Urological aspects of the tethered cord syndrome

We performed urological evaluations in 18 patients with the tethered spinal cord syndrome. Of the patients 4 had low grade vesicoureteral reflux but none had significant upper tract anomalies. Urodynamic studies documented a flaccid bladder in 9 patients (5 with supersensitivity to bethanechol), an uninhibited bladder in 5, mixed bladder dysfunction in 2 and normal function in 2. Repeat urodynamic studies were performed in 15 patients at least 6 months after release of the tethering. Of 8 patients with flaccid bladders studied postoperatively 6 had significant changes: 2 became normal, 2 had limited contractions and 2 had evidence of upper motor neuron abnormalities. Supersensitivity to bethanechol was of no predictive value. All 4 patients with uninhibited bladders who were evaluated preoperatively and postoperatively had improved and 3 currently are entirely normal. Careful urological evaluation of patients with the tethered spinal cord syndrome is helpful not only for diagnosis but also for appropriate management preoperatively and postoperatively. The fact that lower urinary tract function improves in some patients should encourage early and aggressive neurosurgical management of patients with this syndrome.     

Jejunoileal bypass: Late metabolic sequelae and weight gain

Of 101 patients originally operated on, the status of 98 is known. Given the mortality and reanastomosis rates, the operation must be considered an absolute failure in 28 percent of the patients. Given the other complications that appear (or persist) late postoperatively, only 18 percent of the entire series of patients have had what can be considered a good result. We therefore conclude that intestinal bypass is not an appropriate operation for morbid obesity and that complete long-term follow-up is essential for all patients who undergo the operation, despite what might seem to be a smooth course in the 1st 2 years postoperatively.     

Acute Wernicke's Encephalopathy following Bariatric Surgery: Clinical Course and MRI Correlation

Postoperative complications and nutritional deficits resulting from bariatric surgery can lead to severe vitamin-deficiency states, such as Wernicke's encephalopathy (WE). Patients with acute WE generally present with the classic clinical triad of inattentiveness, ataxia, and ophthalmoplegia. We describe a patient who presented with acute WE at 2 months after laparoscopic bariatric surgery. Initial MRI of the brain demonstrated the characteristic injuries of WE, and repeat imaging showed resolution after 4 months of thiamine supplementation, at which time the patient had normal gait but persistent memory deficits. Even with early recognition and aggressive therapy, acute WE commonly results in permanent disability due to the irreversible cytotoxic effects on specific regions of the brain. Since the clinical onset of acute WE follows a predictable time-course in postbariatric surgery patients with malnutrition, we recommend prevention by administration of parenteral thiamine beginning at 6 weeks postoperatively in malnourished patients.     

Vasoplegic syndrome after off-pump coronary artery bypass surgery.

OBJECTIVE: The vasoplegic syndrome (VS) has been implicated in life-threatening complications after open heart surgery, where the whole-body inflammatory reaction is attributed to the cardiopulmonary bypass (CPB). Off-pump coronary artery bypass grafting (OPCAB) has been recently achieving growing enthusiasm mainly due avoiding the side effects of CPB. However herein the occurrence of VS in OPCAB is reported. METHODS: The vasoplegic syndrome usual findings occurring in the early postoperative period include severe hypotension, tachycardia, normal or elevated cardiac output and low systemic vascular resistance. Four patients underwent to OPCAB presented all the signs of VS intraoperatively or within the first 6 postoperative h. RESULTS: The patients needed aggressive vasoactive drug support for hemodynamic stabilization and all of them developed complications. These patients also had tendency to require administration of blood and blood derivatives due to diffuse and oozing type bleeding. Mean intensive care unit stay of surviving patients was 70 h and mean period of postoperative hospitalization was 9 days. Tumor necrosis factor-alpha blood levels in one patient were elevated postoperatively though no signs of infection were observed. One patient died. CONCLUSIONS: Although vasoplegic syndrome can complicate OPCAB surgery, the rationale for avoiding CPB remains valid considering the benefits provided by OPCAB.     

Myositis ossificans after a supracondylar fracture of the humerus in a child

Myositis Ossificans After a Supracondylar Fracture of the Humerus in a Child. In addition to the better known complications of supracondylar humerus fractures, myositis ossificans is often listed as a less common complication. This complication is extremely rare in children and historically has been attributed to high-energy trauma, manipulation, surgical intervention, aggressive passive range-of-motion exercises, or associated head injury. We present a case report of a 3-year-old girl who developed myositis ossificans after a low-energy supracondylar fracture of the humerus despite having been treated without manipulation, surgery, or physical therapy. This report illustrates that supracondylar humerus fractures can be complicated by myositis ossificans despite the best attempts at prevention.     

