Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group

OBJECTIVE: This multicenter study retrospectively analyzes the data on 114 patients with protein-losing enteropathy after Fontan-type surgery. Special attention was given to the different treatment strategies used and their effect on outcome. METHODS AND RESULTS: In 35 participating centers 3029 Fontan operations were performed. The incidence of protein-losing enteropathy in the survivors was 3.7%. The median age at Fontan-type surgery was 8.2 years (range: 0.6 to 32.9 years). Median age at diagnosis of protein-losing enteropathy was 11.7 years with a median time interval between surgery and diagnosis of 2.7 years (range: 0.1 to 16.4 years). Most patients had edema (79%) and effusions (75%). Hemodynamic data revealed a mean right atrial pressure of 17 +/- SD 5.3 mm Hg with a cardiac index of 2.4 +/- 0.8 L/min/m2. Medical treatment only (n = 52) resulted in a complete resolution of symptoms in 25%, no improvement in 29%, and death in 46%. Surgical treatment (n = 52) was associated with relief of protein-losing enteropathy in 19%, no improvement in 19%, and death in 62%. In 13 patients 16 percutaneous interventions were performed. This resulted in symptomatic improvement after 12 interventions and no improvement after 4 interventions. CONCLUSIONS: We conclude that the current treatment of protein-losing enteropathy after Fontan operation is associated with a very high mortality and morbidity rate. Preventive strategies and new therapeutic approaches are necessary.     

Value of reduction mammoplasty in the conservative treatment of breast neoplasms. Apropos of 70 cases

Breast cancer surgery is on the increase. Until now conservative treatment has been limited to tumors less than 3 cm; it is now extending to surgery on reduced tumors after chemotherapy or radiotherapy. Some cancers still require mastectomy because a carcinologic satisfactory tumorectomy would create a major deformity not compatible with conservative treatment. It is technically possible to perform major tumor resection with good cosmetic results using the reduction mammoplasty technique well known in plastic surgery. Between 1983 and 1991, 70 patients were treated at Henri Mondor Hospital for breast cancer with breast reduction mammoplasty and irradiation. We present the result with an average five years follow-up in terms of the cosmetic results relapses and survival rate. The actuarial local relapse was less than 10%, the survival with local relapse was 86% after 5 years, cosmetic results were good in 81% of cases. The association of reduction mammoplasty and radiotherapy seems to be a good extension of conservative treatment in some large breast tumors.     

Treatment of non-metastatic rhabdomyosarcomas in childhood and adolescence. Results of the second study of the International Society of Paediatric Oncology: MMT84

The second International Society of Paediatric Oncology (SIOP) study for rhabdomyosarcoma (MMT84) had several goals. The two principal aims were: (1) to improve the survival of children with rhabdomyosarcoma; and (2) to reduce the late effects from therapy by restricting the indications for surgery and/or radiotherapy after good response to initial chemotherapy. A further aim was to investigate the role of high-dose chemotherapy in young patients with parameningeal primary tumours. 186 previously untreated eligible patients entered the study. Patients with completely resected primary tumour received three courses of IVA (ifosfamide, vincristine and actinomycin D). Patients with incompletely resected tumour received six to 10 courses of IVA according to stage. Patients achieving complete remission with chemotherapy alone did not usually receive radiotherapy or undergo extensive surgery, but patients remaining in partial remission received local therapy with surgery and/or radiotherapy. Only patients over 5 years of age with parameningeal disease and patients over 12 years with tumours at any site were given systematic irradiation. Complete remission was achieved in 91% (170/186) of all patients. With a median follow-up of 8 years, the 5-year overall survival was 68% (+/- 3% standard error of the mean (SEM) and the 5-year event-free survival 53% (+/- 4% SEM). These results show an improvement over previous SIOP study (RMS75) in which survival was 52% and event-free survival was 47%. Among the 54 patients who exhibited isolated local relapse, 35% (19/54) survived in further remission longer than 2 years after retreatment, including local therapy (surgery +/- radiotherapy). Analysis of the overall burden of therapy received by all surviving children (including primary treatment and treatment for relapse if required) showed that 24% (28/116) were treated by limited surgery followed by three courses of IVA, 29% (34/116) were treated by chemotherapy alone (after initial biopsy) and 13% (15/116) received chemotherapy plus conservative local treatment (limited surgery or radiotherapy for residual disease). Only 34% (39/116) received intensive local therapy defined as radical wide field radiotherapy or radical surgery or both. Compared with the results obtained in the previous SIOP study, treatment in MMT84 was based on response to initial chemotherapy and, despite an overall reduction of the use of local therapy, significantly improved survival for patients with non-metastatic disease. This trial, also for the first time, provides evidence that retreatment after local relapse can achieve long-term second remissions.     

