Giant biliary cystadenoma: case report and literature review

Biliary cystadenoma is a very rare cystic neoplasm of the liver. This tumor is insidiously progressive and usually presents in white females in their fifth decade. It has a characteristic appearance on ultrasound, computed tomography, and angiography. The exact etiology of these tumors is unknown, but several theories have been proposed. Historically these cystic tumors have been treated by a variety of techniques including aspiration, fenestration, internal drainage, and resection. Previously reported series have confirmed a >90 percent recurrence rate with anything less than complete excision. In addition biliary cystadenoma is a premalignant lesion and only surgical excision can differentiate it from its malignant counterpart, biliary cystadenocarcinoma.     

罕见胆道结石Bouveret综合征一例 并文献复习

布韦雷氏综合征(Bouveret’s syndrome)是一种少见的胆石性肠梗阻,因胆结石通过“胆肠瘘”导致幽门或近端十二指肠流出道梗阻。本综合征发病率低,国内外仅见零星个案报道,诊治过程不一,治疗也有多重方案。本文结合一病例并文献复习,阐明其病因及临床诊治注意事项。     

Smith-Lemli-Opitz syndrome: case report and literature review

The authors report the case of a 6-week-old boy with the Smith-Lemli-Opitz-syndrome (SLOS) and review the literature on the subject. Intersexuality was suspected and a laparoscopy performed. Abnormalities of the gastrointestinal tract, the lower extremities, and the face prompted DNA analysis, which found a defect of cholesterol biosynthesis in the form of the Smith-Lemli-Opitz-syndrome, a rare congenital defect. The clinical course of this case is compared with similar cases in the literature.     

Relief of intractable pruritis in Alagille syndrome by partial external biliary diversion

Background

Patients with Alagille syndrome (AGS) may develop pruritis, skin hypertrophy, and xanthomas because of chronic cholestasis and hypercholesterolemia. Partial external biliary diversion (PEBD) has been used successfully to treat chronic cholestasis in patients with progressive familial intrahepatic cholestasis (PFIC) and is a potentially useful treatment for patients with severe and intractable pruritis because of AGS.

Methods

Four children with chronic cholestasis and intractable pruritis were treated with PEBD, 1 by surgical cholecystostomy and 3 by cholecystojejunostomy.

Results

Three patients had a known diagnosis of AGS. The fourth was an 11-month-old infant boy with PFIC. The first patient, a 15-month-old boy with AGS, underwent surgical cholecystostomy, which has required frequent tube changes to maintain patency. Three patients underwent PEBD using a segment of jejunum as a conduit between the gallbladder and the skin, where bile is collected in a standard ostomy appliance. Mean follow-up is 15.5 months (range, 9-26 months).All patients experienced rapid and enduring relief of pruritis. Two adolescents with AGS had significant improvement of the hypertrophic skin of their hands. There was one significant complication: the infant with PFIC required reoperation for bleeding from the jejunal anastomosis 1 week after PEBD; he has subsequently done well. One adolescent girl with AGS initially had difficulty with her ostomy because of poor site placement and partial retraction, but nevertheless has managed quite well. The patient treated by cholecystostomy has had excellent relief of his pruritis and is being considered for conversion to cholecystojejunostomy.

Conclusions

Chronic cholestasis caused by AGS can cause debilitating symptoms that are resistant to medical therapy. Partial external biliary diversion is a safe and technically straightforward operation that may be effective for the relief of intractable pruritis and other symptoms in patients with AGS.     

Bertolotti综合征1例报告并文献复习

腰骶部移行椎(LSTV)是腰骶椎最常见的先天性畸形之一,发生率为4.0％ ～ 35.0％,骶化的腰椎椎体形态表现多样,包括从横突肥大到横突与骶骨完全融合等多种类型[1-3].腰化的骶椎椎体倾向方形化,并且有完整的S1/S2椎间盘结构.LSTV易合并腰椎椎间盘突出症及腰椎滑脱,LSTV的上位节段更容易发生退行性疾病,当腰...     

Zinner综合征1例报道并文献复习

目的探讨先天性精囊囊肿伴同侧肾缺如的诊断及治疗,提高对本病的认识。方法报告1例先天性精囊囊肿伴同侧肾缺如,并结合相关文献复习,对先天性精囊囊肿伴同侧肾缺如的诊断及治疗进行分析。结果本例经B超、CTU、NBI膀胱镜等证实为先天性左侧精囊囊肿,合并左肾缺如。行腹腔镜左侧精囊切除术,完整切除左侧精囊及输尿管残端囊肿,术后病理回报:左侧精囊囊肿及输尿管残端。结论对于精囊囊肿需同时完善上尿路检查,明确是否合并其他泌尿系畸形。对于先天性精囊囊肿合并同侧肾缺如患者,腹腔镜手术是一种比较安全有效的治疗方式,值得推广。     

Pinch-off syndrome: case report and collective review of the literature

Pinch-off syndrome (POS) occurs when a long-term central venous catheter is compressed between the clavicle and the first rib. The compression can cause transient obstruction of the catheter and may result in a tear or even complete transsection and embolization of the catheter. POS may be preceded by a finding of "pinch-off sign" on chest X-ray (CXR) films in which the catheter is indented as it passes beneath the clavicle. We performed a collective review of the 109 cases of POS in the medical literature and report 3 new cases. On average, POS occurs 5.3 months after the insertion of the catheter but has ranged from immediately after insertion to 60 months later. If the subclavian vein is used for access, then an upright CXR should be obtained after the procedure and periodically thereafter to rule-out POS. Treatment of POS is removal of the catheter. If the tip of the catheter has embolized, it can usually be retrieved percutaneously with a transvenous snare. POS can be prevented by using the internal jugular vein for access rather than the subclavian vein.     

