Bilateral Preiser’s disease: a case report and review of the literature

Avascular necrosis of bone may affect specific areas of the body where the blood supply to the bone or part of the bone is vulnerable to injury or disease. In the carpal scaphoid, it can find this entity consequent a trauma, microtrauma, or fractures, especially in the proximal pole (Preiser in Fortschr Geb Roentgenstr 15:189-197, 1910), associated with a systemic disease, steroid ingestion (Ferlic and Morin in J Hand Surg-A 14:13-16, 1989), chemotherapy (Green in Hand Clinic 3:163-168, 1987) or a hypoplastic scaphoid (Gunal et al., in J Hand Surg-B 20(6):736-740, 1995). Preiser's disease is a rare entity and is commonly associated with avascular necrosis of the scaphoid without fracture or trauma. A case of bilateral idiopathic avascular necrosis of the scaphoid is reported.     

Perianal Paget’s disease: case report and review of the literature

A peri-anal skin lesion, often eczema-like and with symptoms of pruritus, that does not resolve after classical local therapy should be biopsied. We present a case of peri-anal extramammary Paget’s disease (EMDP) and associated anal adenocarcinoma. Reviewing the literature, more than 30% of patients with EMDP present a second primary tumour in their past, present or future history. In Europe, the risk of developing a new primary tumour in patients with this condition is increased compared with the standard population. In cases of peri-anal Paget’s disease (PPD), specific histochemical markers allow us to differentiate between a primary and a secondary form, the secondary one is strongly associated with colorectal and anal tumours. We provide information about the most commonly suggested therapy for PPD with or without associated malignancy and about the recommended follow-up.     

Fibular grafting in diaphyseal Gorham’s disease: report of two cases and review of literature

Gorham’s disease of bone is a rare entity where the bone undergoes progressive osteolysis. Management of diaphyseal Gorham’s disease is a real challenge to manage as a large bony defect is created because of osteolysis. We report two such cases where we reconstructed the defect with fibular graft after a radiation dose of 30 Gy. The graft was well incorporated and radiological sign of union was noticed in proximal and distal metaphysis after a follow-up of 4 and 2 years. Fibular grafting can be a good method of reconstruction in diaphyseal Gorham’s disease of long bone, provided the diseased tissue has been excised adequately after local radiation therapy.     

Case report of Kummell’s disease with delayed onset myelopathy and the literature review

European Journal of Orthopaedic Surgery & Traumatology - Kummell’s disease is an avascular necrosis of the vertebral body, secondary to a vertebral compression fracture. This entity is...     

Renal complications of Castleman’s disease: report of two cases and analysis of 75 cases

Biopsy-proven renal complications of Castleman’s disease (CD) are rare and current knowledge is largely based on sporadic case reports. We reported two more cases, both of which were multicentric CD with hyaline-vascular pathological pattern and presented with chronic renal failure. Case 1 was multicentric CD with renal mesangial proliferative glomerulonephritis complications, and case 2 was multicentric CD with membranoproliferative glomerulonephritis-like complications. Although both were eventually administered corticosteroids combined with cytotoxic drugs, both behaved in an aggressive and relapsing manner. We then made an analysis of 75 cases of biopsy-proven renal complications of CD (including our two cases) which were reported in 51 English literatures from January 1954 to March 2011. We found that the clinical and histological findings of renal complications of CD were heterogeneous. Death was observed in 17% patients after a median follow-up time of 22 months (0–204 months) since histological diagnosis of renal complications. The estimated 5-year cumulative survival rate was 75%. Better understanding and therapeutic interventions are required in further investigations.     

