Evaluating the impact of pop-off mechanisms in boys with posterior urethral valves.

Introduction: Posterior urethral valves are urethral leaflets that cause Lower Urinary Tract Obstruction (LUTO) in boys and are associated with congenital renal dysplasia and abnormal bladder function. They affect 1:4,000 to 1:25,000 births and can be responsible for End-Stage Renal Failure in childhood. There have been several studies on the effect of pop-off mechanisms in boys with posterior urethral valves, but results are contradictory. We aimed to assess and discuss the effect of pop-off mechanisms on renal function in a large cohort of patients. Patients and method: Boys with PUV with and without pop-off mechanisms (urinoma, VURD or giant bladder diverticula) were divided into three severity groups for renal function according to their nadir creatinine (low-risk NC < 35 µmol/L, intermediate-risk NC between 35 and 75 µmol/L, and high-risk NC > 75 µmol/L). We compared children with and children without pop-off mechanisms for mean renal function as well as patient distribution within each severity group. Results: We included 137 boys of which 39 had a pop-off mechanism. Patients had complete data for at least 5 years follow-up. Though there was no significant statistical difference in mean renal function between the pop-off and non-pop-off group, patient distribution within each severity group varied according to whether patients had a pop-off mechanism or not. Conclusion: Though there was no significant difference in mean renal function between boys with and without pop-off mechanisms, it is possible that these are two different patient populations and direct comparison is not possible.

Circumcision and Risk of Febrile Urinary Tract Infection in Boys with Posterior Urethral Valves: Result of the CIRCUP Randomized Trial.

BACKGROUND: Boys with posterior urethral valves (PUVs) have an increased risk of febrile urinary tract infections (fUTIs). Circumcision is believed to reduce the risk of fUTIs in boys, although there are no randomized trials demonstrating this. OBJECTIVE: To determine the effect of circumcision on the risk of fUTIs in boys with PUVs. DESIGN, SETTING, AND PARTICIPANTS: A clinical randomized trial that ran between August 2012 and July 2017 was conducted. The trial was multicentric, including 13 referral centers for pediatric urology. Male boys, aged 1-28 d, diagnosed with posterior urethral valves, confirmed by voiding cystogram, were included. The exclusion criteria included presence of a genital malformation contraindicating performing a circumcision. INTERVENTION: Participants were randomized to neonatal circumcision + antibiotic prophylaxis (CATB) or antibiotic prophylaxis alone (ATB), and followed for 2 yr. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: The primary outcome was a risk of presenting fUTIs in each group. An fUTI was defined as fever (>38.5 °C) with evidence of pyuria and culture-proven infection on urinalysis, obtained by urethral catheterization or suprapubic aspiration. A bivariate analysis of the primary outcome was performed using the Kaplan-Meier method. RESULTS AND LIMITATIONS: In total, 91 patients were included: 49 in group CATB and 42 in group ATB. The probability of presenting an fUTI was 20% in group ATB versus 3% in group CATB. The hazard ratio of presenting an fUTI within 2 yr in the ATB group compared with that in the CATB group was 10.3 (95% confidence interval: 1.3-82.5). Sixty-four children (70.3%) had a complete follow-up at 2 yr of age. CONCLUSIONS: Circumcision significantly decreases the risk of presenting an fUTI in boys with PUVs. PATIENT SUMMARY: In this report, we compared, in a multicentric trial, the number of febrile urinary tract infections (UTIs) in boys with posterior urethral valves who had either antibiotic prophylaxis alone or antibiotic prophylaxis and circumcision. We found that those who had a circumcision had a significantly lower risk of febrile UTIs.

Cortical transit time as a predictive marker of the need for surgery in children with pelvi-ureteric junction stenosis: preliminary study.