Early active training after lumbar discectomy. A prospective, randomized, and controlled study

STUDY DESIGN: A prospective, randomized, and controlled study was conducted. OBJECTIVES: To evaluate two training programs, both of which started immediately after lumbar discectomy. SUMMARY OF BACKGROUND DATA: In previous studies, patients began physiotherapy between 4 weeks and 60 months after surgery. No studies have been conducted to evaluate a physiotherapy program that begins immediately after surgery. METHOD: Twenty-six patients were treated according to an early active training program. Twenty-six patients were treated with a traditional, less active training program (control group). All patients were examined immediately before and after surgery and 3, 6, 12, and 52 weeks after surgery by an unbiased observer. Two years after surgery, patients completed a questionnaire. Range of motion of the lumbar spine and straight leg raising were measured. pain intensity and location was measured by a visual analog scale. The duration of sick leave was documented. RESULTS: Six and 12 weeks after surgery, patients with dominating residual leg pain had significantly less intense pain in the early active training group than those in the control group (P < 0.05). Twelve weeks after surgery, range of motion of the lumbar spine was significantly more increased in the early active training group (P < 0.01). One year after surgery, there was no significant difference between the groups regarding the duration of sick leave, results in a positive straight leg raising, or pain intensity. Twenty-two (88%) patients in the early active training group and 16 (67%) in the control group were satisfied with the treatment outcome 2 years after surgery (P < 0.10). CONCLUSIONS: Patients rehabilitated according to the early active training program had a better short-term outcome of objective values. At 2 years' follow-up, more patients were satisfied with the result of the operation. The early active treatment program is recommended.     

Home medical equipment suppliers: a critical home care partner. Interview by Sarah F Zarbock

Conventional management of gastroesophageal reflux (GER) and hiatus hernia in children affected with encephalopathy can deteriorate their pulmonary function, already compromised by their leaning position and their spine deformations. The results after laparoscopic surgery for GER in 6 encephalopathy children are reviewed; their ages ranged from 9 to 14 years, their accompanying diseases were: spastic tetraparesia, hydrocephalus, scoliosis, epilepsy and Reye's syndrome. The laparoscopy procedures followed the same surgical steps as open surgery; the surgical time was 3 to 5 hours; the average hospital stay was 3 days. Oral intake started 8 hours after surgery. The successful results of these first cases in this group of patients with neurologic anomalies, suggest that laparoscopic Nissen funduplication is the treatment of choice for GER and hiatus hernia.     

Primary mediastinal seminoma. Efficacy of chemo-radiotherapy alone

A treatment policy must be established for primary mediastinal seminoma. We have treated five patients with this entity during 18 years. All our patients presented with a bulky mass as is usual for this disease. Our first patient was treated surgically and then with radiation, but developed recurrences and died 11 years after the initial diagnosis. Three subsequent patients, one with multiple lymph node metastases, were treated with radiation followed by cisplatin. Our most recent patient received two courses of adriamycin-reinforced PVB, and then radiation for consolidation, followed by another course of chemotherapy. For these five patients, the 5- and 10-year survival rates were both 100%. A review of the literature emphasized the fact that either radiation or surgery has recently been replaced by chemotherapy as the front-line treatment of this rare tumor in light of a better response to the latter form of treatment. We fully agree with this policy. Based on the favorable long-term results of our patients we conclude that chemo-radiotherapy can cure primary mediastinal seminoma, even in its extended form, without surgery. An initial three courses of cisplatin-based chemotherapy like adriamycin-reinforced PVB or BEP should be followed by radiation of up to 4,000 cGy for consolidation. Surgery may play a limited role for tumors that are small at presentation, or for any possible viable residue seen on roentgenograms following chemo-radiotherapy.     

