Biomedical subjects

A L Bensoussan

Publications and source records attributed to A L Bensoussan.

At least 19 recordsLinked to original sources

PubMed2003

Gastrointestinal duplications.

BACKGROUND/PURPOSE: The aim of this study was to review the presentations of gastrointestinal duplication (GID) and to assess the influence of prenatal diagnosis on treatment. METHODS: Retrospective review of all GID at 2 pediatric hospitals from 1980-2002 was conducted. RESULTS: Seventy-three patients (M43:F30) were identified: 21 neonates, 28 infants (1 to 24 months), 15 children (1 to 10 years), 9 adolescents (>/=11 years). GID location by frequency was ileum (31.5%), ileocaecal valve (30.2%), duodenum (9.6%), stomach (8.2%), jejunum (8.2%), colon (6.8%), and rectum (5.5%). In neonates and infants, vomiting and distension were the most common presentations. Volvulus, caused by a duplication, occurred in 23.8% of neonates and caused the death of one neonate. Intussusception was identified in 10.9% of patients. In older children and adolescents, pain and vomiting were the most common associations. Six of these patients were being treated for Crohn's disease, with the diagnosis of duplication made at laparotomy. Eighteen patients had a prenatal diagnosis by ultrasound scan, with 77.2% of these asymptomatic after birth. Most prenatal diagnoses occurred after 1991 (77.8%). When comparing an earlier period (1980 to 1991; 29 patients) with the current (1992 to 2002; 44 patients), a greater proportion of the latter patients were asymptomatic (36.4 v 13.8%) and had a lower incidence of complications (volvulus/intussusception). CONCLUSIONS: GID can lead to life-threatening complications. Prenatal diagnosis should lead to expeditious postnatal investigation and treatment before the onset of symptoms or complications. GID in older children can mimic Crohn's disease. Laparoscopy/laparotomy should be considered in patients with atypical Crohn's disease or when the diagnosis of an intraabdominal mass is unclear.

Adolescent↗

PubMed2001

Surgical resection and chemotherapy improve survival rate for patients with hepatoblastoma.

BACKGROUND: The authors reviewed retrospectively their experience in 30 children with hepatoblastoma (HB). Despite an increased trend in the incidence of HB during the last 2 decades, an encouraging cure rate has been achieved with complete resection of the tumor and chemotherapy before or after surgery with cisplatin plus doxorubicin (Adriamycin) or cisplatin plus vincristine plus 5-Fluorouracil. RESULTS: There were 10 female and 20 male patients. For the period from 1963 to 1980 there were 8 patients, and for the period from 1981 to 1998 there were 22 patients. Their mean age at surgery was 16 months (range, 3.5 months to 5.5 years). Tumors were localized to the right lobe in 10 (42%), to the left lobe in 7 (29%), and in both lobes in 7 (29%) of the resected patients. Tumors were greater than 10 cm in size in 16 (67%) of these patients. Twenty-four patients (80%), underwent liver resection before or after chemotherapy. One patient (3%) with an unresectable tumor received chemotherapy and a liver transplant. In 5 patients (17%) the hepatic involvement was too extensive for resection. The types of resection performed were right lobectomy in 7, left lobectomy in 6, right trisegmentectomy in 8, left trisegmentectomy in 2, and middle hepatectomy in 1. The overall survival rate for 35 years of the study was 60% (18 of 30). With the association of surgery and chemotherapy (1981 through 1998) survival rate is 82% (14 of 17). Overall median follow-up in our study is 8 years (range, 2.5 to 24 years). CONCLUSIONS: There has been a dramatic improvement in the results of treatment of hepatoblastoma. Formerly, only 25% to 30% of patients were cured, whereas today, with combination of chemotherapy and surgery, 75% to 80% may be cured.

Actuarial Analysis↗

PubMed2000

Repair of the huge umbilical hernia in black children.