Zinner综合征1例报道并文献复习

目的:探讨精囊囊肿伴同侧肾缺如(Zinner综合征)的临床诊断和治疗特点,提高对本病的认识。方法:回顾性总结我院1例Zinner综合征患者的诊断、治疗等临床资料。复习文献对Zinner综合征进行讨论。结果:患者23岁,经影像学检查确诊为Zinner综合征,入院后行经直肠精囊囊肿穿刺抽吸术,术后3 d出院。出院6个月后随访,患者排尿终末期会阴部不适症状消失,但经直肠前列腺彩超提示左侧精囊腺积液大小同前,考虑复发。结论:一侧精囊囊肿伴同侧肾缺如,需要考虑Zinner综合征的可能。经直肠精囊囊肿穿刺抽吸术明显缓解患者局部不适症状,但精囊囊肿容易复发,不推荐作为该病治疗的首选方法。     

Megacystis-microcolon-intestinal hypoperistalsis syndrome: case report and review of the literature

We report a case of the megacystis-microcolon-intestinal hypoperistalsis syndrome and review the literature. Generally, this disease affects female neonates and is usually fatal within the first year of life. All patients have microcolon megacystis, intestinal hypoperistalsis, abdominal distention and dilated small bowel. Although the dilated bowel and urinary tract suggest an obstruction, such is not usually the case and surgical intervention is not always necessary. The abdominal distension results primarily from distension of the urinary tract.     

Mirizzi syndrome: a case report and review of the literature

The Mirizzi syndrome is a rare benign cause of obstructive jaundice. The syndrome is a result of the impaction of a large stone, or several smaller ones, in either the Hartmann's pouch or the cystic duct, causing obstruction to the common hepatic duct. It is particularly interesting to surgeons because the surgery has to be carefully planned to avoid unnecessary damage to the common bile duct. Furthermore, it poses a differential diagnosis dilemma for surgeons as well as radiologists because there are no diagnostic procedures or clinical features that have a 100% specificity and sensitivity. As a result, the Mirizzi syndrome often has been mistaken for carcinoma of the gallbladder. We report one case of Mirizzi syndrome to draw attention to the importance of this syndrome and to describe the clinical, diagnostic, and therapeutic aspects of the disease.     

Proteus syndrome: a case report and review of the literature

Proteus syndrome is a rare hamartomatous condition comprising overgrowth of some part of the body in association with various cutaneous, neurovascular, musculoskeletal, and other organ systems. In some cases, there is a family history. The case presented is that of a 6-year-old boy with left hemihypertrophy, widespread capillary hemangiomatosis in the extremities, a pigmented nevus on the right forearm, macrosyndactyly of the second and third fingers, together with a history of urolithiasis. The literature on this rare syndrome is reviewed. Received: 6 August 1999 / Accepted: 15 June 2000     

A case of far-out syndrome: case report and review of the literature

Far-out syndrome (FOS) is a rare disease in which the L5 nerve root is compressed by a transverse process, the sacral ala, and a bony spur at the extraforaminal zone. We report the case of patient with FOS due to a bulging disc and review the literature. This 34-year-old male experienced severe left leg pain and slight motor weakness of the left extensor digitorum. Radiographic studies revealed no abnormality in the spinal canal. The intervertebral disk bulged to the left. Because of fat it was difficult to discern the left L5 nerve root from the sacral ala in the extraforaminal zone. His symptoms failed to respond to conservative treatment and he underwent decompression of the L5 nerve root without fusion. During the operation, the left L5 nerve root was exposed by drilling the sacral ala via the left paraspinal approach. The sacral ala was removed along with the left L5 nerve root to obtain sufficient pulsation and movement of the left L5 nerve root. Neither the intervertebral joint nor the intervertebral disk was removed. The patient's symptoms disappeared immediately after surgery. Due to anatomical factors, the L5 nerve root is easily compressed and the presence of this syndrome should be considered in a differential diagnosis. Careful and appropriate decompression without fusion yielded an excellent outcome.     

Purple urine bag syndrome: case report and literature review

Purple urine bag syndrome (PUBS) is a medical syndrome in which there is purple discoloration of the urine of catheterized patients as well as discoloration of the collecting bag and the associated tubing. This rare condition, which mostly affects women, is generally associated with catheter-associated urinary tract infection, chronic constipation and alkaline urine. PUBS may be caused by sequential chemical reactions involving tryptophan from food in the gastrointestinal tract. The clinical course of PUBS is generally benign, and intensive treatment is not usually needed. We present 3 cases of this unusual and interesting phenomenon and a literature review.     

Turcot syndrome: report of a case and review of the literature

We report a case of Turcot's syndrome in a 20-year old man with multiple adenomatous polyps of the colon and glioblastoma multiforme. Detailed histopathological study of all 25 polyps removed from his colon confirmed the distinct morphological and numerical features of the colonic polyposis in Turcot's syndrome. Moreover, 45% of the total polyps and all polyps with a diameter exceeding 2 cm showed malignant transformation, indicating the precancerous nature of these polyps. These findings are discussed together with data obtained from a literature review of 32 histopathologically confirmed cases of Turcot's syndrome with reference to the possible heterogeneous nature of the syndrome at the present time. The current views on the relationship of Turcot's syndrome to other polyposis coli syndromes are presented.     

Partial priapism secondary to tamsulosin: a case report and review of the literature

Partial priapism is also called partial segmental thrombosis of the corpus cavernosum. It is a rare pathology, and its aetiology, physiopathology and treatment are still not completely understood. To our knowledge, partial priapism due to alpha blockers has not been reported previously in the literature. In this study, a successfully treated case of partial priapism occurring after the usage of alpha blocker is presented and discussed in light of the related literature.