Pierre Vergnes

Propranolol for Severe Infantile Hemangiomas: Follow-Up Report

OBJECTIVE: Infantile hemangiomas (IHs) are the most-common soft-tissue tumors of infancy. We report the use of propranolol to control the growth phase of IHs. METHODS: Propranolol was given to 32 children (21 girls; mean age at onset of treatment: 4.2 months) after clinical and ultrasound evaluations. After electrocardiographic and echocardiographic evaluations, propranolol was administered with a starting dose of 2 to 3 mg/kg per day, given in 2 or 3 divided doses. Blood pressure and heart rate were monitored during the first 6 hours of treatment. In the absence of side effects, treatment was continued at home and the child was reevaluated after 10 days of treatment and then every month. Ultrasound measurements were performed after 60 days of treatment. RESULTS: Immediate effects on color and growth were noted in all cases and were especially dramatic in cases of dyspnea, hemodynamic compromise, or palpebral occlusion. In ulcerated IHs, complete healing occurred in <2 months. Objective clinical and ultrasound evidence of longer-term regression was seen in 2 months. Systemic corticosteroid treatment could be stopped within a few weeks. Treatment was administered for a mean total duration of 6.1 months. Relapses were mild and responded to retreatment. Side effects were limited and mild. One patient discontinued treatment because of wheezing. CONCLUSION: Propranolol administered orally at 2 to 3 mg/kg per day has a consistent, rapid, therapeutic effect, leading to considerable shortening of the natural course of IHs, with good clinical tolerance.

Accidental caustic ingestion in children : is endoscopy always mandatory?

Background Patients who have experienced severe caustic injury to the gastrointestinal tract are at high risk of esophageal strictures. Early endoscopy is usually recommended systematically in children after caustic ingestion to assess the severity of the initial digestive lesions. The aim of this study was to determine the predictive value of clinical symptoms and ingested-substance types as markers of severe esophagogastric lesions and to define indications for endoscopy. Methods Ingested-product types, clinical symptoms, endoscopic data and outcome were prospectively recorded in 85 children admitted after accidental caustic ingestion. Results Forty-eight children (57%) had no symptoms; the others presented with vomiting, hematemesis, drooling, respiratory distress, and/or oropharyngeal lesions. Endoscopy showed no or minimal lesions in 63 cases (74%). None of the children developed digestive sequelae. Severe esophagogastric lesions were present in 22 cases (26%), mostly caused by lye ingestion (14 of 22) but also by strong acids (4 of 22); 9 of the 22 children (41%) developed esophageal stenosis. Vomiting, drooling, and oropharyngeal lesions did not predict severe endoscopic lesions. Hematemesis, respiratory distress, or presence of at least three of the symptoms was associated with severe lesions (positive predictive value = 1). The absence of symptoms was always associated with no or minimal lesions (negative predictive value = 1). Conclusions In conclusion, endoscopy is not recommended for children living in developed countries who are asymptomatic after accidental caustic ingestion.

Multicentric assessment of the safety of neonatal videosurgery

BackgroundComplex procedures for managing congenital abnormalities are reported to be feasible. However, neonatal videosurgery involves very specific physiologic constraints. This study evaluated the safety and complication rate of videosurgery during the first month of life and sought to determine both the risk factors of perioperative complications and the most recent trends in practice.MethodsFrom 1993 to 2005, 218 neonates (mean age, 16 days; weight, 3,386 g) from seven European university hospitals were enrolled in a retrospective study. The surgical indications for laparoscopy (n = 204) and thoracoscopy (n = 14) were congenital abnormalities or exploratory procedures.ResultsOf the 16 surgical incidents that occurred (7.5%), mainly before 2001, 11 were minor (parietal hematoma, eventration). Three neonates had repeat surgery for incomplete treatment of pyloric stenosis. In two cases, the incidents were more threatening (duodenal wound, diaphragmatic artery injury), but without further consequences. No mortality is reported. The 26 anesthetic incidents (12%) that occurred during insufflation included desaturation (<80% despite 100% oxygen ventilation) (n = 8), transient hypotension requiring vascular expansion (n = 7), hypercapnia (>45 mmHg) (n = 5), hypothermia (<34.9°C) (n = 4), and metabolic acidosis (n = 2). The insufflation had to be stopped in 7% of the cases (transiently in 9 cases, definitively in 6 cases). The significant risk factors for an incident (p < 0.05) were young age of the patient, low body temperature, thoracic insufflation, high pressure and flow of insufflation, and length of surgery.ConclusionDespite advances in miniaturizing of instruments and growth in surgeons’ experience, the morbidity of neonatal videosurgery is not negligible. A profile of the patient at risk for an insufflation-related incident emerged from this study and may help in the selection of neonates who will benefit most from these techniques in conditions of maximal safety.