Surgical repair of large umbilical hernias may present a challenging surgical problem. The currently described surgical techniques often yield disappointing results. The authors describe a new technique that allows for the repair of the fascial defect and the creation, with the use of a square cutaneous flap, of a neoumbilicus with sufficient depth and a good cosmetic appearance.

Black People↗

PubMed2000

Past and future of biliary atresia.

BACKGROUND: With the advent of liver transplantation the outcome of children with biliary atresia (BA) has improved. Is Kasai hepatic portoenterostomy (KHPE) still a valuable option for the treatment of these patients? METHODS: From 1974 to 1998, 77 patients with biliary atresia have been treated at our institution: 50 girls and 27 boys. RESULTS: Seventy-four patients had a KHPE, and 3 patients had no KHPE because of delay in diagnosis. A total of 65 of 74 patients (88%) had undergone KHPE type I, 4 patients (5.4%) KHPE type II, 3 patients (4%) had a Suruga modification, and 2 patients (2.6%) had a portocholecystostomy. Among the 74 patients, 11 were lost to follow-up and their cases were considered failures. Seventeen of our patients are alive at long-term follow-up after KHPE. Among the 77 patients, 33 (43%) had an orthotopic liver transplantation (OLT). Successful KHPE patients underwent transplant at a mean age of 9 years, and KHPE failed at a mean age of 11 months. A total of 25 of 77 (32%) of patients are alive thanks to OLT. In the cohort, the overall survival rate for the KHPE plus OLT is 42 of 77 (55%). Mortality and morbidity rates were more frequent among the younger patients who had early OLT after KHPE failure. In our series, overall survival rate was improved when the patient had a successful KHPE (P < .001). CONCLUSIONS: Kasai hepatic portoenterostomy (KHPE) continues to be a valuable procedure in the treatment of infants with biliary atresia (BA). Successful KHPE permits transplantation at an age at which mortality and morbidity are decreased leading to a better outcome.

Biliary Atresia↗

PubMed1998

Injection sclerotherapy in the treatment of rectal prolapse in infants and children.

PURPOSE: The aim of this study was to examine the efficacy of injection sclerotherapy as treatment for persistent rectal prolapse. METHODS: The records of 28 patients with rectal prolapse treated with injection sclerotherapy over a 16-year period were reviewed. Initial management included assessment and correction of predisposing factors. If rectal prolapse persisted or if the prolapse required repeat emergency or operating room reduction, injection sclerotherapy was performed. The sclerosing agent included D50W in 21 patients (sole agent in 15, combined with ethanolamine oleate in four, and with phenol 5% in two). Phenol 5% alone was used in six patients, and 25% saline was used in one patient. Number of injections, recurrences, and complications were reviewed. RESULTS: Two patients were lost to follow-up, and one patient was cured once a polyp was recognized and removed. Of the remaining 25 patients, 21 were cured. Sixteen required one injection, three required two injections, and two required three injections (64% cure rate after one injection, 84% cure rate after three injections). There were 4 of 25 failures: two went on to low anterior resection after having failed two injections each; one patient was treated with Thiersch cerclage and injection after two failed injections; and one patient did not respond after three injections but had less severe prolapses. Of those injected with D50W alone, 13 of 14 were cured with injection sclerotherapy. Nine received one injection, two received two injections, and two received three injections (64% cure rate after one injection, 93% cure rate after three injections). The only complication was excessive oozing at the injection site in one patient. He was simply observed in hospital overnight. Follow-up averaged 33 months. The only significant underlying abnormality in our patient population was spina bifida in one patient. This patient was cured with injection therapy. Cystic fibrosis was ruled out by clinical examination and sweat chloride test in all patients. Constipation was the most common condition identified with the onset of rectal prolapse (15 of 28). CONCLUSIONS: Injection sclerotherapy is simple and should be considered as the first line treatment of recurrent rectal prolapse after failure of conservative measures. D50W is effective, easily available, inexpensive, and associated with few complications.

