Bifid scrotum, perineal hamartoma and high imperforate anus: a case report

This is a case report of a newborn patient with imperforate anus, urethro-colonic fistula, perianal hamartoma, and bifid scrotum. Successful staged repair of these anomalies is described together with review of the embriology related to the case.     

Tethered cord and associated vertebral anomalies in children and infants with imperforate anus: evaluation with MR imaging and plain radiography

PURPOSE: To evaluate in children and infants with imperforate anus the prevalence and types of occult myelodysplasia that may result in tethered cord and the association of these lesions with vertebral anomalies. MATERIALS AND METHODS: Records and images were retrospectively reviewed in 86 patients who underwent spine magnetic resonance imaging and had either low-level imperforate anus (n = 30), intermediate-level imperforate anus (n = 15), or high-level imperforate anus (n = 41). RESULTS: Thirty-one of 86 patients (36%) had occult myelodysplasia suggestive of tethered cord (27% of all patients with low-, 33% of all patients with intermediate-, and 44% of all patients with high-level lesions). Of these 31 patients, 16(52%) were asymptomatic, 24, (77%) had a thickened fatty filum, 13 (42%) had normal conus position, and 23 (74%) had vertebral anomalies. Twenty-four of the 31 patients (77%) underwent surgical untethering. CONCLUSION: A substantial number of patients with all types of imperforate anus have occult myelodysplasia that may necessitate surgical intervention, including those patients with normal spine radiographs.     

Surgical treatment of ischemic heart disease complicated with severe left ventricular dysfunction: experience in 200 cases

BACKGROUND: The study was designed to evaluate short and long-term benefits of coronary artery bypass graft in patients with coronary artery disease and severely depressed left ventricular ejection fraction and to identify contemporary risk factors associated with significantly greater mortality in this high-risk subgroup. METHODS: From 1985 to 1995, 200 consecutive pts with EF < or = 0.30 underwent CABG. Among these patients, 60% were older than 70 years. NYHA functional class III/IV was present in 31% of pts. Preoperative mean cardiac index was 2.7 +/- 7 l/min/m2, mean pulmonary artery pressure was 29.9 +/- 7 mm Hg and contractility score (generated by appropriate software for left ventricular kinesis analysis) mean value was 50.1 +/- 11.6 points. Urgent operation was required in 32 pts (16%). The majority of pts were completely revascularized. RESULTS: Operative mortality was 9% (18 pts). Low output syndrome was the most common postoperative complication (13.5%) followed by ventricular arrhythmia (8%), mean length of postoperative hospitalization for survivors was 13 +/- 10 days. Of 23 possible operative risk factors evaluated, four were associated with significantly greater mortality: cardiac index < or = 2.1 l/min/m2, urgent operation, contractility score > or = 80 and associated surgical procedures. Survivors experienced significant improvement in CHF class (p < 0.001) and follow up EF (p < 0.001). Kaplan-Meier estimate of survival at 1 year, 5 years and 8 years was 85%, 65% and 54%. CONCLUSION: Through more careful assessment of preoperative risk factors, patients selection and perioperative management, actually coronary artery bypass graft may be offered to pts with low ejection fraction with reduced morbidity and mortality.     

Sturge-Weber syndrome: experience with 14 cases

We report a first documented case in Senegal with simple transposition of the great arteries diagnosed in a 2 months old girl treated by Rashkind atrioseptostomy. Our patient benefited from clinical examination, ECG (15 derivations), chest X ray and standard laboratory tests. Pulsed-Doppler, two dimensional and TM echocardiography have been performed with an ATL MK 600 echocardiograph. Cardiac catheterism, angiocardiography and Rashkind procedure have been realized in our Department. These data are discussed and compared to the literature. At admission this patient presents with major cyanosis and polypnea. At examination, there is a 3/6 murmur at the left sternal border and a subclavicular continuous murmur. Laboratory tests showed metabolic acidosis and severe hypoxemia. Chest x-ray showed a cardio-thoracic ratio at 0.64 with increased pulmonary vascular markings. ECG showed right ventricular hypertrophy. Echocardiography-Doppler revealed ventriculo-arterial discordance with restrictive atrial septal defect and persistent ductus arteriosus. Rashkind procedure was followed by an increased aortic saturation. After 6 weeks there was an improvement of cyanosis and cardiac failure. Diagnosis of transposition of the great arteries is actually easier with development of ultrasonography which is useful when performed by experienced cardiologist. Spontaneous prognosis of this malformation is very poor. Rashkind atrioseptostomy is an important step for the initial treatment of transposition of the great arteries in terms of survival before open heart surgery.     