INTRODUCTION: Postnatal management of prenatally detected hydronephrosis remains controversial. It has been suggested that cortical transit time (CTT) could successfully predict deterioration in children with pelvi-ureteric junction (PUJ) obstruction. We decided to conduct a retrospective study in our hydronephrosis population to evaluate whether initial CTT was significantly correlated with the need for surgery. PATIENTS AND METHOD: We reviewed the charts of all our patients managed for significant PUJ obstruction (>12 mm) between 2007 and 2010 and determined CTT retrospectively, on the first diuretic scan of each of these patients. We then determined the relationship between initial CTT and the need for surgery. RESULTS: We identified 37 patients with hydronephrosis (pelvic size >12 mm) of which 26 were diagnosed prenatally. Out of 22 patients with an initial abnormal CTT, 20 underwent surgery. Out of 15 children with a normal initial CTT, 4 underwent surgery (OR 27.5 (IC95%: 4.3-174.9)). CONCLUSION: Initial CTT could be a reliable prognostic factor for future evolution of renal function in children with hydronephrosis. CTT is easy to determine on diuretic renal scan. A prospective trial is being devised to confirm what role it could have in the management of children with hydronephrosis.

Combined laparoscopic-assisted nephrectomy, augmentation ureterocystoplasty and Mitrofanoff appendicovesicostomy.

Conventional and robotic-assisted laparoscopy is being used for more and more complex urological procedures in children. There have recently been reports of laparoscopic or laparoscopic-assisted appendicovesicostomies in children. We report a case of combined laparoscopic-assisted nephrectomy, augmentation ureterocystoplasty and Mitrofanoff appendicovesicostomy in a 5-year-old boy with valve bladder syndrome.

[Management of pediatric liver abscess]. / Abcès hépatique chez l'enfant : à propos d'un cas.

Liver abscess in the pediatric population remains uncommon in developed countries, except in cases of septicemia or in children with major debilitating diseases, granulocyte dysfunction, or immunosuppression. Although much is known about the etiopathogenesis of liver abscess, the gold standard of investigations and treatment is still debatable in developing countries. We report the case of a 6-year-old child living in Reunion Island, with no medical history, presenting with right and pyretic abdominal pain in the right upper quadrant. Ultrasound and CT scan showed a large hypodense nonenhanced area in segment IV. Final diagnosis was, by exclusion, pyogenic liver abscess based on negative serology, recent liver lesion, and normal tumor test results, even if blood culture remained negative. No percutaneous puncture was done because of positive outcome after 4 days of antibiotics. Treatment consisted in three intravenous antibiotics (ceftriaxone, aminoxide, and metronidazole) until complete biological normalization. Ultrasound remained normal 3 months later. Even if liver abscess is uncommon in developing countries, the diagnosis must be raised in cases of isolated liver tumor with fever. Management in the nonimmunosuppressed child must be discussed associating parenteral antibiotic therapy, percutaneous drainage, or surgery in very uncommon cases, according to the liver location and first day's progression. Etiological investigation such as colonoscopy in adults must be adapted to pediatric data.

Giant hepatic cysts: prenatal findings and uncommon postnatal outcome.

With modern prenatal imaging, liver cysts are being diagnosed more often. Although large cysts are usually asymptomatic, they may present as an abdominal emergency requiring surgery in the first weeks of life. We report a series of 3 patients with prenatal diagnosis of isolated cystic liver lesions diagnosed at 22, 31 and 33 weeks of gestational age. The hepatic origin of the cysts was confirmed prenatally by a MRI in 2 cases, with visualization of a normal gallbladder. The prenatal course was uneventful. Postnatal ultrasound confirmed the diagnosis of liver cyst, showed normality of the biliary tract and in one case, rupture of the cyst during delivery. Because of an uncommon rapid increase in size, the 3 children underwent surgical excision of the cysts within the first weeks of life. These were non-bile-containing intrahepatic cysts arising from segment IV. Long-term follow up was uneventful.

[Eruptive naevi associated with octreotide treatment]. / Naevus éruptifs sous octréotide.

