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Biomedical subjects

P Vergnes

Publications and source records attributed to P Vergnes.

At least 37 records · Page 2Linked to original sources

Oesophageal atresia, VACTERL association: Fanconi's anaemia related spectrum of anomalies.

Oesophageal atresia usually occurs without any genetic background. Three cases associated with Fanconi's anaemia are reported. One neonate had growth retardation and numerous malformations including oesophageal atresia and four other components of the VACTERL association. In the two others, oesophageal atresia was isolated. In patients with such malformations an early diagnosis of Fanconi's anaemia may have important genetic and therapeutic implications.

Esophageal Atresia↗

[Severe hemangiomas treated with interferon alpha-2b: seven cases].

INTRODUCTION: High-dose corticosteroids are the primary means of controlling alarming hemangiomas. However, only 2/3 of these life-threatening hemangiomas regress or are stabilized with corticosteroids: and for others there is no regularly safe and effective treatment. Contradictory publications have been reported about the efficacy of interferon (INF) alpha in hemangiomas, we report our experience concerning the management of 7 cases. PATIENTS AND METHODS: Seven infants have been treated with INF alpha-2b in our Pediatric Dermatology Unit. All hemangiomas were corticosteroid resistant. Hemangiomas were located on head and neck in 6 cases and on lower limb in one case in association with platelet trapping syndrome. Mean age at beginning of treatment was 7.5 months and mean duration of treatment was 5 months. INF alpha-2b was given daily in subcutaneous injections of 3 millions units per square meter of body surface area. RESULTS: After one month of treatment, 2 cases of facial hemangiomas showed a dramatic improvement, and INF alpha-2b was maintained for one year. One case was stabilized, but INF was ineffective in 3 cases after 2 months of treatment (the child with platelet trapping syndrome died at 4 months of age). The treatment had to be stopped in one case because of hepatitis. No long term side effects were noted with a mean follow-up of 27 months. CONCLUSION: INF alpha can be an interesting alternative therapy for infantile hemangiomas. However, only a minority of patients can be considered as excellent responders at current dosages and long term secondary effects cannot be excluded.

Anti-Inflammatory Agents↗

[Severity of ingestion of caustic substance in children].

BACKGROUND: Caustic ingestion is frequent in children, sometimes leading to esophageal stricture. PATIENTS AND METHODS: Between 1988 and 1994, esogastroscopy was performed in 65 children after caustic ingestion. The children were classified in three groups: no lesion (group A), minimal lesions (group B) and severe lesions (group C). Nature of the caustic substance, clinical signs and evolution were compared in the three groups. RESULTS: Median age was 2 years for the 65 children (24 girls, 41 boys). Ingestion occurred at home (94%) during meal periods. Substances were dishwater detergents (n = 14), oven cleaner (n = 10), bleach (n = 9), washing powder (n = 4), others (n = 20), more often in a liquid form (n = 37) than solid (n = 28). Children had no symptoms (57%), presented emesis (n = 20) or abdominal pain (n = 10) not correlated to endoscopic findings, and hematemesis (n = 3) or respiratory distress (n = 4), both symptoms seen only in group C. Buccal lesions (41%) were not correlated to endoscopic findings. After endoscopy, 28 children (43%) were classified into group A and 20 children (31%) in group B. Among the 17 children (26%) of the group C, eight developed an esophageal stricture: seven long strictures requiring replacement of the esophagus, one short stricture requiring repeated dilations. CONCLUSION: Esophageal stricture is still a severe complication after caustic ingestion. These data stress the interest of controlled studies to confirm the preventive role of high dose corticosteroids, and the importance of the prevention of accidental caustic ingestions in children.

Accidents, Home↗

[Increasing frequency and diagnostic difficulties in intestinal stenosis after necrotizing enterocolitis].

BACKGROUND: Stenosis after necrotizing enterocolitis (NEC) has increased from 15 to 57% over the last 10 years in our unit. The aim of this study is to point out the difficulty of diagnosis and treatment, and search for factors explaining this increase. PATIENTS AND METHODS: From 1986 to 1991, 42 newborns had NEC, followed by intestinal strictures in 19 of them (57% of the 33 survivors). Data from these 19 patients were compared with those of the 14 without intestinal strictures. The 33 survivors were also compared with those of an earlier study including 25 NEC seen from 1979 to 1986. RESULTS: After medical treatment (n = 12), intestinal stenosis led to occlusion after three weeks, was located to both small and large intestine and was short and tight. After surgical treatment (n = 7), stenosis was shown by opacification before digestive anastomosis (n = 5) or revealed by occlusion (n = 2); it stayed on the colon, was long or multiple, requiring extensive resections. No difference could be found between data from patients with or without stenosis. Although newborns were actually more premature, the risk of stenosis was more frequent when newborns of same gestational ages and/or weights were compared. CONCLUSIONS: Intestinal stenosis is a frequent complication after NEC; its diagnosis is often difficult and requires extensive digestive resections. No clinical or therapeutic factor could be found to explain the actual increase in frequency.