Treatment of severe forms of sickle cell anemia with bone marrow allograft: French experience (15 cases). SFGM

OBJECTIVE: To determine whether home uterine activity monitoring reduces the likelihood of preterm birth in women successfully treated for preterm labor in their current pregnancies. METHODS: Women between 20-34 weeks' gestation who had been treated successfully for preterm labor were solicited to participate in a randomized clinical trial of home uterine activity monitoring versus routine high-risk care. The sample size of 56 was based on power calculations using the results of earlier investigators. Twenty-eight women were randomized to home uterine activity monitoring and 29 were assigned to the type of care appropriate for women discharged after hospitalization for parenteral treatment of preterm labor. One of the routine-care subjects was lost to follow-up. The two groups were comparable in distribution for race, insurance status, multiple gestation, marital status, gestational age at beginning of the study, and incidence of prior preterm birth. RESULTS: The 28 women receiving routine care had a 54% incidence of preterm birth, whereas the incidence was 57% in monitored women (relative risk 1.08, 95% confidence interval 0.6-1.9; P = .79). The incidences of delivery before 32 weeks and 34 weeks also were unaffected by the intervention. CONCLUSION: Home uterine activity monitoring is not effective in reducing the likelihood of preterm delivery in patients successfully treated for preterm labor in their current pregnancies.     

Penetrating stab wounds of the chest: experience with 200 consecutive cases

The incidence of penetrating wounds of the chest is rising rapidly. Opinions continue to differ on their management. Our experience with 200 consecutive cases of stab wounds of the chest between 1972 and 1975 were reviewed. There were 176 males and 24 females. The average age was 31 years; about two-thirds of the patients were under 30. About 74% presented with hemothorax or hemopneumothorax; 21 presented with pneumothorax. Eleven per cent had associated intra-abdominal injuries. Seventy-nine per cent were successfully treated with tube thoracostomy. About 15% underwent thoracotomy, with three deaths (mortality, 10%); the mortality for cardiac wounds was 16%; overall mortality was 1.5%. The overall complication rate was 5.5%, most occurring in patients with cardiac wounds and associated intra-abdominal injuries. The average period of hospitalization was 6.5 days. Treatment was individualized. Indications for each course of therapy are discussed.     

Gastric cancer with obstructive uropathy: clinical experience with 17 cases

In Tunisia a growing extension of chronic diseases has resulted from the environmental and behavioral changes such as the adoption of new dietary habits, sedentarity habitat, stress of urbanization and work conditions. To illustrate this trend, we undertook a household epidemiologic survey of a representative sample of the adult urban population of Soussa (n = 957) to assess the main cardiovascular risk factors except the lipid profile distribution. This study showed a high prevalence of hypertension (> 160/95 mm Hg): 18.8% with an adjusted rate of 15.6%. History of diabetes 10.2%, obesity (BMI > 30) 27.7% significantly higher in women (34.4%), android obesity 36%, smoking habits 21.5% significantly higher in men (61.4%). With this risk factor profile, Tunisia has to implement a national strategy of primary prevention and heart health promotion in addition to the efforts recently made in secondary prevention of some chronic diseases such as hypertension and diabetes.     

Anteriorly located anus: is constipation caused by abnormal location of the anus?

Anteriorly located anus (ALA) is frequently associated with severe constipation accompanied by defecation pain. Between 1988 and 1994, the authors treated 27 children (26 girls, 1 boy; age range, 0 to 11 years) to surgically correct ALA. The operation was performed according to a uniform protocol to longitudinally divide the internal sphincter muscle from the anal skin level to 2 cm above the dentate line on the posterior wall of the anorectum. For anal reconstruction, any of the conventional procedures was employed. Twenty-two of the 27 patients have had follow-up in our clinic for 12 months to 6 years (mean, 2.75 years). Eighteen are completely free of constipation and defecation pain and have regular spontaneous bowel movements. The other four require occasional use of enemas or laxatives. Anal incontinence did not occur in any patient. The results of this study suggest that abnormal function of the internal sphincter is the most likely cause of constipation or defecation pain in patients with ALA and that internal sphincterotomy is the cornerstone of surgical treatment.     