Orthotopic liver transplantation for alagille syndrome

Alagille syndrome (AS) is a dominantly inherited, multisystem disorder involving the liver, heart, eyes, face, and skeleton. From October 1997 through July 2004, 260 pediatric orthotopic liver transplantations (OLTx) were performed in 231 patients. This report describes 21 patients of median age 1.95 years (range, 0.7-16.7) who had alagille syndrome. We present the technical features of the OLTx, incidence and type of complications, medical conditions related to the syndrome, need for retransplantation, as well as patient and graft survival rates. A split liver technique was used in 16 patients (76%) who received a left lateral segment (LLS) graft whereas 7 patients (33%) received a whole liver. Only cadaveric donors were used. The major surgical complications requiring reintervention in 11 patients (52%) included biliary problems (19%) and vascular complications (17%). One case of hepatic artery thrombosis required retransplantation. Three recipients (14%) died. All other patients are alive with an actuarial survival rate of 90% at 1 year and 80% at 5 years. The actuarial graft survival rate is 85% at 1 year and 75% at 5 years. Patients with AS, despite the associated cardiovascular anomalies, can be treated successfully by a combined approach between cardiologist, radiologist, cardiothoracic, and liver transplant surgeons. With careful planning and operative management, the results are comparable with those obtained with other more common cholestatic diseases.     

Surgical treatment strategies and outcome in patients with breast cancer metastatic to the spine: a review of 87 patients

Aggressive surgical management of spinal metastatic disease can provide improvement of neurological function and significant pain relief. However, there is limited literature analyzing such management as is pertains to individual histopathology of the primary tumor, which may be linked to overall prognosis for the patient. In this study, clinical outcomes were reviewed for patients undergoing spinal surgery for metastatic breast cancer. Respective review was done to identify all patients with breast cancer over an eight-year period at a major cancer center and then to select those with symptomatic spinal metastatic disease who underwent spinal surgery. Pre- and postoperative pain levels (visual analog scale [VAS]), analgesic medication usage, and modifed Frankel grade scores were compared on all patients who underwent surgery. Univariate and multivariate analyses were used to assess risks for complications. A total of 16,977 patients were diagnosed with breast cancer, and 479 patients (2.8%) were diagnosed with spinal metastases from breast cancer. Of these patients, 87 patients (18%) underwent 125 spinal surgeries. Of the 76 patients (87%) who were ambulatory preoperatively, the majority (98%) were still ambulatory. Of the 11 patients (13%) who were nonambulatory preoperatively, four patients were alive at 3 months postoperatively, three of which (75%) regained ambulation. The preoperative median VAS of six was significantly reduced to a median score of two at the time of discharge and at 3, 6, and 12 months postoperatively (P < 0.001 for all time points). A total of 39% of patients experienced complications; 87% were early (within 30 days of surgery), and 13% were late. Early major surgical complications were significantly greater when five or more levels were instrumented. In patients with spinal metastases specifically from breast cancer, aggressive surgical management provides significant pain relief and preservation or improvement of neurological function with an acceptably low rate of complications.     

Thoracic cage deformities in the early diagnosis of the Marfan syndrome and cardiovascular disease

The Marfan syndrome is frequently complicated by cardiovascular abnormalities. Of these, aortic dissection and aortic valve regurgitation are the most life-threatening. The most noticeable abnormalities of the Marfan syndrome--the skeletal abnormalities--may be subtle and limited. Presented here are five reports of cases of the Marfan syndrome. All patients had potentially lethal cardiovascular complications. Either the syndrome had not been previously diagnosed or the patient had not been adequately monitored despite the the presence of thoracic cage deformities present from youth. The purpose of this report is to heighten recognition of the association of thoracic cage deformities with the Marfan syndrome to permit early diagnosis of the associated cardiovascular complications. Surgical management of these complications can favorably alter the natural history of the Marfan syndrome.     

Relief of Iliofemoral Vein Occlusion with the Palma Bypass in a Patient with Klippel Trenaunay Syndrome

Klippel Trenaunay syndrome is usually managed conservatively with surgery reserved for patients with symptomatic but mild cosmetic deformity or persistent venous hypertension despite nonoperative measures. Deep venous reconstruction is necessary in a small group of patients who present with significant chronic venous insufficiency due to hypoplastic, absent, or occluded deep venous outflow. Most often, venous outflow occlusion results from inadequate or surgically removed superficial collateral veins or from complications of endovascular procedures. In this article, we review the English-language literature and the Mayo Clinic experience, and report on a patient with Klippel Trenaunay syndrome who developed symptomatic iliofemoral venous occlusion following iliac vein stenting, which was successfully treated with a crossover saphenofemoral vein (Palma) bypass. Presented at the Fourth Pacific Vascular Symposium on Venous Diseases, Kohala Coast, Hawaii , November 12-16, 2002.     