Causality↗

PubMed1996

Anticoagulation without catheter removal in children with catheter-related central vein thrombosis.

Catheter-related central venous thrombosis is a serious and common problem among children. The traditional management has been anticoagulation and early catheter removal. Unfortunately, many patients require a new catheter, which is associated with complications that include possible further thrombosis. Although others have used thrombolytic agents in attempts to avoid catheter removal, the authors of the present study believe that the associated complications occur too frequently and are too serious. They have had success with standard anticoagulation in a limited number of patients. Between February 1991 and April 1994, 17 patients (6 weeks to 19 years of age) were treated for catheter-related deep venous thrombosis. Eight patients underwent early catheter removal accompanied by anticoagulation; two of them had intrinsic catheter problems that necessitated removal, and one had hemophilia. Nine others received anticoagulation without catheter removal. Of these, one required catheter removal after 10 days heparin administration failed to diminish the thrombosis. Another patient responded well to anticoagulation but required catheter removal several weeks later because of catheter-site infection. The other seven patients responded well to anticoagulation, and their catheters were retained. For patients with a functional catheter essential to their care, anticoagulation may safely prevent catheter removal.

Adolescent↗

PubMed1996

Laparoscopic excision of a cystic lymphangioma.

A 16-year-old female was evaluated for recurrent episodes of acute abdominal pain and distension. Initial abdominal radiographs were consistent with recurrent gastric volvulus. Following nasogastric tube decompression, reexamination revealed a mobile abdominal mass in the left upper quadrant. Contrast studies followed by computed tomography demonstrated a cyst measuring 9 X 7 X 6 cm with no obvious relationship to abdominal viscera. Laparoscopy was performed with a 10-mm port at the umbilicus and right upper quadrant,and a 5-mm port in the left lower quadrant. The cyst was excised from the mesentery of the proximal jejunum and placed in a laparoscopic sac. Controlled incision and drainage of the cyst within the sac facilitated its removal from the abdomen with neither enlargement of the trocar site nor intraabdominal spillage. To prevent recurrent gastric volvulus, a gastropexy was performed from the greater and lesser curvatures to the anterior abdominal wall. The histology was typical of a cystic lymphangioma. The patient was discharged on the third postoperative day without complication. Laparoscopic technique allowed the performance of both procedures without large incisions, This is the first reported laparoscopic excision of a cystic lymphangioma.

Adolescent↗

PubMed1995

Vascular complications after pediatric liver transplantation.

From February 1986 to July 1994, 81 hepatic transplantations were performed in 73 children, with an overall patient survival rate of 83%. Forty-two patients received whole-liver grafts (WLG) and 39 had reduced-size grafts (RSG). The mean patient weight was 19.7 kg, with 29 patients weighing less than 10 kg. Seventeen vascular complications (21%) occurred in 13 children: 8 (10%) had hepatic artery thrombosis (HAT), 5 (6%) had portal vein thrombosis (PVT), 1 had both HAT and PVT (1%), and 3 (4%) had aortic conduit perforation (ACP). There was no significant difference in the incidence of HAT between RSG (5%) and WLG (14%) or between children weighing less than 10 kg (10%) and those weighing more than 10 kg (10%). The site of arterial reconstruction, end-to-end to the recipient common hepatic artery or end-to-side to the infrarenal aorta, had no significant effect on the occurrence of HAT (7% v 8%), but HAT occurred in 2 of 6 cases (33%) in which an aortic conduit was used. PVT documented in 5 cases (6%) was associated with technical complications (2), preduodenal portal vein (2), and a circulating cardiolipid antibody (1), and required thrombectomy, with no graft loss. Combined HAT and PVT was found in one patient 2 years postretransplantation for HAT. Although graft function is normal, portal hypertension persists. The aortic conduit, used in six patients, led to arterial perforation (3), HAT (2), and death (2). Of the 8 cases of HAT, 1 was diagnosed during autopsy and 7 occurred within 30 days and required retransplantation (6) or thrombectomy with rearterialization (1).(ABSTRACT TRUNCATED AT 250 WORDS)

Anastomosis, Surgical↗

PubMed1995

Imperforate anus, malrotation and Hirschsprung's disease: a rare association.

