Perianal Paget's disease-Report of a rare case

INTRODUCTIONPerianal extra-mammary Paget's disease is a rare skin disorder of unknown aetiology, which is frequently associated with malignancy. This case report draws attention to this rare condition and comments upon its diagnosis and treatment.PRESENTATION OF CASEA 64-year-old otherwise fit man, presented to us in 2006 with one-year-long history of perianal irritation. On examination there was an erythematous discoid skin lesion in the right perianal area. The lesion was excised with wide margins and the defect closed with a local transposition flap. Histology confirmed extra-mammary Paget's disease (EMPD) with a focus of invasion showing a well-differentiated mucinous adenocarcinoma. Adjuvant therapy was not advised. On follow-up in 2011, a small irregular skin lesion, well away from the previous excision site was noted on the left perianal area. Biopsies from this lesion confirmed EMPD with no focus of invasion. Once again wide local excision with closure using local transposition flap was undertaken. Long term follow up has been advised.DISCUSSIONThe optimal treatment for Perianal Paget's disease (PPD) remains controversial. Surgery is the commonest modality used with wide local excision being the treatment of choice for resectable disease. We report herein a short review of various therapies reported so far in the management of this rare disorder.CONCLUSIONA thorough initial evaluation and long-term follow-up is essential to identify recurrence and the development of other related malignancies.     

A large incarcerated Meckel's diverticulum in an inguinal hernia

INTRODUCTION

Littre''s hernia is a rare finding consisting of a Meckel''s diverticulum inside of a hernia sac. Clinically, it is indistinguishable from a hernia involving small bowel and therefore may be difficult to diagnose pre-operatively.

PRESENTATION OF CASE

We report a case of an inguinal hernia involving an unusually large Meckel''s diverticulum measuring 15 cm in length. The diverticulum was resected using a linear GI stapler and the hernia was repaired without complication.

DISCUSSION

Meckel''s diverticulum is an embryologic remnant of the vitelline duct occurring in 1–3% of the adult population with an estimated 4% becoming complicated and presenting with intestinal obstruction, infection, bleeding or herniation. Surgical resection is the recommended treatment for any Meckel''s diverticulum causing symptoms. In the case of a Littre''s hernia, resection of the diverticulum should be followed by repair of the fascial defect in a standard fashion.

CONCLUSION

Littre''s hernia, although rare, should be a consideration at the time of repair for any abdominal hernia involving small bowel as resection of the Meckel''s diverticulum is critical in avoiding recurrent complications.     

Minimally invasive management of achalasia cardia: results from a single center study.

BACKGROUND: Since the performance of the first laparoscopic cardiomyotomy for achalasia cardia in 1991, the popularity of the minimally invasive approach for this troublesome disease has been growing. We present our experience of 226 patients who underwent laparoscopic cardiomyotomy and discuss the relevant issues. METHODS: A retrospective analysis was carried out of 226 patients who have undergone laparoscopic cardiomyotomy since 1993. The preoperative workup, surgical technique, and postoperative management are described. RESULTS: Patients included 146 males and 80 females; average age was 36.4 years (range, 6 to 85). Mean duration of symptoms was 1.4 years. Nearly half of the patients (112) had undergone prior pneumatic dilatation. In 20 patients, myotomy alone was done, 44 patients had a Dor's fundoplication, and 162 had Toupet's fundoplication. The average operating time was 96 minutes. Mean postoperative hospital stay was 2.2 days. Dysphagia was eliminated in 88.9% of the patients with an overall morbidity of 4.4% and nil mortality over a mean follow-up of 4.3 years. CONCLUSION: Laparoscopic cardiomyotomy with Toupet's fundoplication is a safe and effective treatment of achalasia cardia. Dor's fundoplication is done selectively, especially when suspicion is present of mucosal injury.     

