A case of Degos disease successfully treated with corticosteroid combined with cyclophosphamide

Degos disease is a rare disorder characterized by systemic vasculitis involving various organs. There is no established, effective treatment for the disorder, and its prognosis is still poor. Combination therapy with corticosteroid and cyclophosphamide is considered effective for vasculitides involving the small arteries such as ANCA-associated vasculitis. We present here a 42-year-old man who developed Degos disease over several months, and was successfully treated using combined treatment with corticosteroid and cyclophosphamide.     

Effective infliximab treatment for a recurrent type of acute intestinal graft-versus-host disease accompanied by steroid-induced depression

A 22-year-old man with chronic active Epstein-Barr virus infection underwent allogeneic bone marrow transplantation (allo-BMT) from an HLA two allele-mismatched unrelated donor. Ten months after allo-BMT, he developed protein-losing enteropathy following a respiratory syncytial virus infection. A diagnosis of a recurrent type of acute graft-versus-host disease (GVHD) was made based on the histopathological findings, such as the infiltration of T lymphocytes into the superficial epithelium and crypts, and apoptotic bodies in crypts. Although methylprednisolone (mPSL: 10 mg/kg) administration for two consecutive days improved gastrointestinal symptoms, acute pancreatitis and severe depression developed in association with corticosteroid treatment. Reduction of mPSL and administration of infliximab (5 mg/kg/dose, 3 times) resulted in rapid improvement of depression and pancreatitis without aggravating intestinal GVHD. Recent studies have demonstrated that tumor-necrosis-factor (TNF)-α is associated with not only GVHD but also depression and acute pancreatitis. In the present case, anti-TNF-α treatment enabled us to reduce corticosteroid dose without aggravating GVHD, which suggests that this approach might be effective for the treatment of depression and acute pancreatitis.     

Congenital prepubic sinus associated with a urachal remnant: report of a case

Congenital prepubic sinus is a rare congenital anomaly situated in the midline of the lower abdomen. We report a case of congenital prepubic sinus, closely associated with a urachal remnant. Preoperative magnetic resonance imaging showed clearly that the sinus tracked the urachus caudally. This finding supports the theory that the anomaly is caused by abnormal remnant tissue originating from the cloacal membrane, which tracks the allantois duct caudally along with fetal longitudinal growth.     

Innervation pattern at the undersurface of the extensor carpi radialis brevis tendon in recalcitrant tennis elbow

Purpose

We investigated the general, sensory, and sympathetic innervation patterns at the undersurface of the extensor carpi radialis brevis (ECRB) origin in patients with recalcitrant tennis elbow.

Methods

Eight elbows in eight consecutive patients (6 females and 2 males) with tennis elbow who underwent arthroscopic surgery were included in this study. The mean age was 45 years (38–66 years), and the mean duration of symptoms before surgery was 23 months (13–52 months). Operative treatment consisted of an arthroscopic inspection and debridement of the ECRB origin. Control tissues were obtained from biopsy of the ECRB capsule in two patients with osteochondritis dissecance of the capitellum who underwent arthroscopic resection of loose bodies. The tissue specimens were investigated immunohistochemically with antibodies delineating general (PGP9.5), sensory (SP/CGRP), and sympathetic (NPY) nerve patterns.

Results

In the non-tendinosis control tissue, SP/CGRP and NPY immunoreactions were heterogeneously distributed in association with blood vessels. Pathologic evaluation of the biopsy tissue showed atypical fibrous granulation containing numerous vessels and nerve structures in all eight patients. Marked reactions to PGP 9.5 took the form of nerve fibers associated with arteries and arterioles in the atypical granulation. Most of the perivascular innervation was found to express NPY. The immunoreactions for SP and CGRP were invariably weak.

Conclusion

Increased perivascular sympathetic innervation accompanied with loss of sensory innervation at the undersurface of the ECRB tendon may play a role in chronic pain generation in recalcitrant tennis elbow.

Level of evidence

Diagnostic, Level IV.     

Efficacy of percutaneous ethanol sclerotherapy for venous malformation in lower extremities: a retrospective review of 21 cases

Background

Venous malformations (VM) of lower extremities have characteristic symptoms, especially swelling, pain at rest or with dependence, or in the morning, or with exertion. Sclerotherapy has been applied and has been showed to alleviate the associated signs and symptoms. The aim of this study is to evaluate the outcomes of ethanol sclerotherapy for VM of lower extremities.

Methods

The 21 patients of intramuscular VM of the lower extremities, who received percutaneous sclerotherapy using absolute ethanol in our institute, were reviewed retrospectively. The average age at the time of the initial diagnosis was 18.6 years, and average follow-up period after last sclerotherapy is 19.4?±?13.5 months. The postinterventional changes of the associated signs and symptoms were evaluated by utilizing original VM scoring system.

Results

The average number of sclerotherapy sessions was 2.6 times per case. The average total amount of ethanol used in each patient was 41.3 ml. Sclerotherapy reduced the associated signs and symptoms in many of the patients (n?=?19/21). Preinterventional VM score as the overall baseline status was 10, and the average VM score after sclerotherapy was 4.1. There was no inverse correlation between the amount of ethanol used and the VM score. In seven cases treated more than three times, reverse correlation between the “VM score” and the number of sclerotherapy sessions was demonstrated (p?<?0.05, ρ?=?0.8214).

Conclusions

The characteristic symptoms and signs of the VM were improved by sclerotherapy. More than three sessions of ethanol sclerotherapy improved the overall status of extensive intramuscular VM in lower extremities. Level of Evidence: Level IV, therapeutic study.     

Totally laparoscopic complete resection of the remnant stomach for gastric cancer

Background

In patients having carcinoma in the remnant stomach, total resection of the remnant stomach with lymph node dissection is a prerequisite.

Materials and methods

We present the first series of successful totally laparoscopic complete gastrectomy (TLCG) for gastric remnant cancer.

Results

TLCG was successfully performed without adverse events during surgery in five patients with gastric remnant cancer. The median age of the patients was 72 years (range, 56-84 years), and there were three men and two women. Three of them had a Billroth I reconstruction and two had a Billroth II reconstruction, and in four cases following partial gastrectomy for gastric cancer and one for gastroduodenal ulcer. The median operative time was 360 min; blood loss was 20 ml. The median number of retrieved lymph nodes was 19. No complications occurred postoperatively, and all of the patients were discharged within the ninth postoperative day.

Conclusions

Although TLCG for gastric remnant cancer is a technically difficult and challenging operation that requires careful lysis of adhesion and dissection along the major vessels, as well as intracorporeal anastomosis, this procedure is technically feasible. Long-term follow-up is mandatory to validate oncological outcome.     

Anal Canal Carcinoma in a Child With Disorders of Sex Development

Squamous cell carcinoma of the anal canal in children is rare. To date, the etiology and outcome of this condition have been not fully understood. Here, we report an 11‐year‐old child with anal canal cancer who had concomitant disorders of sex development. Radiotherapy followed by salvage surgery achieved disease‐free survival of 3 years. Since overexpression of cell cycle regulatory protein p16 was immunohistochemically evident in tumor tissue, human papillomavirus infection was considered as a causative factor in the carcinogenesis.