Parenteral nutrition in esophageal cancer patients

A review of operative therapy in 244 patients with esophageal cancer from 1960 to 1980 was done to evaluate the impact of TPN in 72 patients treated from 1973 to 1980 with 43 non-TPN patients treated during the same period and to 129 patients operated upon before 1973. Mean age, sex distribution, site, stage, and treatment of the disease were similar for the two study groups. The TPN group lost less weight during treatment (3 lbs vs. 11 lbs) and had fewer overall complications postoperatively (24% vs. 41%). Significant reductions in major wound, infectious, and postoperative complications were noted in these patients who received at least 5 days of preoperative TPN compared with postoperative TPN or the non-TPN groups (4% vs. 24% and 23%). Malnourished esophageal cancer patients can more safely undergo aggressive operative therapy and radiation treatment when adequate perioperative nutritional support is added to the treatment armamentarium.     

Management of peritonitis in the critically ill patient

Severe secondary peritonitis carries significant mortality, despite advancements in critical care support and other therapies. Surgical management requires a multidisciplinary approach to guide the timing and the number of interventions necessary to eradicate the septic foci and create optimal healing with the fewest complications. Research is needed regarding the best surgical strategy for very severe cases. The use of deferred primary anastomosis seems safe in patients presenting with hemodynamic instability and hypoperfusion. These patients have a high risk of anastomotic failure and fistula formation. Allowing for aggressive resuscitation and judicious assessment of the progression of local inflammation are safe strategies to achieve the highest success and minimize serious and protracted complications in patients who survive the initial septic insult.     

Gastrointestinal manifestations of chronic granulomatous disease

OBJECTIVE: Chronic granulomatous disease is a rare clinical entity characterized by recurrent infective and inflammatory complications. Patients are usually assigned to specialist centres, but nonspecialist clinicians may be required to treat these patients in the emergency setting. This review serves as a management guide to those clinicians who are faced with patients presenting with gastrointestinal manifestations of chronic granulomatous disease. METHODS: This review is based on a literature search (Medline and NLM PubMed) with manual cross-referencing of all articles related to gastrointestinal chronic granulomatous disease. RESULTS: Gastrointestinal tract involvement is present in most affected patients. Clinical presentation can mimic common surgical complications such as colitis, perianal sepsis, gastric outlet obstruction and liver abscess. A history of recurrent infections during childhood is common. Management involves haematological, microbiological, endoscopic and radiological investigations. Treatment modalities include early aggressive empirical antimicrobial therapy for sepsis, immunomodulation for inflammatory complications and surgical drainage of abscesses. CONCLUSION: Early involvement of a centre with immunological expertise combined with aggressive management of complications significantly improves morbidity and mortality from this rare condition.     

Cytoreductive surgery in patients with advanced-stage carcinoid tumors

The role of aggressive surgical resections as well as criteria for resectability in patients with advanced carcinoid tumors is not clearly defined. Thirty patients (17 male and 13 female) who were previously diagnosed to have "unresectable carcinoid disease" were treated using a multimodality approach over a period of 2 years. Extensive liver involvement was present in 28 of 30 (93%) of the cases. Small bowel involvement was noted in 22 of 30 (73%), and peritoneal/retroperitoneal/mesenteric invasion was observed in 15 of 30 (50%) of the cases. Three patients had remote metastases (brain, bone, and eye). Twenty of 30 (66%) patients had carcinoid syndrome with severely disabling symptoms. Eight patients (26%) had small bowel obstruction. All patients underwent at least one surgical exploration/intervention. Radiofrequency ablation (RFA) of one or more liver lesions was performed as an adjunct in 22 of 30 (73%) patients. Six patients (20%) had a second surgical procedure. There were 11 complications in eight patients (27%) after the initial operation. Median hospital stay for patients who underwent RFA only, RFA/liver resection, and liver resection with abdominal tumor debulking were 2, 4, 8, and 16 days respectively. Twenty-five of 30 patients (83%) showed symptomatic improvement. Mean pre- and postoperative Karnofsky physical performance scores were 55 and 85 respectively (P < 0.02). Small bowel obstruction was due to adhesions in five patients. All patients with intestinal obstruction had complete relief of their symptoms postoperatively. 5-Hydroxyindolacetic acid levels decreased by 50 per cent in all patients with follow-up determinations available. Aggressive surgical exploration and tumor debulking could be performed with significantly improved symptomatic outcome and relatively minor complications. Longer follow-up is needed for assessment of effect on survival.     