The association of imperforate anus with anomalies of rectal innervation is very rare. Moreover, malrotation only rarely accompanies these malformations. We report 2 patients with this unusual triple association and its impact on their management. Retrospectively both patients presented at birth with the same radiological picture of distended distal colon and right-sided small bowel location. We discuss the importance of a systematic search for associated intestinal malformations in cases of imperforate anus in order to avoid serious complications and to choose the optimal operative strategy.

Anastomosis, Surgical↗

PubMed1994

Results and complications of Toupet partial posterior wrap: 10 years' experience.

A 270 degrees posterior wrap, described by Toupet, was performed in 112 patients with medically refractory gastroesophageal reflux (GER) over a 10-year period. The mean age was 39 months (range, 2 months to 19 years). Thirty percent of the patients were neurologically impaired (NI). The approximation of the crura, the posterior fixation of the wrap, and the posterior partial fundoplication were performed with nonabsorbable sutures, over a bougie. Thirty percent of the NI and 15% of the neurologically normal (NN) children underwent a gastrostomy. Early postoperative complications were found in 24% of NI and 18% of NN children; these were treated medically, except for two bowel obstructions. Eight patients died for reasons unrelated to surgery, and nine were lost to follow-up. The mean follow-up period for the 95 remaining patients was 3.5 years for NI and 4.9 years for NN children. The evaluation showed that 6 patients had temporary dysphagia and two had food impaction. Nine wrap herniations (10%) were found; three of them without symptoms were noted by routine upper gastrointestinal series and received no treatment. Six wrap herniations with recurrent reflux were reoperated on successfully. With 90% of patients free of symptoms, the results of the Toupet procedure compare favorably with those of the Nissen fundoplication because of the retained ability to belch or vomit.

Adolescent↗

PubMed1993

Biliary tract complications in pediatric orthotopic liver transplantation.

Biliary tract complications are reported in 15% to 30% of orthotopic liver transplantations (OLTs). Since 1986, 53 OLTs were done in 48 children with a mean age and weight of 5.3 years and 18.9 kg, respectively. Twenty-seven transplantations (51%) were reduced liver grafts (RLG) and 26 (49%) were whole liver grafts (WLG). Since 1988, 70% of transplantations have been RLG. Choledochocholedochostomy (mean weight, 25 kg) with a T-tube (CC) or choledochojejunostomy (CJ) (mean weight, 14.5 kg) were done in 24 (45%) and 29 (55%) cases, respectively. The overall mortality was 19% but none of the deaths were related to biliary problems. There were 13 biliary tract complications (24.5%) in 11 patients including 7 leaks, 5 obstructions, and 1 intrahepatic biloma. Leaks leading to bile peritonitis were managed with simple suture and drainage and were related to the T-tube (4), to the Roux-en-Y loop (2), and to the transection margin of a RLG (1). Obstruction was documented in 5 cases with none associated with hepatic artery thrombosis (HAT). Stenosis after CC reconstruction (2) required conversion to CJ. Two patients had revision of CJ because of kinking of the common bile duct after a left lateral segment graft and an anastomotic stricture 46 months after OLT. The last patient developed a vanishing bile duct syndrome 4 months posttransplant and is awaiting retransplantation. One patient had multiple episodes of cholangitis after HAT and was retransplanted. Neither the type of grafts (RLG 25.9% v WLG 23.1%) nor the type of biliary reconstruction (CC 25% v CJ 24%) influenced the rate of biliary complications.(ABSTRACT TRUNCATED AT 250 WORDS)

Amino Acid Metabolism, Inborn Errors↗

PubMed1993

Surgical and metabolic aspects of liver transplantation for tyrosinemia.