Carbohydrate antigen 195 (CA 195) in the sera of patients with esophageal, hepatic and biliary tract carcinomas

In this work the problems of obtaining medicinal forms of lactobacterin with the use of nontraditional methods for the stabilization of biomass are considered. The data on the stability of tablets, suppositories and microgranules of the preparation prepared on the basis of immobilized cells, are presented.     

Endoscopic treatment of postoperative bronchopleural fistula: experience with 45 cases

Transforming growth factor-beta (TGF-beta) is synthesised as an inactive precursor protein; this is cleaved to produce the mature peptide and a latency associated protein (LAP), which remains associated with the mature peptide until activation by LAP degradation. Isoform specific antibodies raised against the LAPs for TGF-beta 2 and -beta 3 were used to determine the myocardial levels of LAP (activatable TGF-beta) and full length precursor (inactive TGF-beta) forms during post-natal development in the rat. TGF-beta 2 was present predominantly as the precursor in 2 day old myocardium. There was an age-dependent shift from precursor protein to LAP between 2 and 28 days. A corresponding increase in the level of mature (activatable) TGF-beta 2 was found. TGF-beta 3 was detected in significant quantities only as LAP. However, a four-fold increase in the expression of TGF-beta 3 LAP was observed between 2 and 28 days. The substantial increases in activatable forms of TGF-beta 2 and -beta 3 that occur in myocardium during the first 28 days of life in the rat support a role for these proteins in post-natal cardiac development.     

Prognostic factors in agnogenic myeloid metaplasia: a report on 195 cases with a new scoring system

The present study evaluated end-stage renal disease (ESRD) patient survival in Lombardy, Italy, and the United States (U.S.) using data from two registries, the Lombardy Dialysis and Transplant Registry (RLDT) and the U.S. Renal Data System (USRDS), respectively. For this purpose, 4,196 white patients (2,900 from the USRDS Case Mix Severity Study and all 1296 from RLDT) who started renal replacement therapy in 1986 and 1987 were studied. Compared to Lombardy patients, those in the USA were significantly older (mean age 59.9 +/- 16.4 vs. 55.9 +/- 14.7 years), had a lower proportion of males (53.7 vs. 62.1%), a greater proportion with diabetic nephropathy (29.9 vs. 9.7%) and a significantly greater proportion of patients with the recorded comorbid conditions (heart disease, peripheral vascular disease, cirrhosis, cachexia, malignancy). U.S. patients were less frequently treated with peritoneal dialysis (PD) by day 30 of ESRD (21.2 vs. 30.7). Survival was compared in the Cox proportional hazard regression model, using age, sex, comorbid conditions and early modality of treatment as explanatory covariates. Overall, 48% of the 4196 patients died during the 48 to 72 months follow-up to 12/31/91. Per 100 patient-years the gross death rate for USRDS patients was 28.7 compared to 13.0 of RLDT patients. The unadjusted death relative risk for RLDT was 0.439, that is, 56% lower death rate compared to USRDS patients. Age, sex, diabetic status, each of the recorded comorbid conditions and treatment modality were significantly related to survival and included in the model. The Cox cumulative survival adjusted for all these explanatory covariates survival was for U.S. patients 84.4% at one year, 67.0% at two years and 33.4% at five years, and for RLDT patients 88.3% at one year, 75.9% at two years and 45.9% at five years. The relative mortality risk (RR) for the patients treated in Lombardy adjusted for all the reported covariates was 29% lower than for US patients (RR = 0.71; P < 0.0001). This comparative risk varied significantly by age (P < 0.0001) and was 65 percent lower for Lombardy compared to U.S. patients in the age range 25 to 44 years (RR = 0.35) and about 20% lower for patients over age 65 years (RR = 0.80). This relative risk was mainly related to hemodialysis and was not statistically significant for PD patients. The observed lower mortality risk in Lombardy was less pronounced when adjusted for demographic and comorbid covariates, but was still large and therefore suggests the need for further studies regarding treatment related factors and unmeasured patient factors, particularly in hemodialysis patients.     