Haemolymphangiomas of the lower extremities: two case reports and review of the literature

Haemolymphangiomas are congenital malformations of the vascular system, benign in origin. They can be either defined at birth or in early childhood, but the recurrence rate is usually high and complete resection is difficult to be achieved. We report two cases of haemolymphangiomas present at the tibias. We used radiographic control, ultrasound and magnetic resonance to detect the nature of the tumour and the anatomical relation with the surrounding tissues providing the appropriate surgical treatment. The diagnosis was confirmed postoperatively by histopathology. The first case presented multiple recurrences because of insufficient draining and immobilization of the leg, whereas the second case had no complications or recurrence postoperatively. Both patients had no complications or recurrence in 24-month follow-up. Haemolymphangiomas are very challenging to be treated as they can grow slowly and remain asymptomatic for many years. Sometimes they can be very aggressive in growth without characteristics of malignancy. The choice of treatment is the complete surgical excision of the tumour, sufficient and long-lasting draining, and immobilization of the extremity to abolish the possibility of recurrence. Other methods of treatment did not show better results than surgical resection.     

Surgical management of life-threatening and disfiguring sequelae of fulminant meningococcemia

In recent years, because of prompt diagnosis and effective, aggressive resuscitation, the majority of infants and children with fulminant meningococcemia are surviving. From 1974 through 1984, 135 patients with this diagnosis were treated, and 126 of them survived. Although a purpuric skin rash developed in almost all of these patients initially, in eight of them it progressed to multiple confluent areas of cutaneous gangrene, usually associated with extensive necrosis of underlying subcutaneous fat, fascia, skeletal muscle, and even bone. Tissue necrosis seemed to be most extensive in regions of reduced blood flow, such as the extremities, but it almost never followed a pattern of anatomic vascular distribution. A most significant microscopic finding was the presence of multiple fibrin thrombi in vessels, often in close proximity with the foci of tissue necrosis. Five children who ranged in age from 6 months to 12 years required operation. Initially, all surface wounds were treated like full-thickness burns with silver sulfadiazene (Silvadene) dressings. Once the patient's condition had stabilized and the extent of tissue necrosis was apparent, all necrotic tissue was excised and the resulting wounds were temporarily covered with biologic dressings to assure a clean, viable base for skin grafting. Because the resulting quality of life postoperatively in all five surviving patients has been satisfactory, we recommend an aggressive surgical approach in patients with fulminating meningococcemia, despite what may initially appear to be devastating and even lethal complications of this disease.     

Wet gangrene in hemodialysis patients with calciphylaxisis is associated with a poor prognosis

Calciphylaxis is a rare syndrome characterized by progressive vascular calcification and ischemic tissue loss in patients with chronic renal failure. We report our five-year experience with five patients who developed foot gangrene due to calciphylaxis. All five patients had characteristic clinical, laboratory, and radiologic findings of the disorder, but no diagnostic variable was uniformly present. All five had progressed to advanced gangrene at the time of surgical consultation. Despite aggressive local attempts to control infection, all five patients died of septic complications. Parathyroidectomy was performed in three patients but did not alter the course in any case. This small experience suggests that the outcome of foot gangrene associated with calciphylaxis is predicated on the degree of tissue loss. Aggressive local wound care does not appear to be adequate to control infection in patients who have already developed gangrene. Although parathyroidectomy may have important long-term advantages in patients with calciphylaxis, it does not appear to affect outcome in these advanced cases.     

Anaesthesia for advanced rectal cancer patients treated with combined major resections and intraoperative radiotherapy