BACKGROUND: Octreotide is a synthetic octapeptide having properties related to those of natural somatostatin, a hypothalamic hormone. We report a case of eruptive naevus in patients treated with octreotide. PATIENTS AND METHODS: A 52-year-old man consulted for multiple achromic naevi of recent onset. He was undergoing treatment with octreotide 30 mg per month for hepatic metastases in a setting of operated colonic carcinoid tumor. Since the start of this treatment, he had presented an efflorescence of diffuse naevus comprising over 200 lesions, certain of which were highly atypical. Annual follow-up was carried out. Ten years later, the patient was still alive and undergoing octreotide therapy at the same dose. The number of naevi had stabilized and there was no evidence of melanoma. DISCUSSION: Eruptions of naevi are rare; they may occur at the sites of lesions in bullous diseases or during immunodepression (malignant or drug induced, HIV infection). There are questions concerning possible stimulation of naevus cells and the risk of onset of melanoma. Routine monitoring of pigmented lesions is thus necessary in patients treated with octreotide.

The return of the solitary testis.

PURPOSE: To assess what is done and what is recommended concerning fixation of the solitary testis. MATERIAL AND METHOD: We conducted an e-mail survey of current practices in 28 pediatric surgery departments in 28 university or general hospitals in France. We then reviewed what evidence could be found in the literature. RESULTS: All surgeons fix the contralateral solitary testis following intravaginal torsion. Sixteen out of 28 fix the contralateral solitary testis following extravaginal torsion, 13/28 in cases of monorchia, and 8/28 following orchiectomy for trauma or tumour. Five surgeons have observed one case each of torsion of a solitary testis, and three have witnessed testicular necrosis following orchiopexy. CONCLUSION: There is no consensus regarding fixation of the remaining testis in the literature. Scientific evidence does not show clearly whether fixation is necessary, regardless of the clinical situation. However, if fixation is performed it should be done using the dartos pouch sutureless technique.

Two more cases of evaluation of POEMS syndrome using 18-FDG PET/CT.

Following the article of Alberti et al., we would like to provide our own experience with two more cases in evaluation of POEMS syndrome using morphological and functional imaging modalities, including plain X-rays, computed tomography (CT), magnetic resonance imaging (MRI), bone scintigraphy and positrons emission tomography with computed tomography (PET/CT). Among them, 18-FDG PET/CT proved its usefulness allowing extensive screening of the bony lesions involved.

[Pulsed dye laser treatment for Jessner's lymphocytic infiltration of the skin]. / Infiltrat lymphocytaire de Jessner et Kanof traité par laser à colorant pulsé

BACKGROUND: Jessner's lymphocytic infiltration of the skin (LIS) is a chronic, benign T-cell infiltrative disorder, usually manifesting as erythematous papules or plaques on the face, neck and back. PATIENTS AND METHODS: five patients presented LIS with numerous skin lesions on the face and back characteristic of this disease. Histological examination showed a lymphocytic infiltrate in the dermis without any modification of the epidermis. Direct immunofluorescent study was negative in all cases. Response to dermocorticoids proved inconsistent or negative in all patients. An excellent outcome was achieved in all five patients with pulsed dye laser. In one case, further skin lesions appeared at 1 year and responded to the same treatment. Following a single treatment session with 6-8 J/cm(2), three of five patients showed normal skin. Regression occurred in the other two cases after two to three sessions. Pulsed dye laser appeared to be the best treatment for Jessner-Kanof disease for three patients at 4-8 years of follow-up. DISCUSSION: only one case of Jessner-Kanof disease treated by pulsed dye laser has been reported. Pulsed dye laser has been used in cutaneous lupus and annular granuloma. Selective photothermolysis allows photocoagulation of dilated vessels. CONCLUSION: pulsed dye laser at 595nm could offer a valuable therapeutic alternative, and even a first-line treatment with no side effects.

Should we perform orchidopexy for cryptorchidism in children with severe encephalopathy?