Successful perioperative management of patients with Parkinson’s disease following gastrointestinal surgery: Report of three cases

Parkinson’s disease is a chronic progressive disease with symptoms of tremors, rigidity, and akinesia. Parkinson’s disease is a major risk factor for postoperative complications and it is difficult to manage patients with Parkinson’s disease after gastrointestinal surgery. This report presents the cases of three patients with Parkinson’s disease who underwent gastrointestinal surgery and had no serious postoperative complications. Antiparkinsonian medications were reduced before surgery and replaced by an intravenous infusion of levodopa until the patients could take oral antiparkinson drugs after surgery to prevent malignant syndrome or exacerbate parkinsonism. Prokinetics were also used for the prevention of paralytic ileus. These cases demonstrate an effective method to administer antiparkinson drugs during perioperative management of patients with Parkinson’s disease and also indicate that sufficient doses of antiparkinson drugs can control the symptoms of parkinsonism and prevent complications in cases of surgery with parkinsonism.     

Kienbock��s disease in a varsity football player: a case report and review of the literature

Objective:

To present the diagnostic, clinical features, and management of Kienbock’s disease and create awareness of the differential diagnosis of this condition in patients presenting with insidious, progressive dorsal wrist pain.

Clinical Features:

A 23-year old male varsity football player presented with insidious progressive dorsal sided wrist pain with reduced wrist flexion and extension. A diagnosis of Kienbock’s disease was made based on radiographs and magnetic resonance imaging.

Intervention and Outcome:

A 3mm ulnar-minus variance was found and a joint leveling procedure to shorten the radius was performed. Conservative therapy was provided pre and post surgical management.

Summary:

This case report demonstrates the importance of findings on radiographs, MRI, and clinical examination in the accurate diagnosis and management of a patient with wrist pain.     

Pulmonary renal syndrome associated with Wegener’s granulomatosis: a case report and review of literature

Pulmonary renal syndromes are rare but serious complications of systemic vasculitis. The majority of these cases are related to ANCA-associated vasculitis. These syndromes represent a combination of diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. Here we report a case of 21-year-old female with a clinical picture of community-acquired pneumonia that rapidly evolved to pulmonary hemorrhage and acute renal failure. Combined pulse steroid treatment, immunosuppressive treatment, and plasmapheresis were instituted and resulted in the improvement of the patient. The diagnosis of Wegener’s granulomatosis was established based on the clinical and serological findings (positive cANCA). Here we review the relevant literature on pulmonary renal syndromes associated with Wegener’s granulomatosis and discuss their diverse clinicopathologic features.     

Adult Hirschsprung’s disease: A case report and literature review

Introduction & importanceAdult Hirschsprung’s disease (AHD) is a difficult diagnosis to make due to its rarity, frequently after emergency interventions have been conducted. We present a case of possible AHD and sketch a classic presentation of Adult Hirschsprung’s Disease. This would help recognize and include AHD in the differential diagnoses of chronic constipation where appropriate.Case presentationThe case involved a 41-year-old male with a history of multiple abdominal surgeries for volvuli and a ventral hernia repair complicated by post-operative SBO. Presenting symptoms were chronic constipation, abdominal colic, and dilation. SBO secondary to volvulus was discovered, decompressed, and emptied of 3000cc fecal material in the OR. His case was again complicated twice by SBO which led to the suspicion of AHD and instigated this systematic review. Papers were extracted from the EBSCO and PubMed databases. Papers were excluded if patients were younger than 10 years old.Clinical discussionThe classic patient will be a male over the age of 10 years old with an average age of 30 years old and a long history of chronic constipation, often complicated by an acute symptomatic obstruction. Patients may have had a history of multiple surgical or non-surgical interventions to relieve their constipation.ConclusionAHD is being seen more frequently with the increasing availability of healthcare in underserved areas of the world. Duhamel’s procedure is the most effective procedure after diagnosis has been made. Barium enema and a biopsy show hypo- or a-ganglionic segments that are to be resected for curative purposes.     

Osteomyelitis complicating Sever‘s disease: A report of two cases

Sever's disease is a common cause of heel pain in young children and it has a self-limiting course without long term complications. Osteomyelitis of calcaneus is a sinister condition which can lead to serious complication if treatment is delayed. Indolent nature of calcaneus osteomyelitis leads to overlap in its clinical picture with Sever's disease. Two cases of Sever's disease which were later diagnosed and managed for osteomyelitis are reported. Salient differentiating features of Sever's disease and calcaneus osteomyelitis along with management strategy are discussed.     