Expression of collagens type I and IV, osteonectin and transforming growth factor beta-1 (TGFβ1) 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.

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.

INFANTILE PERIANAL PYRAMIDAL PROTRUSION: A MARKER OF CONSTIPATION?

To the Editors: We read with interest the article by Paller et al. “Pulmonary Hemorrhage: An Often Fatal Complication of Henoch-Schonlein Purpura” (1). We have previously published a similar report of pulmonary hemorrhage as a complication of Henoch-Schonlein purpura (2). Our patient, a 14-year-old girl, presented with abdominal pain, melena, arthritis, generalized edema, diffuse proliferative glomerulonephritis, and palpable purpura and had lifethreatening pulmonary hemorrhage requiring intensive care unit admission. While a skin biopsy specimen showed vasculitis with positive IgA staining, pulmonary pathology of a lung biopsy specimen showed acute vasculitis with IgM and C3 staining. The patient responded well to ICU supportive care, peritoneal dialysis, and pulse methylprednisolone. Our patient’s case was not included in the literature summary presumably because it was published as a case of ‘‘anaphylactoid purpura.” Our article also referenced two other pediatric cases not included in the Paller et al. article (3,4). Additionally, since our publication there has been another case report of a 15-year-old boy with pulmonary hemorrhage complicating HSP (5) . We concur with Paller et al. on the importance of physician knowledge of pulmonary hemorrhage with Henoch-Schonlein purpura and of the severe morbidity and mortality of this fairly rare complication.

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. Am. J. Hematol. 59:91–94, 1998.

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

Abstract 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.

The role of E-cadherin in nevogenesis: an experimental study using epidermal reconstructs

Background: In the development of congenital nevi, how nevus cells migrate in the dermis remains unclear. As shown in an earlier study designed to investigate Unnas Abtropfung hypothesis, dermal invasion does not occur when nevus cells are seeded on epidermal reconstructs. In melanoma, the decrease of E‐cadherin expression is associated with the dermal invasion of melanoma cells. Objective: To study the expression of E‐cadherin in dermal‐cultured nevus cells from congenital nevi and its relevance to explain the absence of dermal invasion noted in epidermis reconstructed with cultured nevus cells. Methods: Comparison of the immunohistochemical expression pattern of E‐cadherin in congenital nevi in vivo and after culture in monolayers and in a three‐dimensional system. Results: E‐cadherin was not expressed in vivo by dermal nevus cells, either isolated or in nests. However, in monolayer cultures, dermal nevus cells expressed E‐cadherin. When these cells were used in reconstructed epidermis, nevus cells did not invade the dermis and they expressed E‐cadherin when isolated and just weakly or not when grouped in junctional nests. Conclusions: The absence of dermal invasion of nevus cells could be due to the expression of E‐cadherin in these cells in reconstructed epidermis. Our experiments suggest, a restoration of the control of keratinocytes, that nevus cells escape in the dermal compartment.

Management of congenital nevi at a dermatologic surgical paediatric outpatient clinic: consequences of an audit survey 1990-1997.

Background: Since 1987 we have run a Dermatologic Surgical Paediatric Outpatient Clinic (DSPOC) within the Children’s Hospital in Bordeaux. Objective: We analyse the consequences of an audit survey concerning the management of patients with congenital nevi (CN) seen at this clinic. Methods: We reviewed the cases of 192 children examined and photographed at the DSPOC during the period January 1990–December 1997. Patients were contacted for a reassessment of their status. The management options chosen at the DSPOC were reviewed as well as the satisfaction of the patients or parents of young children. Results: Of 192 children prerecruited, 56 girls and 52 boys could be included in the survey. They were mostly European whites and 67% were <6 months of age at the first DSPOC visit. 65/108 (61%) had been operated following the first DSPOC visit. The mean follow-up based on the 1997–1998 survey was 33 months (8 months to 10 years). The size of the nevus, independently of location, influenced decision for early surgery. Another important factor was the estimated disfigurement risk (15% of decisions) mostly related to CN of the face. There was a significant risk of pigmentary recurrence around the scar in children operated before the age of 2, but long-term follow-up indicated a spontaneously regressive course. Conclusions: Nevus recurrence in cases operated early suggests a time-dependent phenomenon in nevogenesis. Early counselling is important. Early surgery seems associated with a better scar quality. Explanations concerning risks and outcome are best given with the cooperation of a surgeon and a dermatologist.