyze influencing factors in case of this surgery's failure. MATERIAL AND METHODS: Retrospective single-center study conducted from 2009 to 2012 including all patients under 18 years of age for a TLAP with follow-up equal to or longer than 1 year. The results of surgical treatment were assessed using the AOFAS score. Failure was defined as no significant improvement in the AOFAS score at the last follow-up. HYPOTHESIS: Predictive factors of the result of surgical treatment for TLAP can be identified. RESULTS: At the mean follow-up of 2.5 years, 35 patients (43 feet) fulfilled the inclusion criteria. Thirteen feet (30%) presented surgical failure. According to the AOFAS score, the results were excellent in 30 feet (70%), good in four (9%), fair in five (12%), and poor in four (9%). Surgical failure was influenced by the patient's age at the onset of symptoms and at the time of surgery, the degree of functional limitation, the duration of symptoms before surgery, the number of sprains, and gender (P<0.05). CONCLUSION: Firstly, in this pediatric population with its high functional demand, the overall rate of failure of TLAP surgery was 30%. Secondly, the factors associated with failure demonstrated made it possible to identify the ideal patient for this surgery: male, with symptom onset between 7 and 10 years of age, who had experienced fewer than 15 sprains, and undergone surgery in the 3 years following the beginning of symptoms. LEVEL OF EVIDENCE: IV.

[Congenital pseudarthrosis of the forearm in a neurofibromatosis patient: case report and review of the literature]. / Pseudarthrose congénitale de l'avant-bras associée à la neurofibromatose: à propos d'un cas et revue de la littérature.

We report a case of congenital pseudarthrosis of the forearm associated with neurofibromatosis type 1 which was treated by free vascularized periosteal flap transplant and repeated bone grafting. The young female patient recovered good hand and forearm function. A review of the literature revealed the rare occurrence of this disease (approximately 100 cases reported to date), which probably explains the wide variety of surgical proposals.

Epidermal aquaporin-3 is increased in the cutaneous burn wound.

INTRODUCTION: Aquaporins (AQP) are a family of transmembrane proteins that transport water and small solutes such as glycerol across cell membranes. It is a mediator of transcellular water flow and plays an important role in maintaining intra/extracellular fluid homeostasis by facilitating water transport in response to changing osmotic gradients. In the skin, AQPs permit rapid, regulated, and selective water permeability and have been demonstrated to play a role in skin hydration, cell proliferation, migration, immunity, and wound healing. However, the expression of AQP-3 in the cutaneous burn wound has never been elucidated. We sought to assess the expression of AQP-3 in patients with burn wounds. METHODS: A fresh full thickness biopsy sample was taken from the center of the burn wound, the burn wound edge, and the graft donor site in 7 patients (n=21), approximately 3-7 days post injury. Fixed, paraffin embedded sections were stained using AQP-3 specific antibody and examined by immunofluorescence. Fresh samples were processed to quantify AQP-3 protein expression with Western blot analysis. RESULTS: The central portion of the burn wound revealed destruction of the epidermis and dermis with no AQP-3 present. Along the burn wound edge where the epidermal architecture was disrupted, there was robust AQP-3 staining. Western blot analysis demonstrated deeper staining along the burn wound edge compared to unburned skin (control). Quantification of the protein shows a significant amount of AQP-3 expression along the burn wound edge (3.6±0.34) compared to unburned skin (2.1±0.28, N=7, *p<0.05). There is no AQP-3 expression in the burn wound center. CONCLUSION: AQP-3 expression is increased in the burn wound following injury. While its role in wound healing has been defined, we report for the first time the effect of cutaneous burns on AQP-3 expression. Our data provides the first step in determining its functional role in burn wounds. We hypothesize that development of AQP3 targeted therapies may improve burn wound healing.

Pericardial effusion in adults undergoing surgical repair of atrial septal defect.

The incidence of pericardial effusion and tamponade postatrial septal defect repair in adult patients are 16 and 1.5%, respectively. Small, medium, and large effusions progressed equally, and echocardiographic study on days 7, 14, and 28 best detects potentially significant effusion.

Mediastinal irradiation for graft-versus-host disease in a heart-lung transplant recipient.