OBJECTIVE: To conduct a survey of practices in France concerning cryptorchidism in patients presenting with severe encephalopathy. MATERIAL AND METHOD: We conducted an e-mail survey of current practices in 27 pediatric surgery departments in 27 French university or general hospitals. The study concerned children with severe mental deficiency. RESULTS: The survey showed a disparity in attitudes towards cryptorchidism in patients with severe encephalopathy, ranging from no treatment to orchidopexy in all cases. In most cases, the indication for surgery was the psychological aspect for the parents or accessibility to clinical examination. CONCLUSIONS: This survey illustrates the unease concerning the pertinence of performing orchidopexy in these children. As improved fertility is not really an issue in children with severe encephalopathy, psychological aspects apply only to the family, and, as the benefit of orchidopexy for prevention or early diagnosis of testicular cancer is marginal, the risk/benefit ratio of orchidopexy for children with severe encephalopathy and cryptorchidism is unclear.

[Response to oral propranolol therapy for ulcerated hemangiomas in infancy]. / Efficacité des bêtabloquants par voie orale dans le traitement des hémangiomes prolifératifs du nourrisson.

INTRODUCTION: Hemangioma is the most common tumor of infancy. Ulceration and functional prognosis justify systemic treatment. CASE REPORT: We report the case of an 8-month-old girl with a voluminous ulcerated hemangioma of the left forearm with a large, painful, central ulceration. Conventional treatment with topical and/or systemic antibiotics was unsuccessful. Flashlamp-pumped pulsed-dye laser (FPDL) could not be used because of the thickness of the lesions. We decided to use beta-blocker therapy (propanolol) for 4 months with a noteworthy efficacy. DISCUSSION: Systemic corticosteroids and FPDL are currently the reference treatment of the superficial hemangioma during infancy, but the head and neck location or complications such as ulceration or the need for surgical embolization require the use of alternative treatments such as the beta-blocker. We discuss this option.

[Interventional radiology: transoral approach to C2]. / Abord transbuccal de l'axis en radiologie interventionnelle.

PURPOSE: To assess the value of a direct transoral approach to C2 for interventional procedures. MATERIALS AND METHODS: A transoral approach to C2 was used in 4 patients (9-64 year old) with lytic lesion of the C2 vertebra and/or base of the odontoid over a 10 year period. Three patients underwent biopsy and 1 patient underwent vertebroplasty using biplane fluoroscopy. All procedures were performed under general anesthesia using a 13G needle directly introduced in the C2 vertebra through the posterior pharyngeal wall. RESULTS: None of the patients reported ill-effects from the procedure. Needle placement was satisfactory in all patients, without technical difficulty. No technical complication was noted. Specifically, no infectious complication occurred. CONCLUSION: Our results indicate that a direct transoral approach to C2 is safe for biopsy or vertebroplasty.

Successful management of a retroperitoneal kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using alpha-interferon.

It has been shown recently that Kasabach-Merritt phenomenon, the association of a vascular tumour and consumption coagulopathy, does not--as previously thought--complicate "classical" infantile hemangiomas but distinctive entities called kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), both tumours on the same neoplastic spectrum. These tumours have been found in the neck, face, thorax, abdomen, retroperitoneum and limbs and are associated with a mortality rate of as high as 30 %. Several therapeutic modalities, including alpha-interferon, vincristine, radiotherapy and surgery have been reported in the literature. We report a case of retroperitoneal kaposiform hemangioendothelioma regression using alpha-interferon and discuss the current knowledge of this entity and its treatment.

[Topical tacrolimus treatment for ocular cicatricial pemphigoid]. / Traitement d'une localisation oculaire de pemphigoïde cicatricielle par tacrolimus topique.

