Critical Analysis of a Series of Early Inguinal Ureterostomies: Are They Useful and How Well Are They Tolerated?

INTRODUCTION: The study aimed to evaluate the advantages of temporary inguinal ureterostomy in the management of neonates with uropathies and early or recurrent pyelonephritis. PATIENTS AND METHODS: We performed a retrospective analysis of all patients who underwent ureterostomies between 1989 and 2012, with specific regards to indications and outcomes. We also performed a survey of parents to evaluate their acceptance of diversion. RESULTS: We included 18 patients (12 primary high-grade vesicoureteral reflux [VUR] and 6 primary obstructive megaureters [MUs]). Indications were recurrent febrile urinary tract infections (UTIs) despite antibiotic prophylaxis, doubtful function of the overlying kidney for the oldest cases, when renal function was only assessed by intravenous urography, or both. Cutaneous diversion was performed between the ages of 2 weeks to 5 months (median: 1.8 months). Renal function was assessed prior to undiversion to choose between reimplantation and nephrectomy. The incidence of febrile UTIs significantly decreased during the period of diversion. Urinary diversion was judged socially acceptable by parents. Ureterostomy did not modify the overlying kidney function. CONCLUSION: Temporary inguinal ureterostomy does not enable better evaluation of renal function by suppressing the pressure of an obstacle or refluxing urines. Its remaining indication seems to be the prevention of recurrent UTIs in neonates and infants with VUR or MU, pending reimplantation.

Identification of a complex 17q rearrangement in a metanephric stromal tumor.

Metanephric stromal tumor is a rare benign entity belonging to the group of metanephric renal tumors in children. Although metanephric stromal tumors can be cured by simple nephrectomy, differential diagnosis based on histopathologic criteria with other pediatric renal tumors requiring aggressive chemotherapy can be difficult. To our knowledge, cytogenetic characterization of metanephric stromal tumor has never been reported. We describe conventional ("R-bands" karyotyping) and molecular [fluorescence in situ hybridization (FISH), multicolor FISH, oligo array-comparative genomic hybridization] cytogenetic examinations of a metanephric stromal tumor in a 3-year-old boy. Cytogenetic analysis revealed a complex homogeneous gain between bands 17q22 and 17q25.3, resulting in partial triplication of the segment between bands 17q22 and 17q24.3, and duplication of the segment between bands 17q24.3 and 17q25.3. Cytogenetic confirmatory studies in metanephric stromal tumors are currently needed to assess 17q22q25.3 gain as a recurring cytogenetic abnormality of metanephric stromal tumors.

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.

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 pre-recruited, 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.

Postoperative cystography and endoscopic treatment of low-grade vesicoureteral reflux.

INTRODUCTION: The endoscopic subureteral injection of tissue-bulking agents has become an established alternative to long-term antibiotic prophylaxis and open surgery with a high success rate, especially for low-grade reflux (>90%). Though it is recognized that a routine postoperative voiding cystourethrography (VCUG) is unnecessary following a ureteroneocystostomy, most teams perform one after an endoscopic treatment. MATERIALS AND METHODS: In this paper, we report on our experience with the endoscopic correction of vesicoureteral reflux in 72 ureteral units, for whom no routine postoperative cystography was performed. DISCUSSION: Two children presented with postoperative recurrent febrile urinary tract infections (UTIs), which were not correlated with cystography findings. Postoperative VCUGs after a ureteroneocystostomy are invasive and expose the child to radiation, they are associated with a substantial cost, and most of all, they do not allow the identification of those patients at risk of recurrent febrile UTIs. Further, the endoscopic subureteral injection of tissue-bulking agents have been used for several years, and numerous studies, set in various clinical settings, have since been published, confirming excellent long-term results for low-grade reflux. CONCLUSIONS: We feel that postoperative cystograms should be reserved for children who present with recurrent UTIs, new sonographic abnormalities, or who were treated for high-grade reflux.

Further documentation of spontaneous regression of infantile digital fibromatosis.

Infantile digital fibromatosis is a rare benign fibromatous tumor characterized by both its location on fingers and toes and its distinctive light microscopic appearance. However, treatment modalities are not yet standardized. Surgical excision had been preferred in the past but has the disadvantage of a high recurrence rate. More recently, isolated instances of spontaneous regression have been reported with a short follow-up. We report four children with infantile digital fibromatosis, who underwent spontaneous regression after clinical monitoring and long-term follow-up. We recommend regular follow-up in order to allow for intervention in case of functional impact on the affected extremities.

Multicentric assessment of the safety of neonatal videosurgery.

BACKGROUND: Complex 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. METHODS: From 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. RESULTS: Of 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 degrees 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. CONCLUSION: Despite 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.

Glomus tumor of the mesocolon.