Tyrosinemia is the diagnosis of a very small percentage of patients undergoing liver transplantation worldwide. Tyrosinemia is endemic within our referral area however, and fully one-third of the liver transplantations at our institution are done for this disease. Since 1986, 15 patients with tyrosinemia (TYR) and 31 patients with various other indications (non-TYR) have undergone a total of 51 liver transplantations. The 36-month actuarial survival for TYR patients is 87%, compared with 74% for non-TYR patients. Liver transplantation for hereditary tyrosinemia and other metabolic disorders without portal hypertension or previous portohepatic operations is notably easier to perform. Intraoperative blood loss was less, length of hospital stay was shorter, and incidence of infections was lower in TYR than in non-TYR patients. Less than 10% of TYR patients had foci of hepatocellular carcinoma at the time of transplantation. For this reason, and while most patients with tyrosinemia will eventually require liver transplantation, our results do not support systematic early transplantation before the age of two years.

Adolescent↗

PubMed1993

[Surgical and metabolic aspects of liver transplantation for tyrosinemia].

Tyrosinemia represents a very small percentage of patients undergoing liver transplantation world-wide. This disease is endemic within our referral area however, one-third of the liver transplantations at our institution are done for this disease. Since 1986, 16 patients with tyrosinemia and 34 patients with various other indications (non-tyrosinemic) have undergone a total of 55 liver transplantation. The survival rate for tyrosinemic patients is 87%, compared to 75% for non-tyrosinemic patients. Liver transplantation for hereditary tyrosinemia and other metabolic disorders without portal hypertension or previous portohepatic operations is notably easier to perform. Intraoperative blood loss was less, length of hospital stay was shorter and incidence of infections was lower in tyrosinemic than in non-tyrosinemic patients. Less than 10% of tyrosinemic patients had foci of hepatocellular carcinoma at the time of transplantation. For this reason, and while most patients with tyrosinemia will eventually require liver transplantation, our results do not support systematic early transplantation.

Adolescent↗

PubMed1993

[Biliary complications in pediatric liver transplantation].

Biliary tract complications are reported in 15% to 20% of orthotopic liver transplantations (OLT). Since 1986, 55 OLT were done in 50 children with a mean age and weight of 5.6 years and 18.8 kg respectively. There were 28 (51%) reduced liver grafts (RLG) and 27 (49%) whole liver grafts (WLG). Since starting using RLG in 1988, 70% of transplantations have been RLG. Choledochocholedochostomy with a T-tube (CC) or choledochojejunostomy (CJ) were done in 25 (45%) and 30 (55%) cases, respectively. The overall mortality was 19% with one death related to biliary problems. There were 14 biliary tract complications (25%) in 12 patients including 7 leaks, 6 obstructions and one intrahepatic biloma. Leaks leading to bile peritonitis were managed with simple suture and drainage and were related to the T-tube (4, to the Roux-en-Y loop (2) and the transection margin of a RLG (1). Obstruction was documented in 6 cases, none of which were associated with hepatic artery thrombosis (HAT). Stenosis after CC reconstruction (3) required conversion to CJ. Two patients had revision of CJ because of kinking of the common bile duct and an anastomotic stricture 46 months after OLT. One patient developed a vanishing bile duct syndrome 4 months post-transplant and died while waiting for retransplantation. One patient had multiple episodes of cholangitis after HAT and was retransplanted. The rate of biliary complications was not influenced by neither the type of graft (RLG : 25% vs WLG : 25,9%) nor the type of biliary reconstruction(CC : 28% vs CJ : 23%).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1992

Diaphragm of the gallbladder: a case report.

Gallbladder anomalies are rare and normally affect its shape, size, and position. We report on a 3-year-old boy with a gallbladder divided transversally by a windsock-type diaphragm, isolating the fundus from the rest of the biliary tree. Bilobed and multiseptated gallbladder have been described before, but this is the first isolated case of a congenital hourglass gallbladder.

