[Inclusion body fibromatosis: a case report]. / Fibromatose digitale infantile : à propos d'un cas.

Infantile digital fibromatosis or inclusion body fibromatosis is a rare, benign fibroproliferative lesion with recurrent potential that occurs on the digits of infants. A highly characteristic morphologic finding is the presence of paranuclear inclusion within the tumoral cells. We report here a case occurring in an 8-month-old infant with 2 asynchronous lesions of the toes.

Complications occurring during conservative management of splenic trauma in children.

AIM: Conservative management of splenic rupture in haemodynamically stable children is now generally accepted. However, during follow-up, many complications can occur. The aim of this study was to describe the complications we observed and to propose a standardised follow-up adapted to them. METHODS: Between March 1992 and December 2002 we managed 65 children (aged between 3 and 15 years old) with accidental splenic rupture. Follow-up and treatment consisted of a 10-day bed rest with sonogram and Doppler controls on the 5th and 10th day and subsequently every month until complete healing. Complications included secondary haemorrhage in 3 cases, cystic evolution in 5 cases, and pseudo-aneurysm in 2 cases. All were treated by renewed bed rest. Three of the cysts resolved spontaneously, the other two underwent cystic resection and epiploplasty by laparotomy (n = 1) or laparoscopy (n = 1) and both pseudo-aneurysms were selectively embolised. There were no splenectomies. CONCLUSIONS: Complications essentially occurred in older children and were not related to gender, type of fracture, or extent of bleeding. Cystic evolution of the sub-capsular haematomas can appear up to 1 month after trauma. Peripheral pseudo-aneurysms which could be responsible for secondary haemorrhages were selectively embolised. We favour the use of the Doppler sonogram for follow-up until total recovery of these patients, even in low-grade traumas. Considering the number of complications encountered we do not believe the American evidence-based guidelines are suitable for our population.

[Spontaneous rupture of a choledochal cyst in a 11-month-old girl]. / Rupture spontanée d'un kyste du cholédoque chez une enfant de 11 mois.

Choledochal cysts are rare congenital malformations of the biliary tract. Though most cysts are diagnosed incidentally, some present directly with complications. We report on the case of an 11-month-old girl admitted for abdominal pain, fever and vomiting. Ultrasonography revealed intraabdominal fluid and the absence of a choledochal cyst diagnosed 2 months earlier. Laparotomy for suspected rupture of a choledochal cyst was planned and a choledochojejunostomy with Roux-en-Y was performed. Spontaneous rupture of a choledochal cyst is rare and occurs most frequently in children under the age of 4. The exact cause is yet unknown and several factors have been implicated. The most probable cause is the combination of pancreatic reflux and epithelial irritation of a weakened cyst wall. Choledochal cysts should preferably be treated as soon as the child is 6 months old. Complete excision of the cyst is mandatory because of the risk of malignant transformation.

Right pulmonary agenesis associated with remaining bronchus stenosis, an equivalent of postpneumonectomy syndrome. Treatment by insertion of tissue expander in the thoracic cavity.

We report a case of right pulmonary agenesis with worsening of mediastinal shift during a viral bronchiolitis at the age of 5 months. The respiratory failure was due to stretching and narrowing of the remaining bronchus, as in postpneumonectomy syndrome. Weaning of respiratory assistance was possible after inserting a tissue expander in the empty side of the thoracic cavity. The correction of the shift brought a liberation of the left main stem bronchus. The main risk is heart compression. The child is now 3 years old and is at home. Filling of the prosthesis is done according to the clinical evolution.

Life-threatening haemorrhage as a complication of a congenital haemangioma.

UNLABELLED: Life-threatening complications of congenital haemangiomas are rare. A case of haemorrhage in a neonate is reported here. A neonate presented a congenital haemangioma of the limb complicated by a massive life-threatening haemorrhage. Colour ultrasonography showed a hypervascularized, highly haemodynamically active mass with a large, superficial drainage vein just under the ulceration. Surgical resection was performed with satisfactory postoperative outcome and no evidence of recurrence. CONCLUSION: Early surgical removal can be a good therapeutic option for complicated congenital haemangiomas.