Glomus tumors are rare distinctive benign neoplasms, which arise from modified smooth muscle cells of the normal glomus body and are most commonly located in the subungual region of the finger. Intraabdominal locations are relatively rare. We report a case of glomus tumor of the mesocolon in a 10-year-old girl. Surgical exploration showed a lesion in the transverse mesocolon, which was excised. Histopathology showed it to be a glomus tumor of the mesocolon.

Surgical treatment of haemangioma in infants.

Haemangiomas usually can be identified by their clinical course. They are characterised by presentation at birth or shortly thereafter, and a rapid proliferative phase over the first 12 months. The haemangioma then usually stabilises and slowly involutes over a period of 5-7 years. For a long time, surgery has been limited to complicated cases, and correcting after-effects following involution. Nevertheless, aesthetic, psychological or functional prejudices may justify early surgery. We conducted a retrospective study of patients treated between 1995 and 2001. A total of 31 patients with facial and cervical haemangiomas were studied. For each, the type of lesion and its topography, age and operative indications, surgery, postoperative complications and aesthetic and functional results have been considered. Thirty-one haemangiomas were operated. The average age was 30 months (1-60 months). After an average follow-up of 3 years, the results were very good in 20%, good in 66%, and fair in 14% of cases. Early curative surgery of haemangioma before spontaneous involution, and before school-age is justified because of social and psychological considerations in infants and their family.

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 Unna's 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.

The 'Abtropfung phenomenon' revisited: Dermal nevus cells from congenital nevi cannot activate matrix metalloproteinase 2 (MMP-2).

Since Unna's Abtropfung hypothesis, the process of migration of nevus cells in the dermis remains unknown. To investigate its mechanisms, we studied the role of gelatinases in dermal nevus cells obtained from congenital pigmented nevi, which are major actors in the remodeling of basement membrane proteins. Our previous studies have shown that dermal nevus cells express pro-matrix metalloproteinase (MMP)-2 exclusively and cannot return to the dermis when seeded together with keratinocytes on top of the dermis in a skin reconstruction model. To examine why MMP-2 was not in its active form, we used Western blot to study the expression of members of the MMP-2 activation pathway (membrane type 1-MMP and tissue inhibitor of metalloproteinase-2), which proved to be normally expressed. To induce the dermal passage of nevus cells artificially, we also tried to activate gelatinases with phorbol-12-myristate-13-acetate and epidermal growth factor, using epidermis reconstructed with nevus cells. No migration in the dermis could be triggered. We conclude that the absence of active MMP-2 is due to a functional blockade of its activation pathway and may prevent dermal nevus cells from reaching the dermal compartment in skin reconstructs. Furthermore, our findings reinforce the concept that dermal nevus cells originating from congenital nevi are in a quiescent status.

[Epididymal tumor in a six-months-old infant]. / Tumeur épididymaire chez un nourrisson de six mois.

A rare tumor of the epididymis was discovered in a 6-month-old infant. Macroscopically, the tumor had a black focal color. Immunohistochemistry staining and electron microscopy led to the diagnosis of mealnotic neuroectodermal tumor, or progonoma. Prognosis of progonoma, a benign tumor, is generally good, but malignant transformation has been reported.

Dermal nevus cells from congenital nevi cannot penetrate the dermis in skin reconstructs.

Congenital nevi are composed of pigment cells bearing common features with melanocytes but showing altered differentiation which leads to nesting and dermal involvement. Using a dead de-epidermized dermis seeded with a combination of keratinocytes and various sources of pigment cells (normal melanocytes, dermal nevus cells from congenital nevi, Bowes melanoma cells), we have studied the formation of nests and the dermal migration of pigment cells together with their secretion profiles of matrix metalloproteinases (MMP). Dermal fibroblasts were also used as control cells in epidermal reconstructs. Besides their morphologic features, the absence of pigment donation to keratinocytes was the major characteristic of dermal nevus cells. A positive correlation was established between the increasing percentage of seeded nevus cells and the patchy pigmentation of reconstructs, as well as the clustering of cells in junctional nests. However, the presence of nevus cells in the dermis of reconstructs was never detected, whereas melanoma cells and dermal fibroblasts could invade the dermis during the time span of the experiments. MMP9 was never expressed in congenital dermal nevus cells but pro-MMP2 was constitutively expressed by all strains of congenital nevus cells and dermal fibroblasts. Melanocytes produced comparable amounts of both pro-MMP2 and pro-MMP9, and Bowes melanoma cells secreted a marginal level of pro-MMP2. In view of their three-dimensional behaviour and secretion of MMPs, we propose that dermal congenital nevus cells correspond to an intermediate status of differentiation between normal melanocytes and melanoma cells. Activation of MMPs by a cofactor or the activation of another signalling pathway seems necessary to induce the dermal passage of nevus cells.