BACKGROUND AND OBJECTIVE: Multimodality treatment for patients with locally advanced primary or locally recurrent rectal cancer, including high-dose preoperative external beam radiotherapy, extensive surgery and intraoperative radiation therapy, decreases the local recurrence rates and improves survival. During this aggressive operation, the anaesthesiologist is faced with potential problems such as major transfusion requirements, hypothermia, intraoperative position changes, the need to transport the patient to the intraoperative radiation therapy applicator, and the risks associated with remote monitoring of the patient during the 10 min intraoperative radiation therapy application. The anaesthetic management and perioperative results were evaluated for the anaesthetic results and the complications. METHODS: One-hundred-and-six patients undergoing the multimodality treatment between February 1994 and March 2000 for locally advanced primary (n = 50) and locally recurrent rectal cancer (n = 56) were retrospectively evaluated for their anaesthetic results and complications. RESULTS: All patients were operated upon using a combination of general and epidural anaesthesia. The average duration of anaesthesia was 6 (range 3-10.5) h and the mean blood loss 3.6 (range 0.4-14) L. All patients recovered well from anaesthesia. Two patients (2%) died in the intensive care unit (34 and 48 days postoperatively) because of adult respiratory distress syndrome following postoperative haemorrhage. Severe haemorrhage during or after the operation was significantly related with the development of adult respiratory distress syndrome (P < 0.0001). CONCLUSION: With adequate preoperative assessment and optimalization of the patient's condition, maintaining peroperative haemodynamic stability with the help of adequate remote monitoring, early and fast transfusion, and multidisciplinary communication, anaesthetic complications can be minimized.     

Cystotomy, temporary urinary diversion and bladder packing in the management of severe cyclophosphamide-induced hemorrhagic cystitis

Severe hemorrhage from cyclophosphamide-induced cystitis sometimes requires aggressive open management when conventional endoscopic and intravesical therapies have failed. We present 2 patients with intractable hematuria who were managed by open cystotomy, temporary urinary diversion via external ureteral stents, and continuous postoperative bladder packing with gauze and hemostatic agents. Both patients had a dramatic improvement in the hematuria immediately postoperatively and 1 with prolonged followup has no urological disability. We suggest that this procedure be considered for management of severe hemorrhagic cystitis as an alternative to more aggressive surgical therapies, such as formal urinary diversion with cystectomy.     

Successful parathyroidectomy in primary hyperparathyroidism: a clinical follow-up study of 212 consecutive patients

The long-term clinical courses of 212 "cured" (normocalcemic) patients were analyzed for 1 to 25 years (mean, 6.8 +/- 5.4 years). Preoperatively, 181 patients (85%) were classified as having typical symptoms, 22 patients (11%) as having minimal symptoms, and nine patients (4%) as having no symptoms of primary hyperparathyroidism (PHP). Although the formation of urinary calculi was stopped in 91% of patients, deteriorated renal function and hypertension were seen in patients with symptoms (14% and 8%, respectively) and patients with minimal symptoms of PHP (6% and 15%, respectively). Renal function changes and hypertension were unpredictable despite normalization of the hyperactive parathyroid metabolism and had decisive results: 7% of the patients died of uremia or of the consequences of hypertension (stroke). Large, multiple bone lesions healed functionally and were of no prognostic significance. In the majority of patients with symptoms of PHP, gastrointestinal manifestations healed postoperatively, but two patients who had no preoperative gastrointestinal complaints died of acute pancreatitis. Almost all symptoms of the hypercalcemia syndrome disappeared immediately and permanently in patients with symptoms and patients with minimal symptoms of PHP. Neither deterioration of renal function nor elevation of blood pressure were observed postoperatively in "cured" patients who showed no symptoms of PHP preoperatively. Even in these patients, immediate surgical treatment may have avoided the complications of chronic renal failure or hypertension. As soon as organic manifestations, even in mild form, have been established, it seems impossible to predict the course and to prevent an unfavorable clinical outcome.     

Lumbopelvic fixation for multiplanar sacral fractures with spinopelvic instability

Sacral fractures with both transverse and bilateral vertical fracture components are by definition multiplanar fractures, and often present with spinopelvic instability and cauda equina deficits. The treatment is challenging. Between 2006 and 2009, we treated nine such patients at our trauma centre. There were six men and three women, with a mean age of 32.2 years. Preoperative neurologic deficits were noted in seven patients; four patients had complete cauda equina paralysis, and three patients had incomplete cauda equina syndrome. All patients were treated using lumbopelvic instrumented fixation without other devices for their multiplanar sacral fractures. Six patients who had neurological deficits and sacral canal compression underwent decompression laminectomy. The mean postoperative follow-up time was 21.7 months (range, 14-32 months). All fractures went on to union without loss of reduction or hardware failure. The mean Gibbons score improved from 3.5 preoperatively to 2.3 postoperatively among the patients who underwent decompression laminectomy. Eight out of nine patients had fair or better results based on radiographic criteria and the Majeed pelvic fracture outcome score. Our experience suggests lumbopelvic fixation can be used for the treatment of multiplanar sacral fractures with spinopelvic instability with a low rate of complications. Neurologic improvement can be expected, but whether surgical decompression results in substantially better neurologic recovery than conservative treatment remains uncertain.