Double anal canal cancers associated with a long history of perianal Paget’s disease: report of a case

Extramammary Paget's disease (EPD) is rare. We report a case of double anal canal cancers in a patient with a long history of perianal Paget's disease. The patient, a 68-year-old Japanese woman, refused surgery initially and was treated with electron beam therapy, which achieved remission. However, 6 years later, Paget's disease was found to be progressing again and double anal canal tumors were also detected in the proctos and external skin area. We performed abdominoperineal resection (Miles' operation) and lymph node dissection for the Paget's disease with double anal canal tumors. Immunohistochemical staining revealed cytokeratin (CK)-20 expression in the adenocarcinomas and Paget's disease lesion, but not CK-7 or gross cystic disease fluid protein-15 expression. The lesion was joined to the carcinoma by a stalk. The immunohistochemistry results suggested secondary EPD, although it was originally considered to be Paget's carcinoma (primary EPD) based on the clinical history.     

Dieulafoy’s Lesion of the Rectum

Dieulafoy’s lesion is an uncommon cause of gastrointestinal haemorrhage. It may present with massive and life threatening bleed and although more common in the upper gastrointestinal tract, it is being increasingly reported as affecting the lower gastrointestinal tract. Diagnosis is usually achieved during proctoscopic and endoscopic visualization. In cases where there is profuse and torrential haermorrhage, angiography may help to confirm the diagnosis. There are a few treatment options available, all of which have a varying degree of success. More commonly than not, a combination of treatment is warranted as illustrated by our case. Recurrent bleeding may occur just as in cases of Dieulafoy’s lesion affecting the upper gastrointestinal tract. Even though endoscopic visualization of the lower gastrointestinal tract in the presence of profuse lower gastrointestinal haemorrhage may not be possible, this important procedure should not be omitted as the bleeding source may be lying in a low and accessible location for prompt interventional haemorrhage control.     

Castleman’s disease of the mesorectum: Report of a case

Castleman’s disease is a benign lymphoid proliferative disorder, which most commonly presents as a solitary mass in the mediastinum, although extrathoracic sites have been reported in the neck, axilla, pelvis, mesentery, pancreas, and retroperitoneum. We report a case of asymptomatic, isolated Castleman’s disease in the mesorectum, which is extremely rare. The patient was a 34-year-old woman who underwent investigations for vaginal spotting. A presacral mass was located on the left side of the rectum, 10 cm from the anal verge. Contrast-enhanced computed tomography showed a large, well-demarcated, strongly enhancing mass with internal radiating septa in the mesorectum. The mass was well circumscribed and isointense to muscle on T1-weighted magnetic resonance imaging, appearing as a slightly hyperintense mass on T2-weighted imaging. We performed laparoscopic mesorectal mass excision, and histological examination revealed features typical of the hyaline-vascular type of Castleman’s disease. Thus, when a mesorectal mass is being investigated, Castleman’s disease should be considered in the differential diagnosis.     

Crohn’s disease of the esophagus: Report of a case

(Received for publication on Jan. 18, 1999; accepted on Sept. 17, 1999)     

Primary Ewing’s sarcoma of the maxilla: a patient report

Primary Ewings sarcoma rarely arises in the facial skeleton and only occasionally in the maxilla. To date, there have been 22 cases of maxillary Ewings sarcoma reported in the English-language literature. We report a case arising in the maxilla, successfully treated by wide resection, followed by adjuvant radiotherapy and systemic chemotherapy.     