Treatment of post-appendectomy intra-abdominal deep abscesses.

The treatment of acute appendicitis in children is sometimes followed by complications including intra-abdominal abscess, for which the traditional treatment is surgical drainage. We evaluated the efficacy of antibiotic management compared to classic surgical treatment. This retrospective study investigated 22 children from 5 to 13 years of age with one or many abscesses after appendectomy, treated between 1992 and 2002. Eleven received surgery and the other 11 were treated with triple antibiotherapy. The two groups were comparable. Surgery was efficient in 36% of cases and complications occurred in 64% of cases (digestive fistula, intraperitoneal abscess, gaseous gangrene and septic shock). Average hospital stay in this group was 16.7 days. In the other group, medication was efficient in 91% of cases; a recurrent abscess was operated and a residual stercolith, which was maintaining the infection, was removed. The average hospital stay in this group was 10.4 days. Medical treatment of intraperitoneal abscess seems to be effective. Hospitalisation is shorter with medical management and complications are rare. Therefore, medication may be proposed in most cases, except when there are residual foreign bodies or stercoliths. Poor patient status and septic shock are the two other contraindications, because antibiotherapy is not immediately efficient.

Laparoscopic treatment of ovarian cyst in the newborn.

BACKGROUND: Nine cases of persistent or complicated cyst were analyzed in an attempt to answer two questions: whether laparoscopic treatment of ovarian cyst in the newborn is justified and whether a pneumoperitoneum can be used in such infants. METHODS: Laparoscopic treatment was used for nine newborn babies. The children were 4 days to 2.5 months old. Cyst size ranged from 36 to 72 mm in length. RESULTS: The treatment was exclusively laparoscopic in six cases, and the other three cases required conversion. A maximal insufflation of 4 mmHg was used in five cases. Adnexectomy was necessary in three cases. The duration of the laparoscopic procedure was 20 to 75 min, and that of the postoperative stay was 1 to 5 days. There were no intraoperative or postoperative complications. CONCLUSIONS: Laparoscopy in the newborn is justified, but only in the hands of well-trained teams. The use of a pneumoperitoneum is possible, but should be reserved for difficulties with abdominal wall suspension.

[Early repair for infants with cleft lip. Retrospective study of 263 cleft lip repairs]. / Réparation chirurgicale précoce des fentes labiales. Revue de 218 enfants (263 fentes labiales) opérés.

This study attempts to define the effect of early repair on the surgical procedure and immediate outcome of cleft lip surgery. The first part deals with 263 consecutive cleft lip repairs (218 infants) categorized retrospectively by age at operation. 123 patients (150 cleft lips) were operated on during the first 4 weeks of life; a subgroup of 40 infants was operated on at a week or less of age; 95 patients (113 cleft lips) were operated at an older age (1 to 12 months). There were no significant difference in immediate surgical result between the groups and there was no apparent difference in the operative results as defined by whether or not the child needed subsequent revision. We are currently encouraging early repair in the full-term baby as the optimum method of management of newborns with cleft lip.

Abdominal cystic lymphangiomas in children: presurgical evaluation with imaging.

Cystic lymphangiomas are benign vascular tumors which are most often seen in young children. They are considered to be congenital malformations stemming from sequestration of lymphatic tissue. The authors report 15 cases of abdominal location and detail the findings of imaging in the etiologic and topographic diagnosis of these lesions. The initial incidents were essentially the discovery of a palpable abdominal mass, and more rarely, an acute gastrointestinal complication. There was also one case of prenatal diagnosis. Plain films provide only indirect signs related to the displacement of neighboring organs. Ultrasonography permits the etiologic diagnosis by showing an often voluminous, septated cyst. The intra- or retroperitoneal location of the lesion is sometimes difficult to determine by sonography, in which case CT scanning is usually adequate. For abdominal locations, percutaneous sclerosis is not available, and surgical removal is the only treatment for this disorder. The topography of the lesion and the involvement of retroperitoneal structures is important to determine.