Cullen's sign - Case report with a review of the literature

IntroductionCullen's sign is described as superficial oedema with bruising in the subcutaneous fatty tissue around the peri-umbilical region. It is also known as peri-umbilical ecchymosis.Presentation of caseWe report a case of a fifty-three year old gentleman who presented with non-specific abdominal pain. Significant bruising was present within the subcutaneous fatty tissue on the anterior abdominal wall in keeping with ‘Cullen's sign.’ Of note he denied any alcohol intake and his amylase was normal. A diagnosis of pancreatitis was made following a CT scan of his abdomen.DiscussionA search of the entire English literature using PubMed with the phrase ‘Cullen's sign’ has been performed. Papers were reviewed in relation to recognition of this clinical sign, differential diagnosis, and management.ConclusionOur case and review of the literature highlights the rarity of this clinical sign which a clinician may encounter. In addition we draw to attention the importance of having knowledge of the underlying possible conditions which may lead to this sign, and can be vital in successful acute management.     

Laparoscopy for treating a small bowel obstruction due to a Meckel's diverticulum.

A Meckel's diverticulum is one cause of small bowel obstruction in the virgin abdomen. A 17-year-old female presented with a 24-hour history of lower abdominal pain and emesis. Radiological imaging studies revealed a high-grade partial small bowel obstruction. A diagnostic laparoscopy was performed revealing a bowel obstruction secondary to a Meckel's diverticulum. The diverticulum was resected using an endovascular GIA stapler. The patient was discharged on postoperative day 3, tolerating a regular diet. Laparoscopy is a useful diagnostic and therapeutic tool for a patient with a small bowel obstruction with an unclear etiology.     

Corticosteroid Injection for Morton's Interdigital Neuroma: A Systematic Review

BackgroudThis review aimed to evaluate the effects of corticosteroid injections on Morton''s neuroma using an algorithmic approach to assess the methodological quality of reported studies using a structured critical framework.MethodsSeveral electronic databases were searched for articles published until April 2020 that evaluated the outcomes of corticosteroid injections in patients diagnosed with Morton''s neuroma. Data search, extraction, analysis, and quality assessments were performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). guidelines, and clinical outcomes were evaluated using various outcome measures.ResultsWith 3–12 months of follow-up, corticosteroid injections provided satisfactory outcomes according to Johnson satisfaction scores except in two studies. Visual analog scale scores showed maximal pain reduction between 1 week and 3 months after injection. We found that 140 subjects out of 469 (29.85%) eventually underwent surgery after receiving corticosteroid injections due to persistent pain.ConclusionsCorticosteroid injections showed a satisfactory clinical outcome in patients with Morton''s interdigital neuroma although almost 30% of the included subjects eventually underwent operative treatment. Our recommendation for future research includes using more objective outcome parameters, such as foot and ankle outcome scores or foot and ankle ability measures. Moreover, studies on the safety and effectiveness of multiple injections at the same site are highly necessary.     

Laparoscopic bilateral adrenalectomy with the use of a single midline hand-assist port.

Indications for bilateral adrenalectomy are rarely present in patients with Cushing's syndrome. The laparoscopic approach to adrenalectomy provides a postoperative course that compares favorably with that of open adrenalectomy, and the hand-assisted technique may provide an additional alternative to the open approach in performing bilateral adrenalectomy.     

Healing,Inflammation, and Fibrosis: When Benign Becomes Cancer: Malignant Degeneration of Chronic Inflammation

Chronic inflammation, long implicated in the genesis of malignancy, is now understood to underlie an estimated 25% of all cancers. The most pertinent malignancies, to the plastic surgeon, associated with the degeneration of chronic inflammation include Marjolin''s ulcer, breast implant-associated large cell lymphoma, radiation-induced sarcoma, and Kaposi''s sarcoma. The cellular and genetic damage incurred by a prolonged inflammatory reaction is controlled by an increasingly understood cytokinetic system. Advances in understanding the chronic inflammatory cascade have yielded new therapeutics and therapeutic targets.     