Pancreatic-biliary ascariasis: experience of 300 cases

BACKGROUND: Infestation with Ascaris lumbricoides is seen worldwide. Recently, there has been much interest in the pancreatic-biliary complications of Ascaris infection. In this study, we present our experience of 300 patients seen in a tertiary referral center. MATERIALS AND METHODS: Case charts of patients seen in the Department of Gastroenterology, University of Damascus, Syria, were analyzed, retrospectively, over a 5-yr period (September of 1988 to August of 1993). During this period, 1666 endoscopic retrograde cholangiopancreatographic studies were performed and pancreatic-biliary ascariasis was diagnosed in 300 patients (18%). RESULTS: The most common presenting symptom was abdominal pain, seen in 98% of patients (294 patients). Complications observed were ascending cholangitis (48 patients; 16%), acute pancreatitis (13 patients; 4.3%), and obstructive jaundice (4 patients; 1.3%). History of worm emesis was present in 25% of patients (76 patients). Most patients (240 patients; 80%) had previously undergone a cholecystectomy or an endoscopic sphincterotomy (232 patients; 77%). Worms were successfully extracted endoscopically in all except two patients, and there were no procedure-related complications. CONCLUSIONS: In endemic countries, ascariasis should be suspected in patients with pancreatic-biliary disease, especially if a cholecystectomy or sphincterotomy has been performed in the past. Endoscopic management results in rapid resolution of symptoms and prevents development of complications.     

Hypernephroma in the solitary kidney: experience with 20 cases and review of the literature

Twenty patients with renal cell carcinoma in anatomically or functionally solitary kidneys were treated and followed for up to 18 years. Factors pertinent to management and survival of these patients and 66 other well documented similar patients reported in the literature are analyzed. Most of the patients were unusually young and a significant number had had nephrectomy for contralateral renal cell carcinoma. Survival was closely related to the earlier presence of malignant disease in the other kidney, the duration of the interval between detection of the 2 neoplasms and the stage of the lesion in the solitary kidney. Partial nephrectomy has been the most successful treatment. The mean survival in the Mayo Clinic series has been 6 years for patients still alive and 2.4 years for those dead at the time of this analysis. Results in this and other series emphasize the importance of thorough long-term followup after nephrectomy for hypernephroma and of aggressive therapy when the remaining kidney becomes involved. It is foolhardy to abandon hope merely because there is a malignant tumor in a solitary kidney.     

Posterior interosseous reverse forearm flap: experience with 80 consecutive cases

The results of an anatomic investigation performed in 40 fresh cadaver specimens and 80 consecutive clinical cases of the posterior interosseous reverse forearm flap are reported. It was observed that there is a choke anastomosis between the recurrent dorsal branch of the anterior interosseous artery and the posterior interosseous artery at the level of the middle third of the posterior forearm. Ink injections through a catheter placed in the distal part of the anterior interosseous artery stained the distal and middle thirds of the posterior forearm, but the proximal third remained unstained; this secondary territory cannot be captured through the choke anastomosis between the anterior interosseous artery and the posterior interosseous artery. Intravital fluorescein injection into the distal arterior interosseous artery revealed (under ultraviolet light) that the distal third of the posterior forearm is irrigated by direct flow through the recurrent branch of the arterior interosseous artery (the traditionally called distal anastomosis of the interosseous arteries). Therefore, we can assume that the blood flow is not reversed when the so-called posterior interosseous reverse forearm flap is raised. From this point of view, this flap could be renamed as the recurrent dorsal anterior interosseous direct flap; however, the classical name is maintained for practical purposes. From the venous standpoint, the cutaneous area included in this flap belongs to an oscillating type of venous territory and is connected to the deep system through an interconnecting venous perforator that accompanies a medial cutaneous arterial branch located at 1 to 2 cm distal to the middle point of the forearm.(ABSTRACT TRUNCATED AT 250 WORDS)