The profile surgery. Mandibular osteotomies

During the years 1987-1994, 31 mandibular osteotomies have been performed in 25 patients, 15 had mandibular alteration alone, 10 of them with prognatism, 2 with microretrognatia and 3 with chin hipoplasia. The other 10 had a combined maxillary-mandibular alteration with hipoplasia and maxillary retrussion. The preoperative work-up included cephalometric and dental study, and a cast model was done to asses the theoretical benefic of the osteotomy. All these patients underwent orthodontic treatment before and after surgery. The results have been good or very good in 96% of the cases. The ortognatic surgery offers significant aesthetic and functional improvement to these patients.     

Jones fracture. Surgical versus nonsurgical treatment

Sixty-three patients with 66 transverse and short oblique fractures of the proximal shaft of the fifth metatarsal bone (Jones fracture) were evaluated an average of five years (range, one to ten years) after the injury. There were 27 acute fractures and 39 chronic, or stress, fractures. The primary treatment was surgical for one third of the injuries and nonsurgical for the others. Surgical treatment consisted of the insertion of medullary screws. Nonsurgical treatment consisted of the application of either a plaster cast or an elastic bandage. Almost one fourth of the fractures treated nonsurgically later had to be treated surgically because of delayed unions or refractures. Late surgery was required in 12% of acute fractures and in 50% of chronic fractures with sclerosis narrowing the medullary canal. Irrespective of the primary treatment, all the patients had full function at the time of the follow-up evaluation, and no nonunions were diagnosed.     

Long-term follow-up after temporal lobe resection for lesions associated with chronic seizures

A follow-up study was conducted on 60 patients who had standard en bloc anterior temporal lobe resection, including mesiotemporal structures, as treatment for temporal lobe lesions associated with chronic, medically intractable seizures. Lesions were identified as glial tumors, hamartomas, or vascular malformations. Long-term outcome was assessed in terms of seizure frequency and certain psychosocial sequelae. Seizure onset occurred at an average age of 15 years (median = 13.5 years), and patients experienced seizures for an average of 13 years prior to surgery. The mean time of follow-up was 8.4 years postsurgery (median = 6 years). The Kaplan-Meier curve at median follow-up showed a seizure-free rate of 80%. Late seizure recurrence was documented for three patients; two had been seizure-free for 10 years and one for 15 years after surgery, before re-onset of seizures in the absence of tumor recurrence. A prolonged history of seizures prior to surgery was associated with a poorer seizure outcome (p = 0.06), suggesting that secondary epileptogenesis at sites distant to the lesion may develop with years of uncontrolled seizures. There was a low tumor recurrence rate of 3.3% (two cases). The psychosocial outcome was generally good, with 67% working or engaged in educational studies, and improvement noted in 59% of cases for one or more of the psychosocial factors investigated. This study confirms that anterior temporal lobe resection for temporal lesions associated with chronic seizures is a successful treatment with a high seizure-free rate following surgery and good psychosocial outcome.     

Use of a preanesthetic video for facilitation of parental education and anxiolysis before pediatric ambulatory surgery

PURPOSE: To find out whether there is a "hidden" group of patients with posterior capsule opacification (PCO) 5 years after cataract surgery and to establish the incidence of PCO. SETTING: Department of Ophthalmology, Sahlgrenska University Hospital, Gothenburg, Sweden. METHODS: A random sample (n = 164) was selected among patients who had extracapsular cataract extraction (ECCE) with intraocular lens implantation in 1991 (N = 1672). All surgically treated cases that required neodymium:YAG (Nd:YAG) laser capsulotomy (n = 37) within the first 5 years after surgery were recorded. Patients still alive 5 years after surgery who had not had Nd:YAG treatment were offered an eye examination to determine whether PCO requiring capsulotomy existed. RESULTS: Thirty-seven of 110 patients (34%) alive 5 years after surgery had an Nd:YAG capsulotomy during the first 5 postoperative years. Follow-up was possible in 51 of 73 untreated patients (70%). Clinically significant PCO according to specified criteria was found in 7 cases (14%). Half of them would benefit from treatment; the other half had visual impairment from other eye disease. CONCLUSIONS: The estimated incidence of PCO 5 years after ECCE was 43%. Five years after surgery, there was an untreated group with clinically significant PCO, approximately 9% of the surgically treated population. This hidden group must be considered in PCO studies.     