Birth Weight↗

[Acute torsion of an accessory spleen].

A case of acute torsion of an accessory spleen is reported. In a 15-year-old girl was admitted to hospital with acute abdominal pain. Ultrasonography showed a round, hypoechoic, solid mass. Computed tomography demonstrated a low-density mass with peripheral enhancement after intravenous contrast medium. These findings suggest the presence of a capsule. At laparotomy, the patient was found to have torsion and infarction of an accessory spleen. Sonographic and computed tomographic findings are discussed.

Acute Disease↗

Evaluation of a hydrocolloid dressing.

A hydrocolloid dressing was compared to adhesive skin tapes on children's postoperative wounds. A total of 170 children of varying ages were randomised in two parallel groups, in nine centres of plastic, thoracic, gastrointestinal, urogenital and orthopaedic surgery. Skin closure was satisfactory in both groups, with 76 (89.4%) healthy closures without dehiscence in the hydrocolloid group and 81 (95.3%) in the control group; a relationship was found between partial closures/dehiscence and the type of surgical procedure. No product-related maceration, infection or adverse event was reported during the study and both groups showed very satisfactory cosmetic results.

Adhesives↗

[Lymphomatoid papulosis in a child].

BACKGROUND: Lymphomatoid papulosis (LyP), uncommon in children, has a benign clinical course in contrast with a malignant histology. CASE REPORT: A 9-year old boy developed nodular skin lesions for 6 months on the scalp and penis and a papule skin on his trunk. Surgical excision of scalp nodules was performed to prevent necrosis and ulceration of the ear. The biopsy showed a dense mixed cellular infiltrate including histiocytes, lymphocytes and numerous atypical large cells of which a minority expressed the CD30 antigen. PCR showed an oligoclonal rearrangement of the TCR gamma chain gene. Physical examination, bone marrow aspirate and thoracoabdominal CT scans were normal. Three years after surgery, no other lesions occurred. DISCUSSION: Differential diagnosis of LyP is difficult with non Hodgkin's lymphoma, especially CD30+ lymphoma. Prognosis remains the major problem in LyP. Ten to 20% of cases are associated with lymphomas in adults.

Child↗

Extra-lobar pulmonary sequestration with prenatal diagnosis. A report of 5 cases and review of the literature.

UNLABELLED: THE PURPOSE of this work was to study the pre- and postnatal features of extra-lobar pulmonary sequestration (ELPS) and consider their diagnosis and treatment. METHODS: Five ELPS diagnosed prenatally (1986-1992) were reviewed retrospectively. RESULTS: In 2 cases, prenatal diagnosis was based on the presence of a left suprarenal mass for which tumor markers proved negative postnatally. These 2 infants underwent surgery at 3 weeks of age for supposed neuroblastoma or teratoma. In the other 3 cases, diagnosis was based on the presence of a solid mass at the left base of the thorax. The systemic vessel was visualized in 2 of these cases; mediastinal displacement was noted in one case and hydrothorax (which recurred after puncture) in the other. The latter infant was born at 34 weeks of amenorrhea, and hydrothorax disappeared postnatally after excision of the ELPS. The other two infants were asymptomatic at birth and underwent surgery respectively on the 8th day and during the 6th month of life. CONCLUSION: ELPS can take the form of a mass in the abdomen or at the base of the thorax. For subdiaphragmatic ELPS, surgical excision (possibly preceded by percutaneous puncture) is required if the diagnosis is uncertain. Supradiaphragmatic ELPS can be complicated prenatally by hydrothorax or even hydrops, requiring drainage in utero. If the infant is asymptomatic postnatally, systematic surgical excision should be considered. Extra-lobar pulmonary sequestrations (ELPS) are masses of nonfunctional lung tissue vascularized by an abnormal systemic artery and covered with a pleural layer isolating them from the rest of the parenchyma. The diagnosis of these malformations is based increasingly on obstetrical ultrasonography. The purpose of this study was to specify the prenatal features of these malformations and define the diagnostic approach and therapeutic strategy.

Bronchopulmonary Sequestration↗

Repeated skin expansion for excision of congenital giant nevi in infancy and childhood.

Three children with giant pigmented nevi were treated by repeated tissue expansion. Five flaps were expanded twice, and one flap was expanded three times. The delay between expansions was 4 to 15 months (mean 9 months). With full inflation, vascularization of the flap remained good. However, every new expansion decreased flap length by 50 percent. Overall results with this technique, especially in two infants with giant pigmented nevi involving respectively 20 and 40 percent of the body surface, have been most impressive. Only one major complication has occurred: erosion of the skin caused by a fold of the expander envelope resulting in exposure of the expander. This technique improves the early surgical management of giant pigmented nevi and may provide better long-term functional and cosmetic results.

Abdominal Neoplasms↗

[Apple-peel jejunal atresia. Nutritional management].

Apple-peel jejunal atresia is a rare digestive tract congenital defect often accompanied with a short gut. Two cases managed by a three-stage medical and surgical approach are reported. A protein hydrolysate-jejunal secretion mixture was drip-fed through the distal ileostomy to stimulate trophicity of the distal gut.