Child, Preschool↗

PubMed1992

Neonatal gastrointestinal perforations.

Neonatal gastrointestinal perforation has been associated with mortality rates of 40% to 70%. Over the past 20 years, 81 infants (46 boys and 35 girls) were treated for a gastrointestinal perforation at this institution. Perforation occurred from birth to 50 days (average, 8.2 days). Etiologies included necrotizing enterocolitis (NEC) (68%), meconium ileus (10%), and idiopathic gastric perforation (7%). Seventy-six infants underwent surgical exploration and five infants, considered too small or too sick to withstand a laparotomy, were treated with peritoneal lavage only. There were 29 deaths, an overall mortality of 36%. Ninety percent of the death occurred in patients with NEC, while all patients with gastric perforations survived. There has not been a significant improvement in survival in recent years, partly because of an increase in the proportion of NEC-related perforations. However, there is a narrowing of the mortality gap between low birth weight and normal weight infants. As the risk inherent to laparotomy in neonates is decreasing, other factors, such as the underlying etiology or the site of perforation, play a more important prognostic role.

Enterocolitis, Pseudomembranous↗

PubMed1992

[Cysts of the choledochus in children: experience of the Sainte-Justine hospital].

Choledochal cyst remains a rare pathology in western countries. Over the past 15 years, 18 children were diagnosed with choledochal cyst at Hospital Sainte-Justine. There were 14 girls and 4 boys with an average age of 7.4 years ranging from newborn to 15 years. Abdominal pain, jaundice and abdominal mass were present in 70%, 54% and 7% respectively. Only one patient, a 15 years old girl had all three symptoms. Abdominal ultrasonography was diagnostic in all cases and in two cases, a cystic dilatation was found in the antenatal ultrasonography. Transvesicular cholangiography was performed successfully in 11 patients and showed a detailed anatomy of the biliary tract preoperatively. Sixty-seven percent of the cysts were type I while 33% were type IV. A common channel was found in 43% of the cases. Sixteen patients underwent cyst excision followed by Roux en Y hepatico-jejunostomy, one patient with associated biliary atresia had a porto-enterostomy and one patient had a cysto-duodenostomy. The last patient had recurrent episodes of cholangitis and required reoperation while the others are doing well and free of infection. With the liberal use of ultrasonography, the diagnosis of choledochal cyst is being done earlier improving the long term prognosis. Transvesicular cholangiography is a very useful addition in the preoperative investigation of these patients. Roux en Y hepatico-jejunostomy was associated with minimal morbidity and mortality and gave excellent result.

Adolescent↗

PubMed1991

Gracilis muscle transposition for anal incontinence in children: long-term follow-up.

We report a series of 7 patients, aged 6.5 to 19 years (mean, 12.9), who have been treated for uncontrollable fecal incontinence since 1976: 5 had imperforate anus and multiple subsequent operative procedures, 1 had a low myelomeningocele with bi-sphincteric incontinence, and 1 had a traumatic destruction of the sphincter apparatus. A modified Pickrell procedure was performed, with the gracilis muscle transposed subcutaneously, without constructing a pulley through the median raphe as originally described. All patients were evaluated by anorectal manometry preoperatively and post-operatively. They were followed-up for a period of 0.5 to 12.5 years (mean, 4.4). All patients were continent at follow-up, with a normal defecation pattern and no enemas required. None of the patients had evidence of fibrosis of the muscle or anal canal, and tension in the transposed muscle was maintained. Voluntary contractions remain efficient in all cases. Age was thought to be an important factor: personal motivation and compliance with physiotherapy, essential for a good outcome, is unlikely to be present in the younger child. We conclude that the gracilis sling procedure is an excellent long-term alternative for total fecal incontinence when time and other therapeutic measures have failed.

Adolescent↗