Delusions in the aged. A contribution to anthropological understanding

During the last 25 years, substantial progress has been made in the detection and treatment of bone tumors in adolescents and young adults. Due to more effective chemotherapy treatments, patients are now surviving these illnesses and living quality lives. In the past, the only surgical option for treatment of these tumors was amputation. Today, however, limb-sparing procedures such as allograft implantation, together with effective chemotherapy treatments, provide patients with another treatment option. The allograft procedure includes removing the tumor and affected bone and replacing it with bone procured from deceased donors. During the following 2 years, the body does the work of incorporating the allograft and replacing it, at least in part, with new host bone. After a long rehabilitation process, the patient will have a functioning limb. Nursing care of the adolescent undergoing allograft procedure encompasses meeting important physical and psychosocial needs. Adolescents are often still dealing with the emotional and physical stress of having cancer and chemotherapy or chemotherapy side effects at the time of surgery. They are concerned not only with their immediate recovery but also with their long-term prognosis. All treatment modalities, including allograft surgery, impinges on the normal developmental tasks of adolescents. The nursing challenge is to help the adolescent balance the limits of their illness and recovery with normal developmental needs.     

Acute appendicitis following laparoscopic inguinal hernioplasty. Coincidence or complication?]

The Authors report two cases of renal leiomyosarcomas with atypical clinical features. Despite a malignant histological picture, nephron-sparing surgery was performed. The two patients are alive and disease-free at six years and fifteen months respectively. Specific radiologic findings, indications and rationale for conservative treatment are discussed.     

Triple tenodesis of the knee. A soft-tissue operation for the correction of paralytic genu recurvatum

There are two types of genu recurvatum: one with bone deformity, which responds well to osteotomy, and one with posterior soft-tissue laxity with secondary bone changes. To date, no reliable treatment short of arthrodesis has been effective for the second type. An operation for this type of genu recurvatum (post-poliomyelitic) is described: a soft-tissue reconstruction of the lax tissues posterior to the knee joint done in three layers. The operation was done on sixteen knees in fourteen patients, with an average follow-up of four years and three months. The average recurvatum before surgery was 42 degrees and at the time of follow-up it was 6 degrees. Preliminary surgery is often required and precise surgical technique and prolonged bracing after surgery also are needed. All but one patient was made brace-free provided the limb operated on was not flail.     

Cervical pseudarthrosis in ankylosing spondylitis. A case report

STUDY DESIGN: This report illustrates two different cases of cervical pseudarthrosis in ankylosing spondylitis. OBJECTIVES: To point out the extreme rarity of this condition at cervical level, to discuss the pathogenesis, and to stress the necessity of surgical management. SUMMARY AND BACKGROUND DATA: Pathogenesis of pseudarthrosis in ankylosing spondylitis is discussed. Several factors are involved: trauma, which may be major or minor and undetected; stress fracture; and inflammatory changes. Major trauma was the cause of pseudarthrosis in the first patient, whereas stress fracture and inflammatory changes were the probable causes in patient 2. In patient 1 there were signs and symptoms of cord compression. Patient 2 was referred because of functional disability resulting from kyphosis and because of potential neurologic risk. METHODS: Cervical fusion was performed in both patients. Patient 1 underwent posterior fusion; patient 2 had combined fusion. Patient 1 also underwent a lamineotomy. RESULTS: The course of the disorder after surgery was uneventful in both patients. Neurologic symptoms subsided in patient 1; kyphosis was corrected in patient 2. Both patients resumed their preoperative activities. Follow-up evaluation was done 6 years after surgery in patient 1 and 2 years after surgery in patient 2. CONCLUSIONS: Pseudarthrosis of the cervical spine in ankylosing spondylitis is extremely rare. Presentation of the two patients was different in terms of pathogenesis and signs and symptoms. Surgical treatment is advocated for this disorder.     

Solitary plasmacytoma of the spine associated with neurological complications

We report eight patients with a solitary plasmacytoma of the spine associated with neurological complications. The patients included five men and three women with an average age at presentation of 59 years (range, 47 to 73 years). The tumour was confined to the thoracic spine in six cases, cervical spine in one and lumbar spine in one. Duration of symptoms ranged from 2.5 to 22 months. Treatment consisted of a combination of radiotherapy, melphalan and surgery. One patient progressed to multiple myeloma 7 years after surgery. Surgical treatment (anterior surgery in three cases and posterior surgery in five) produced neurological improvement in all patients. We stress the importance of an early diagnosis followed by appropriate treatment including surgery for this clinical entity and long-term follow-up to detect a disseminated disease.     