[Color Doppler sonography of superficial capillary hemangiomas]. / Doppler couleur des hémangiomes capillaires superficiels.

PURPOSE: Retrospective analysis of the morphologic and hemodynamic characteristics of hemangiomas in infants provided by color Doppler sonography. MATERIALS AND METHODS: 94 hemangiomas were studied in 87 children separated into four classes of age (0-3 months, 3-6 months, 6-12 months, > 12 months). Eleven were followed with several examinations. Echogenicity, compared to normal subcutaneous tissues, and degree of vascularization were assessed qualitatively. Resistive index (RI) was measured in 78 cases. RESULTS: All lesions presented as vascularized solid masses: 63% were hypoechoic, 16% hyperechoic and 21% mixed. All hypoechoic and mixed lesions showed higher vascularity than hyperechoic ones. Hyperechoic hemanigomas were more frequent in the fourth class of age. Mean resistive index was significantly higher in the first (0.61 +/- 0.14) and the fourth (0.6 +/- 0.14) classes of age than in the second (0.51 +/- 0.12) (p = 0.01 and < 0.03, respectively), and higher in the hyperechoic group (0.7 +/- 0.13) than in the hypoechoic group (0.53 +/- 0.11), (p < 0.01). Seven of 11 cases moved from hypoechoic to hyperechoic or mixed. Increase of resistive index with age was noted in 9/11 cases. CONCLUSIONS: Superficial hemangiomas have typical but variable gray-scale, spectral and color flow sonographic patterns. These fluctuations are probably related to phases of evolution.

Phagocytic reaction in contact with macroplastic: application in pediatric surgery.

BACKGROUND/PURPOSE: With regard to the problems of using polytetrafluoroethylene (PTFE) in the treatment of vesicoureteric reflux in children, the authors have tested polydimethylsiloxane (PDMS), which is conveyed by polyvinylpyrrolidone (PVP), a hydrogel. METHODS: The phagocytic reaction was tested with both PDMS and PVP. The phagocytic reaction of PDMS and PVP was tested in vitro, then the outcome of PVP was tested in vivo in the mouse. RESULTS: In vitro PVP was phagocytosed by mouse peritoneal macrophages. However, PDMS particles were not phagocytosed because of their large size. In vivo, PVP migrated to other organs but did not induce clear histologic lesions. CONCLUSIONS: Silicone (PDMS) has been used in the treatment of vesicoureteric reflux in children. It is now necessary to do tests on heavier animals with a long incubation period to know whether these particles migrate and what the histologic and clinical consequences might be.

Treatment of facial dog bite injuries in children: a retrospective study.

PURPOSE: The authors assess their approach to immediate surgical repair with general anesthesia of facial dog bites in children over a 10-year period in their pediatric department. METHODS: The authors reviewed a series of 100 children (59 boys, 41 girls) who required immediate surgical intervention during the first hours after admission between 1985 and 1995. Strict disinfection was always used to preclude the risk of rabies, and parenteral antibiotherapy was used to avoid aero-anaerobic infections. RESULTS: A total of 68% of children were below 5 years of age. The most frequent injury was to the midface. Associated injuries or death were not observed. One case of postoperative wound infection was noted. Four surgical revisions were performed because of unsightly scars, but in most children the scar had attenuated 1 year after injury. CONCLUSIONS: The aim of immediate surgical repair is to obtain a satisfactory cosmetic result and to avoid infections. Better cosmetic results (4 cases of scar revisions) with a minimal risk of wound infections (1 cheek wound infection) are obtained.