Graft-versus-host disease in solid organ transplantation is very rare, but the prognosis is poor when the condition causes pancytopenia. We report a case of graft-versus-host disease in a heart-lung transplant recipient who at 2 weeks after transplantation had development of features of graft-versus-host disease, including bone marrow aplasia, that could not be attributed to drugs or viral infections. The diagnosis was confirmed by skin biopsy and demonstration of chimerism of peripheral lymphocytes. Augmentation of immunosuppression with intravenous methylprednisolone resulted in improvement in liver function but had no effect on the pancytopenia. Mediastinal irradiation was given with increase in both white blood cell and platelet counts. Unfortunately the patient eventually died of gastrointestinal bleeding and fungemia.

Increased incidence of chronotropic incompetence in older donor hearts.

BACKGROUND: Previous Registry studies have reported that heart transplantation with older donor hearts is associated with a more than twofold increase in early mortality. METHODS: An analysis of 77 consecutive patients undergoing heart transplantations at our institution between June 1988 and July 1994 was performed to assess the effect of donor age on mortality and morbidity. Recipients were grouped into those receiving hearts from younger donors (aged < 40 years, n = 60) and those receiving hearts from older donors (aged > 40 years, n = 17). RESULTS: There were two deaths within the first 30 days in the younger donor group and no deaths in the other group. One-year survival rate was 95% and 100% for the "younger" and "older" groups, respectively. The mean recipient age of the younger donor group was lower (46 +/- 14 years) compared with the older donor group (57 +/- 7 years). Morbidity was compared between the two groups; the length of hospital stay (22.6 +/- 15.8 days versus 21.3 +/- 9.4 days), the graft ejection fraction at 1 week (64% +/- 5% versus 62% +/- 7%), and the mean number of rejection episodes within the first 3 months (0.79 versus 0.65) were not statistically different between the two groups. However, the incidence of chronotropic incompetence requiring permanent pacemaker implantation was significantly greater in the group who received older donor hearts (41.2% versus 10.3%, p < 0.05), independent of the ischemic time. CONCLUSIONS: This study shows that older donor hearts may be used safely in selected patients with excellent outcome, although there is an increased incidence of chronotropic incompetence requiring implantation of permanent pacemakers.

Post-transplantation lymphoproliferative disease in Chinese: the Queen Mary Hospital experience in Hong Kong.

Post-transplantation lymphoproliferative disease (PTLD) is an unique iatrogenic complication after bone marrow transplantation (BMT) and solid organ transplantation (SOTx). The pattern of EBV related lymphoma in Chinese is different from Caucasians. We surveyed the incidence, clinical and pathological spectrum of PTLD among 541 cases of allogeneic BMT, 145 cases of renal transplant, 35 cases of heart/lung transplantation and 146 cases of orthotopic liver transplantation (OLT). From 1994 to 2001, 13 consecutive cases of PTLD were diagnosed, ranging from disseminated NK cell lymphoma to localized plasmacytoma. Both donor and recipient derived PTLD was documented. Disease was often heralded by cytomegaloviral disease and antithymocyte globulin (ATG) usage. Two cases were diagnosed post-mortem, and six patients died of PTLD at a median of 3 months. Complete and partial remission was only achieved in 3 and 2 cases, respectively, despite a range of treatment (reduced immunosuppression, explantation, radiotherapy, combination chemotherapy, donor lymphocytes, autologous marrow infusion and rituximab). Most responding patients died subsequently of rejection, infection and graft versus host disease (GVHD). The incidence of PTLD is not increased in Chinese patients. However, some patients may be at increased risk, especially mismatched allogeneic BMT, parental OLT (especially involving young infants) and heavy ATG exposure.

Recurrent aortic valve prosthesis dehiscence secondary to aortoarteritis: three case reports and literature review.

We report three young Chinese male patients who underwent aortic valve replacement for severe aortic regurgitation of subacute onset, complicated by recurrent dehiscence of the aortic valve prosthesis, due to aortitis. One of the three patients underwent aortic valve replacement three times and progression of the prosthesis dehiscence was arrested with the use of corticosteroids. A review of the literature on the prognosis and treatment of aortoarteritis with particular reference to the management of recurrent valvular dehiscence is discussed.

Prominent periportal echogenicity: its sonographic evaluation and its significance.