'Hop the skip' with extended segment intestinal biopsy in Hirschsprung's disease

INTRODUCTIONSkip segment Hirschsprung's is an extremely rare condition, with only 25 reported cases in the English language literature. Diagnosis of skip segment Hirschsprung's may be missed as it is rarely suspected at initial surgery.PRESENTATION OF CASEA case report of an infant with total colonic aganglionosis and a skip segment Hirschsprung's in the cecum is presented. A review of literature on current surgical practice is presented along with our suggested modification to the diagnostic and therapeutic algorithm of this rare condition.DISCUSSIONIn our patient the initial leveling colostomy was incorrectly sited at the level of the first detected ganglionated segment. Subsequent recurrent episodes of enterocolitis led to a diagnosis of a long skip segment involving the ascending colon, cecum and terminal ileum. Correct leveling colostomy and a subsequent Duhamel procedure led to a good outcome.CONCLUSIONDetermination of the transition zone in Hirschsprung's may be incorrect if intestinal biopsies are terminated at the first evidence of ganglion cells. Extended segment intestinal biopsies should be included in the algorithm for management of long segment Hirschsprung's disease and will enable the surgeon to correctly detect rare manifestations such as skip segment disease at the initial procedure.     

Treatment of forearm diaphyseal defect by distraction compression bone transport and continued distraction for radial head reduction: A case study

Treatment of infected forearm nonunion and defects represents a difficult task for the operating sur geons. Conventional methods like composite and vascularized fibular grafts and the induced membranes filled with cancellous autografts or the Masquelet technique have been reported to be useful and suc cessful, but sometimes it is difficult to predict the outcome and cannot address simultaneous deformities or the need to apply gradual distraction for reduction of a chronically dislocated radial head. Ilizarov technique has an answer for such conditions. We report a 43 years old man with infected ulnar defect and dislocated radial head as a result of infected Monteggia fracture: the patient was successfully treated by Ilizarov bone transport after failed attempts by bone spacer and fibular graft.     

Crohn's disease with enterolith treated laparoscopically.

Symptomatic enteroliths are relatively rare. Most occur from ingestion of undigestible materials such as pits or bones. Primary enteroliths are usually from the condition of partial bowel obstruction, diverticular type diseases such as Meckel's or congenital bands. Gallstone ileus is also a more common cause of gastrointestinal stones. Enteroliths associated with Crohn's disease is an extremely rare condition with fewer than 25 cases reported in the literature. Presented herein is such a case successfully treated laparoscopically.     

Anterior radical debridement and anterior instrumentation in tuberculosis spondylitis

The conventional procedure in the treatment of vertebral tuberculosis is drainage of the abscess, curettage of the devitalized vertebra and application of an antituberculous chemotherapy regimen. Posterior instrumentation results are encouraging in the prevention or treatment of late kyphosis; however, a second-stage operation is needed. Recently, posterolateral or transpedicular drainage without anterior drainage or posterior instrumentation following anterior drainage in the same session has become the preferred treatment, in order that kyphotic deformity can be avoided. Information on the use of anterior instrumentation along with radical debridement and fusion is scarce. This study reports on the surgical results of 63 patients with Pott's disease who underwent anterior radical debridement with anterior fusion and anterior instrumentation (23 patients with Z-plate and 40 patients with CDH system). Average age at the time of operation was 46.8+/-13.4 years. Average duration of follow-up was 50.9+/-12.9 months. Local kyphosis was measured preoperatively, postoperatively and at the last follow-up visit as the angle between the upper and lower end plates of the collapsed vertebrae. Vertebral collapse, destruction, cold abscess, and canal compromise were assessed on magnetic resonance (MR) images. It was observed that the addition of anterior instrumentation increased the rate of correction of the kyphotic deformity (79.7+/-20.1%), and was effective in maintaining it, with an average loss of 1.1 degrees +/-1.7 degrees. Of the 25 patients (39.7%) with neurological symptoms, 20 (80%) had full and 4 (16%) partial recoveries. There were very few intraoperative and postoperative complications (major vessel complication: 3.2%; secondary non-specific infection: 3.2%). Disease reactivation was not seen with the employment of an aggressive chemotherapy regimen. It was concluded that anterior instrumentation is a safe and effective method in the treatment of tuberculosis spondylitis.     