Capillary haemangioma of the greater omentum in a 5-month-old female infant: a case report.

Capillary haemangiomas are frequent benign tumours in infancy. The authors report a case of capillary haemangioma of the greater omentum, discovered in a child of 5 months of age and studied with US, CT and MRI. The localization of such a lesion in the greater omentum is exceptional. Abdominal US revealed a heterogeneous, multinodular intraperitoneal mass. Doppler study demonstrated hypervascularity of the lesion. CT localized the mass to the greater omentum. The mass was hypodense on the unenhanced scan and enhanced massively after injection. The infant suffered a reaction to contrast medium during the CT. MRI demonstrated a mass which was hypointense on T1-weighted images and hyperintense on T2-weighted images. Laparotomy confirmed the location of the mass within the greater omentum and allowed resection of the tumour.

Expression of collagens type I and IV, osteonectin and transforming growth factor beta-1 (TGFbeta1) in biliary atresia and paucity of intrahepatic bile ducts during infancy.

BACKGROUND/AIMS: Biliary atresia and paucity of intrahepatic bile ducts are the main causes of neonatal cholestasis leading to hepatic fibrosis. Fibrotic evolution is slow in paucity of bile ducts as compared to the rapid progression to biliary cirrhosis in biliary atresia when cholestasis persists despite hepatoportoenterostomy. Our aim was to compare the expression of collagens type I and IV, alpha-smooth muscle actin, osteonectin and transforming growth factor beta1 in biliary atresia and paucity of bile ducts. METHODS: Liver biopsies were obtained in 12 children with biliary atresia and in five with paucity of bile ducts. Collagens type I and IV, alpha-smooth muscle actin were detected with immunostaining. Collagens type I and IV, osteonectin and transforming growth factor beta1 mRNAs were detected by in situ hybridization. RESULTS: Expression of mRNA and proteins was roughly parallel. In ductular proliferation areas of biliary atresia: (1) the expression of collagens type I and IV and osteonectin was increased, and was localized to periductular myofibroblasts; (2) transforming growth factor beta1 was expressed around biliary ductules, probably in inflammatory cells, and also in biliary cells. Osteonectin expression was also increased in the lobules. In paucity of bile ducts, there was no overexpression of collagens type I and IV and transforming growth factor beta1, except in the only child with marked fibrosis. However, osteonectin expression was enhanced at the periphery of the lobules, even when fibrosis was mild or absent. CONCLUSIONS: These findings suggest that in biliary atresia ductular proliferation areas are the site of a marked production of extracellular matrix proteins in periductular myofibroblasts, probably secondary to transforming growth factor beta1 production by inflammatory cells and by biliary cells. The weak expression of transforming growth factor beta1 could explain the slow progression of fibrosis in paucity of bile ducts.

[Severe hemangiomas treated with interferon alpha-2b: seven cases]. / Hémangiomes graves traités par interféron alpha-2b: 7 cas.

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.

Second thyroid neoplasms after prophylactic cranial irradiation for acute lymphoblastic leukemia.

An understanding of the pathogenesis of second cancers may help in their prevention. We report on two children who were treated for acute lymphoblastic leukemia (ALL), with an exclusively cranial prophylactic irradiation (18 Gy) and who presented with a thyroid carcinoma (TC) 12 and 13 years later. From a thorough review of the literature of TC after ALL and of radiation-induced TC, a strong case can be made that these tumors are caused by late effects of scattered radiation. The risk is at its highest in small children. After cranial irradiation, patients require clinical monitoring of the thyroid and cervical area for nodules, continued indefinitely. We suggest that, in most cases, an alternative form of neuromeningeal prophylaxis should be offered in small children with ALL.

[Severity of ingestion of caustic substance in children]. / Gravité des ingestions de produits caustiques chez l'enfant.

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.

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.

[Lymphomatoid papulosis in a child]. / Papulose lymphomatoïde chez un enfant.

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.