Open grade III “floating ankle” injuries: a report of eight cases with review of literature

Introduction “Floating ankle” injuries result from high-energy trauma and are usually associated with extensive soft tissue damage. Such rare and complex injuries in the acute phase pose therapeutic dilemma to the treating surgeon. When salvage instead of amputation is preferred, a variety of treatment options are available ranging from open reduction along with minimal internal fixation to external fixation. In this retrospective case series study we report eight patients with open “floating ankle” injuries who had been treated with external fixation with or without internal fixation. Materials and methods Eight consecutive patients (7M: 1F) with mean age of 28 years (range 18–35 years) were treated. All had Grade III open fractures of the distal tibia with foot fractures and various degrees of soft tissue injuries. ISS and MESS were used to assess injury severity. Immediate radical wound debridement; skeletal stabilization and early soft tissue coverage were done by combined trauma and plastic surgical services followed by standard post-operative rehabilitation. All the patients were assessed with SF-36 questionnaire at mean follow-up of 2.5 years (range 2–4 years). Results Three patients were treated primarily with Ilizarov ring fixators, one had hybrid fixation and the other four had Hoffman frames. Four patients needed further surgeries. One patient developed metatarsal osteomyelitis, which was debrided and treated with antibiotics. The SF-36 results were compared with age matched UK norms for men and women in all categories. Only two patients returned to their previous employment. Six suffered varying degrees of disability out of which one underwent amputation. Conclusions External fixation with or without internal fixation is an option when salvaging rare injuries like open grade III “floating ankle” injuries. Salvaged patients do suffer a cocktail of crippling disease characterized by psycho-socio-economic and physical disability and result in increased hospital costs. We recommend more prospective studies with long-term follow-up of such complex injuries to identify the indications for salvage and also the criteria for a successful salvage.     

Peyronie’s disease: contemporary review of non-surgical treatment

Background

Peyronie’s disease (PD) remains a therapeutic dilemma for the treating physician. This is in spite of a large array of treatments which have been used since the time of de la Peyronie in the mid 18^th century. Part of this problem is due to an incomplete understanding of the etiopathophysiology of this scarring disorder. Having a better understanding of the how and why the scarring occurs may help prevent progression, but ultimately reversing the existing scar remains the real challenge.

Methods

This review discusses the current non-surgical treatment options for Peyronie’s disease. Published articles in peer-reviewed journals are used, recognizing that the majority of the published trials are compromised by being single-center studies without a placebo control.

Results

A variety of treatments options have emerged, most with limited and unreliable benefit, but a few treatments have shown a consistent albeit incomplete response rate. Could this suggest that all PD is not the same and that the heterogeneous nature of this scarring disorder may account for why some patients respond and others do not? Further investigation of this diverse response rate may yield insights into the pathophysiology of PD. In the meantime, there have been many oral treatments offered for PD. Currently the only scientifically sensible treatments appear to be pentoxifylline, L-arginine, and possibly the phosphodiesterase type-5 inhibitors. Intralesional injection has been used for many years. The current treatment options include verapamil and interferon, with reported benefit with respect to reduced deformity and improved sexual function. Intralesional clostridial collagenase is in the midst of phase 3 trial analysis by the FDA in the USA and may become the newest and only FDA approved treatment for Peyronie’s disease. External mechanical traction therapy has also recently emerged as a technique to reduce curvature, recover lost length, enhance girth, and possibly obviate surgery.

Conclusions

It appears at this time that there is no clear, reliable and effective non-surgical treatment for Peyronie’s disease, but it does appear from the published literature that several of the available treatments can result in reduction of deformity, improved sexual function, and may at a minimum stabilize the disease process so that deformity does not get worse particularly during the acute phase of this scarring disorder. Combination therapy in an effort to create a synergy between the chemical effects of oral and injectable drugs with the mechanical effects of external traction therapy may provide the best opportunity today for reduction of deformity in the man with Peyronie’s disease.     

Visual aspects of behçet’s disease

Beh?et's disease is a systemic vasculitis characterized by recurrent oral and genital ulcers and ocular inflammation. The disease may also involve the joints, skin, central nervous system, the lungs, and gastrointestinal tract. Ocular manifestations include anterior uveitis, posterior uveitis, macular edema, retinal vasculitis, conjunctival ulcers, optic neuropathy, papilledema, secondary glaucoma, cataract, and neovascularization of iris or retina. The cause of Beh?et's disease is unknown, but autoimmune and genetic factors are considered important in its pathogenesis. Promising new imaging techniques and treatments are available to provide better management for patients. This review focuses on recent data on visual aspects, pathogenesis, and the treatment of Beh?et's disease.