Aortic coarctation and complex heart disease. Surgical treatment during the first year of life

During the five years 1990 through 1994, 70 neonates and infants had surgery for coarctation of the aorta. 30 patients with complex coarctation of the aorta underwent a first stage correction with supplementary ductus ligation and pulmonary artery banding as needed. The mode of surgical repair of coarctation was end-to-end anastomosis in ten patients and patch graft in 20 patient. Mean follow-up was 766 (range 3-1812) days. Mortality after primary procedure was 16.6% (5/30) and after secondary procedure 11.1% (2/18). Three surviving patient developed recoarctation, but only one patients needed re-operation. In spite of improvement in neonatal cardiac surgery, we conclude that a staged approach is still the current treatment in most cases of complex coarctation of the aorta.     

First 4-years orthognathic surgery audit of a Saudi teaching hospital

The study represents a retrospective audit of the first four years of orthognathic surgery carried out at a Saudi teaching hospital. The results showed that orthognathic surgery was the most common oral and maxillofacial surgery performed in that particular hospital, which is one of two teaching hospitals covered by the division of oral and maxillofacial surgery, faculty of dentistry, King Saud University in Riyadh, Kingdom of Saudi Arabia. The variation in the patients characteristics, treatment provided, and final outcome is presented.     

Synovial sarcoma of childhood and adolescence. Report of the German CWS-81 study

BACKGROUND: Synovial sarcoma is the third most common pediatric soft tissue tumor. It requires an aggressive approach to achieve a cure. However, optimal treatment modalities adapted to disease extension and histologic variants have not been determined because there is little information about prospectively treated patients. METHODS: A multicenter trial for soft tissue sarcomas (Protocol CWS 81) was conducted in West Germany between 1981-1985, and 31 patients with synovial sarcoma were registered. Treatment included multiagent chemotherapy and irradiation after initial tumor excision or biopsy. The male-female ratio in this group was 1:1.6 with a median age of 14 years (range, 1-19 years). The median follow-up time after diagnosis was 101 months (range, 77-131 months). RESULTS: The overall event-free survival (EFS) for patients with synovial sarcoma was 74.2% at 5 years. Group I-II tumors had a significantly better prognosis than those in Group III-IV (EFS at 5 years 84.4% and 58.3%, respectively; P = 0.024). Small tumors (< 5 cm) responded better than larger tumors (> or = 5 cm; EFS, 93% versus 58%; P = 0.029). Synovial sarcoma involved the extremities in 28 patients who had a better outcome compared with those with extremity rhabdomyosarcoma in this study (EFS for Group I-IV was 82% versus 24%, P = 0.001). CONCLUSIONS: The results appeared superior to previous experience using radical surgery alone and suggested that after initial, nonmutilating surgery, adjuvant chemotherapy, and irradiation contributed to the improved long-term survival.     

Surgical management in atypical pulmonary tuberculosis (MAIS complex)

Forty-four patients with Mycobacterium avium-intracellulare-scrofulaceum (MAIS) complex pulmonary infection who had a surgical procedure for management of their disease are reviewed. Relapse occurred in three cases and two patients died as a late result of their surgery. This gave a satisfactory treatment result in 89% of cases. The follow-up period was from nine months to 14 years after surgery. It is suggested that, despite the advent of modern antituberculosis drugs, resection of lung tissue is still the treatment of choic in suitable cases.     

Nonparasitic splenic cysts. Ultrasonographic features and follow-up

Sixty-three patients with splenic cysts, multiple in 7 cases, were reviewed. Only 3 patients had a history of previous abdominal trauma. The cysts ranged in size from less than 1 cm to 15 cm. They were anechoic in 40 patients, hypoechoic in 16, isoechoic in 4, mixed in one, and in 2 cases the echogenicity could not be assessed due to thick marginal calcifications. The echogenic cysts were larger than the anechoic ones and frequently calcified, and the findings at surgery, fine-needle aspiration biopsy and follow-up suggested the echogenicity to be related to a fresh or previous episode of intracystic hemorrhage. Initially, surgical treatment was undertaken on 10 patients, electively in 9 cases and due to cyst rupture in one. At follow-up (n = 37), the size of the cyst had increased markedly over several years in only 2 patients, necessitating delayed surgery in one. Routine follow-up of asymptomatic splenic cysts was of no clinical value.