Enteral Nutrition↗

[Duckett's+ simple surface or double surface? Comparison of 2 urethroplasty procedures. Report of 91 hypospadias].

The results and complications of the treatment of hypospadias were studied in relation to the two pedicle foreskin mucosal flap techniques described successively by Duckett, "single surface" and "double surface". 91 urethroplasties were performed: 58 "single surface" and 33 "double surface" Duckett urethroplasties. The children were operated between the ages of 18 months and 16 years with a mean age of 4 years. 75% of children had a good result on long-term follow-up. In the case of major complications, essentially fistula, distal necrosis àf the urethra or stricture, a single reoperation increased the percentage of good results to almost 98% for the entire series. However, when the two procedures were compared, a good result was obtained immediately in 81% of cases for the "single surface" operation versus 66% for the "double surface" operation. The fistula rate was 27.2% with the "double surface" flap versus 13.7% with the "single surface" flap and the distal stricture rate was 15.1% with the "double surface" flap versus 3.4% with the "single surface" flap. In the light of these results, Duckett's "double surface" procedure has been abandoned in favour of the "single surface" flap.

Adolescent↗

[Accessory thymus in cervical ectopy: apropos of a case].

Cervical tumors of thymic origin are considered to be uncommon lesions in the differential diagnosis of neck masses. They can be either cystic or solid. The authors present one case of solid cervical thymic tumor in a 2 month-old baby. The embryogenesis of the thymus explains the cervical location of these tumors. Theories of physiopathology are presented. The clinical presentation is variable and their nature is often recognized only upon surgery and preoperative pathologic examination. Some cases of thymoma and respiratory complications resulting from ectopic thymus have been described in the literature, so that total excision of the mass must be performed. This lesion may be more common than suggested in the literature.

Choristoma↗

[Multicystic renal dysplasia with ectopic implantation of the ureter in boys. Apropos of 6 cases].

The prevalence of multicystic dysplastic kidney has increased since the development of antenatal ultrasound diagnosis. Even though, the rarity of symptomatic forms and case reports of spontaneous regressions antenatally or after postnatal ultrasound follow-up have reduced indications of nephrectomy which is less and less appropriate. However, discovery of ectopic ureteric implantation in boys makes certain additional investigations as intravenous pyelography, cystography and ultrasound, essential before making any therapeutic decision. Nevertheless, some cases remain undescriptible. In these cases, operative opacification of any patent ureter can provide more precise information about ureteric implantation. Lastly, only a dissection and resection as complete as possible of any patent ureter and pelvic cystic dilatation can prevent subsequent complications.

Child, Preschool↗

Proteus syndrome in 7 patients: clinical and genetic considerations.

The Proteus syndrome is a congenital hamartomatous disorder delineated in 1983. Because of its polymorphic appearance, the syndrome was named after the greek god Proteus whose name means much less than the polymorphous much greater than. Major clinical findings include hemi hypertrophy, macrodactyly, exostoses, scoliosis, epidermal nevi, haemangiomas, deeply rugated soles of the feet and a variety of deep and subcutaneous masses. We report on 7 new cases of Proteus syndrome. All reported cases have been sporadic. Therefore this syndrome could be due to the action of a dominant lethal gene surviving by mosaicism.

Adult↗

[Sacrococcygeal teratomas in antenatal diagnosis].

The authors report eight cases of antenatal diagnosis of sacro-coccygeal teratoma (SCT) in five girls and three boys in whom the diagnosis was made between the 19th and 34th week of amenorrhea (mean = 27 weeks). The ultrasound pictures taken antenatally of the SCT assist in the discovery of a mass that is usually heterogenous, attached to the distal end of the sacrum, and the discovery is usually made fortuitously or because the height of the uterus is too great. A different series of antenatal diagnoses for SCT have made it possible to work out certain criteria of seriousness to be able to predict intra-uterine death: the presence of anasarca or of hydramnios, the discovery of the lesion before the 30th week of amenorrhoea, the relative weight of the teratoma as against the weight of the fetus being above 50%. We think from our experience that it is important to add the scale of the antenatal growth of the teratoma. A rapid growth of the SCT will lead to a tumour mass which is great as compared to the size of the fetus. Similarly in certain cases the vascular bed will increase in size, and intratumour haemorrhages can occur and give rise to fetal heart failure and also to fetal anaemia, hypoproteinaemia and the appearance of anasarca or of hydramnios. The child dies in utero or immediately after birth because of prematurity from the haemorrhagic state or from cardiac insufficiency. Furthermore accelerated growth of the tumour is nearly always in the immature tumour cells and that means that the child, if it is born alive, should be followed up for a long time because there is a risk of it becoming locally malignant. In practice the monitoring of SCT and the antenatal discovery of the condition should be carried out very seriously in order, in some cases, if it is viable to produce a living child in conditions where the rapid growth of tumour would make it likely that the child would die in utero.

Evaluation Studies as Topic↗