Anoplasty for low anorectal malformation.

BACKGROUND: The presence of a slightly anterior, stenotic anus is a low anorectal malformation. It is a type of rectoperineal fistula. The cardinal symptoms are constipation and straining at stool. Hendren described a technically simple anoplasty for this condition in 1978. Controversy about terminology has led to a lack of clarity about this condition. METHODS: Clinical data were reviewed about the authors' patients, who had Hendren anoplasty, from 2009 to 2015. Followup data were obtained from office visits and telephone interviews with families and primary care doctors. RESULTS: Seven patients (4 boys) presented with intractable constipation. All had a stenotic anal orifice, located anterior to the center of the anal wink, within the pigmented perianal skin. In 4 of 7 cases, the lesion was not recognized at birth. Anoplasty was performed at a median age of 8months (range 6-28). Late followup information was obtained on six of the seven patients at a median of 32months (range 28-61). Four reported no or minimal laxative requirement and two reported daily laxative use but good symptom control. CONCLUSIONS: Low anorectal malformation/rectoperineal fistula may be overlooked in the newborn. When symptomatic, it may be corrected by a simple anoplasty with excellent results.

Midterm results of a novel technique to salvage autogenous dialysis access in aneurysmal arteriovenous fistulas.

PURPOSE: Over the last decade, K-DOQI guidelines have increasingly emphasized the importance of autogenous arteriovenous fistulas (AVF) for dialysis access. A complication of AVF is aneurysmal dilatation with a subset developing massive diffuse aneurysm. Treatment of massive aneurysmal AVF generally involves either ligation or resection with use of prosthetic interposition. To maintain an all-autogenous access, we developed a procedure to treat massive aneurysmal AVF in which the luminal diameter is reduced, excess length is resected, and the new reconstructed AVF is re-tunneled for continued use. The purpose of this study is to examine the midterm outcomes of this novel procedure. METHODS: Over a 4-year period, the reduction/revision procedure was performed on 19 patients with an AVF diameter of 4-7 cm. Indications for operation were thrombosis, skin breakdown, infection, bleeding, and/or poor flow. Revision was performed by resecting redundant length, reducing diameter, and then reconstructing the fistula. RESULTS: The median patient age was 47, interquartile range (IQR) 29. There were 13 men and 6 women. The median follow-up was 23 months, IQR 22. The median primary patency was 14 months, IQR 24. The median secondary patency was 16.5 months, IQR 26. Two patients died, one AVF thrombosed, and two were ligated secondary to infection. Three fistulae developed a stenosis that was treated with percutaneous angioplasty. There are no recurrent aneurysms to date. CONCLUSION: Surgical resection of excess length, reduction of luminal diameter, and reconstruction is a viable option for the treatment of complicated massive diffusely aneurysmal AVF. This technique offers the ability to maintain the benefits of an all autogenous dialysis access while conserving future dialysis sites.

Hemodialysis-related steal syndrome: predictive factors and response to treatment with the distal revascularization-interval ligation procedure.

Hand ischemia due to steal causes major disability in affected members of the hemodialysis population. Between February 2000 and March 2007, 24 patients aged 37-77 years were identified who developed hand ischemia distal to a hemodialysis access and required a distal revascularization-interval ligation (DRIL) procedure. Of the 24 patients, 22 (92%) were diabetic, 14 (58%) were women, 7 (29%) had prosthetic grafts, and 17 (71%) had fistulas, all originating from the brachial artery. Duration between the initial dialysis access and the DRIL procedures ranged 12 hours to 10 months. Conduits used were saphenous vein in 13 (54%) cases, cephalic vein in 3 (12%) cases, basilic vein in 5 (21%) cases, and prosthetic grafts in 3 (12%) cases. There were no operative deaths. Improved blood flow and relief of symptoms were observed in 23 (96%) patients. The procedure failed early in one patient who had thrombosis of a prosthetic graft. Two patients required digital amputations. At a median follow-up of 50 months, 14 (58%) patients died using the access requiring the DRIL, 2 (8%) did not require dialysis, 3 (12%) were using a new access, and 5 (21%) were still using the access that had required the DRIL. In late follow-up, only one DRIL bypass required revision and the remainder were patent. One patient developed an ischemic hand 5 years after his DRIL procedure despite a patent bypass. The development of ischemic steal requiring performance of a DRIL procedure is most likely to occur in diabetic patients with dialysis access originating from the brachial artery. The procedure is effective in ameliorating symptoms while preserving the vascular access. The high long-term mortality rate observed in this series underscores the fact that patients requiring a DRIL procedure represent a subset of dialysis patients with advanced diabetic vascular disease and a limited life expectancy. Despite the effectiveness of the DRIL procedure, efforts should be concentrated on prevention of ischemic steal in order to lessen the morbidity and expense of this condition in the dialysis population.

Chronic traumatic and congenital diaphragmatic hernias: presentation and surgical management.

BACKGROUND: The diagnosis of chronic diaphragmatic hernias, whether due to congenital defects or trauma, may be difficult to make and may rely on clinical suspicion in the setting of persistent nondiagnostic radiographic findings. Repair is indicated to avoid catastrophic cardiopulmonary compromise and/or incarceration of abdominal organs. STUDY OBJECTIVES: To review the varied presentations and treatment of chronic diaphragmatic hernia. DESIGN: Retrospective review. SETTING: University of Washington and Harborview Medical Center, Seattle, Washington. PATIENTS: Between 1997 and 2001, nine patients presented with chronic diaphragmatic hernia (two congenital cases, seven post-traumatic cases). Four cases involved the right diaphragm. The following clinical features were noted: asymptomatic, chest radiograph showing bowel herniation (n=1); chest wall mass (n=1); asymptomatic with the chest radiograph showing marked elevation of hemidiaphragm (n=1); dyspnea with the chest radiograph showing marked elevation of hemidiaphragm (n=1); diarrhea and heartburn (n=1); generalized gastrointestinal upset (n=1); recurrent pneumonia (n=2); recurring effusions (n=4); and dyspnea on exertion (n=5). INTERVENTIONS: Diagnosis was confirmed by chest radiograph in two patients, chest computed tomography scan in one patient, barium studies in three patients and thoracoscopy in three patients. All hernias were repaired via thoracotomy, and two hernias were repaired with artificial patch. CONCLUSIONS: Patients with chronic diaphragmatic hernias present with a variety of symptoms and radiographic findings. When radiology or symptoms suggest bowel involvement, barium studies are appropriate. In other cases, chest computed tomography scans and/or thoracoscopy are useful. Repair is accomplished through the ipsilateral chest, with primary repair of the diaphragm preferred over patch repair.