Scoliosis in Gordon's syndrome

Gordon's Syndrome is described as an autosomal dominant condition with the characteristics of short stature, a stiff spine, camptodactyly (89%), cleft palate (27%) and club feet (73%). The authors present a case report of a patient with this rare entity complicated by an unusual complex spinal deformity. There are no prior reports in the literature concerning operative or nonoperative management of deformity in this patient population. Scoliosis in Gordon's Syndrome shares the characteristics of an arthrogrypotic neuromuscular curve and demands extensive soft tissue release for optimal correction.     

Retroperitoneoscopic lumbar sympathectomy for buerger's disease: a novel technique.

BACKGROUND: The conventional lumbar sympathectomy procedure through the extraperitoneal route requires a muscle cutting-splitting incision, which leads to significant postoperative pain and prolonged convalescence. With increasing experience in retroperitoneoscopic procedures, we did a pilot study to explore the role of retroperitoneoscopy in lumbar sympathectomy. We describe herein our technique used for the surgery. METHODS: The patient was placed in a lateral position. A 15-mm incision was made just below the 12th rib, and retroperitoneal space was created using blunt finger dissection. A custom-made, large balloon was inserted and inflated with the equivalent of 750 mL to 1000 mL of saline. The second 10-mm port was placed in line with the first port above the iliac crest. The third and fourth 5-mm ports were placed anterior to the first 2 ports. Peritoneum was retracted anteriorly. The medial border of the psoas muscle was used as a landmark and a chain identified immediately medial to it. Lumbar vessels were ligated on the right side. The first to fourth lumbar sympathetic ganglia were removed with the intervening chain. The port sites were closed without a drain. RESULTS: We attempted and successfully completed this procedure in 8 patients; 6 on the left side and 2 on the right side. The average operating time was 38 minutes. The mean hospital stay was 1.5 days. All patients had symptomatic pain relief and clinical improvement. CONCLUSIONS: Retroperitoneoscopic lumbar sympathectomy is a safe and effective procedure. It has a short convalescent time and minimal morbidity; hence, it is a viable alternative for the open procedure.     

Giant Neurofibroma in the Right Lower Limb of a 26-Year-Old Woman: Report of a Case

Neurofibromatosis (NF) is a genetically inherited, autosomal-dominant disease with an incidence of 1 in 3000 live births. There are two types of NF, NF 1 and NF 2, and NF 1 is the most common. This study reports on the diagnosis, treatment, and related family medical history of a rare case with NF-1 in the right lower limb.     

A rare combination injury of type III Monteggia fracture dislocation and ipsilateral epiphyseal fracture of distal radius in children. Is there a probability of missing the Monteggia component?

Combined type III Monteggia fracture dislocation and ipsilateral distal radial epiphyseal fracture is a very rare injury. Because of difficulty in performing the proper clinical evaluation of a child in an acute injury state, one of the components of this combined injury may be missed. We report a tenyear-old male child with this kind of injury where the Monteggia lesion was initially missed at the emergency department. Later we found the combined epiphyseal fracture of distal radius and Monteggia lesion in the ipsilateral side of the same limb which was managed by closed reduction and K-wire fixation. Bony union as well as wrist and elbow motion was complete 3 months after surgical intervention.     

马德隆畸形1例报告并文献回顾

正患儿,女,8岁,2015年因玩耍时不慎摔倒,右腕关节着地,当即出现疼痛,腕关节活动尚可,未行治疗,受伤1周后疼痛自然缓解。2016年初家长发现患儿右前臂尺背侧远端隆起,有压痛,认为与既往摔倒受伤有关,就诊于新疆某市人民医院,行X线检查后诊断为陈旧性右下尺桡关节脱位,原拟手术治疗,后因患儿上学放弃治疗。但患者前臂畸形进一步