Prominent periportal echogenicity was detected during sonographic examination of patients suffering from recurrent pyogenic cholangitis, hepatocellular carcinoma and acute cholecystitis. To document the finding, 140 normal individuals were studied to establish a norm for the evaluation of the periportal echogenicity. The significance of this sonographic finding and its possible aetiology are discussed.

Nonsecretory immunoglobulin-derived amyloidosis of the heart: diagnosis by immunohistochemistry of the endomyocardium.

Primary amyloid light chain (AL) amyloidosis of the heart is a rare cause of congestive heart failure. Approximately 15% of patients with primary AL amyloidosis demonstrate no monoclonal proteins on serum or urine immunoelectrophoresis:(so-called nonsecretory immunoglobulin-derived amyloidosis). The histologic findings of endomyocardial biopsy from these patients may be indistinguishable from those with senile cardiac amyloidosis. However, the AL type may respond favourably to chemotherapy while the latter type does not. The prognosis is also better in the senile cardiac amyloid type. The precise diagnosis in the present case was made by applying immunohistochemical techniques on cardiac tissues.

Severe mitral regurgitation due to mitral valve prolapse associated with Bland-White-Garland syndrome.

Bland-White-Garland syndrome refers to the rare congenital cardiac abnormality whereby the left coronary artery arises from the pulmonary artery. The natural history of this condition is highly variable, ranging from death in early infancy to asymptomatic adult survival. It is sometimes diagnosed in adults with mitral regurgitation thought to be of ischaemic origin. We report a case of a 29-year-old man with Bland-White-Garland syndrome and concomitant mitral valve prolapse, and review the literature on the appropriate investigations and management of this abnormality. Recognition and diagnosis of this condition is important because of the potentially life-threatening complications, which may be prevented by surgical intervention.

Combined atrial septostomy and oral sildenafil for severe right ventricular failure due to primary pulmonary hypertension.

Management of primary pulmonary hypertension is usually difficult because the disease is uncommon and the aetiology of the disease is not well understood. The disease is potentially lethal because it can lead to failure of the right ventricle, low cardiac output, and ensuing multiple organ failure. We report the successful treatment of a case of low-output syndrome due to primary pulmonary hypertension using combined drug therapy and atrial septostomy. Latest developments in the treatment of this disease are also discussed.

Ultrasound guided transperineal prostatic aspiration biopsy: experience in Hong Kong.

OBJECTIVE: The purpose of this study is to correlate the result of the ultrasound guided transperineal aspiration biopsy with the clinical findings. MATERIALS AND METHODS: We retrospectively studied 27 patients who had been subjected to ultrasound guided transperineal prostatic aspiration biopsy in our institution during the period 1990 to 1992. The patients were divided into two groups: Group A--those with clinically palpable nodules on digital examination, and Group B--those without palpable nodule but with clinical evidence of prostatic enlargement and/or bladder outlet obstruction. RESULTS: There were 8 biopsy proven cancers, 4 of them were palpable lesions while the other 4 were clinically non-palpable. The overall positive predictive value was 29.6%; this increased to 40% if the lesions were palpable but decreased to 23.5% if the lesions were non-palpable. CONCLUSION: The overall positive predictive value of this technique was found to be consistent with other studies using different methods of biopsies. The positive predictive value was increased if the abnormality was clinically palpable. This technique was also found to be safe and no significant complication was observed in these patients.

Blount's disease: classification and treatment.

Forty-three tibia vara in 27 patients were analyzed retrospectively in two centers. The criteria for diagnosis of the child form are discussed. A simple classification is suggested to facilitate the choice of treatment. In stage 0 (possible Blount's disease), the patient is younger than 2 1/2 years, and an observation period is indicated for gathering data. In stage 1 (confirmed Blount's disease and absence of medial metaphyseal bony bridge), known as physis+, a valgization osteotomy is proposed. In stage 2 (evidence of a medial metaphysoepiphyseal bony bridge) known as physis-, valgization osteotomy with lateral epiphysiodesis and treatment of the lower limb discrepancy is proposed. For stages 1 and 2, there are two possibilities: normal medial tibial plateau or sloping of the medial tibial plateau, indicating a transphyseal elevation osteotomy. When one-step correction is proposed for stage 2 disorder, external fixators such as Orthofix or Ilizarov devices are useful.