BACKGROUND: Ocular involvement in cicatricial pemphigoid often occurs from the onset. Certain forms are seen only in this condition. OBSERVATION: A 31-year-old woman presented highly inflammatory conjunctivitis for several months associated with bilateral symblepharons and superficial punctuate keratitis around the left eye refractory to treatment. The patient had a history of mouth ulcers and sores on the scalp. Examination showed scalp lesions with crusts. Histological examination of these lesions revealed dermal-epidermal cleavage. Direct immunofluorescence showed sub-membrane deposits. Anti-basal membrane antibodies were positive. Immunotransfer confirmed the presence of circulating antibodies. This condition was controlled following administration of three courses of cyclophosphamide as a bolus. However, the symblepharons persisted in both eyes. Improvement lasted 4 years. The patient again consulted for inflammatory conjunctivitis and superficial punctate keratitis resulting in invalidating loss of visual acuity, associated with hypereosinophilia. Cortisone eye drops alone resulted in no improvement. Treatment was initiated with topical tacrolimus (Protopic) 0.03% comprising once-daily application to the conjunctiva in the evening. This therapy was well tolerated, and 2 daily applications could be given, followed by a dosage of 0.1%. Improvement was rapid and spectacular, with frank amelioration of visual acuity and resolution of the patient's keratitis. Treatment was continued for 4 months and gradually reduced to the 0.03% dosage level, with increasingly wide intervals between applications. There has been no relapse within the 12 months following the end of treatment. DISCUSSION: Standard treatment of pharmacological cicatricial pemphigoid involves systemic immunosuppression since topical anti-inflammatories are ineffective. The mortality associated with this disease is due to iatrogenic complications. Tacrolimus exhibits extremely good penetration of the conjunctiva. Following administration at a concentration of 0.06% 3 times daily in 15 patients with inflammatory disease of the conjunctiva or the cornea, improvement was seen in 10 of these patients at 26 weeks. Tacrolimus appears to act through immunomodulatory and anti-inflammatory mechanisms. It induces local inhibition of T lymphocyte activation and reduces production of pro-inflammatory lymphokines. Oral tacrolimus cannot be used to control cicatricial pemphigoid refractory to standard immunomodulators. However, 3 three other cases involving topical treatment of cicatricial pemphigoid showed marked efficacy of treatment given for 2 to 6 months, with complete tolerability. Thus, topical tacrolimus appears to constitute an interesting alternative treatment in cicatricial pemphigoid.

Outcome and long-term growth after extensive small bowel resection in the neonatal period: a survey of 87 children.

UNLABELLED: This retrospective study aims to analyze the outcome, the prognosis factors and the long-term growth of children after extensive small bowel (SB) resection in the neonatal period. PATIENTS AND METHODS: 87 children, born between 1975 and 1991 who had undergone extensive neonatal small bowel resection, were followed up over a mean period of 15 years. Anatomical data influencing PN dependency and duration were analyzed. Data on height and weight were collected and compared using growth standards. Final heights were studied for patients who achieved their puberty and compared to predicted height based on Tanner's formula. Patients were analyzed according to PN weaning and growth: children still receiving PN (group A), patients weaned from initial PN but requiring PN once again or enteral feeding (group B), and children with permanent intestinal autonomy (group C). RESULTS: The overall survival is 89.7 %, depending on the date of birth. The duration of PN-dependency varies according to the intestinal length and the presence of the ileocaecal valve (ICV). All patients who remain PN dependent had less than 40 cm of small bowel and/or the absence of ICV. Patients in group B had a mean small bowel length of 35 +/- 19 cm, resection of the ICV in 50 % of cases, and a PN duration of 47.4 +/- 23.8 months. There was a significant decrease in height and weight gain within the 4 years after cessation of PN, requiring enteral or parenteral feeding. Patients in group C had a mean small bowel length of 57 +/- 19 cm, presence of ICV in 81 % of cases and a PN duration of 16.1 +/- 11.4 months. After PN weaning, they grow up normally with normal puberty and final height as predicted from genetic target height. CONCLUSION: PN duration is influenced by the length of residual SB and the absence of ICV. With good anatomic prognosis factors and short duration of initial PN, normal long-term growth may be predicted. Conversely, poor anatomical factors and protracted initial PN require careful monitoring of growth and may sometimes require nutritional support to be restarted. The last group, permanently dependent on PN, might be candidates for intestinal transplantation.

Peritoneal needle suction for intestinal perforation in the preterm neonate.

UNLABELLED: A few years ago, most intestinal perforations in the premature newborn appeared within the clinical context of necrotising enterocolitis (NEC). Since then, we have observed an increase in the number of isolated perforations appearing outside typical NEC. The fact that the perforations are more often isolated, and the healing capabilities of the premature intestine, led us to propose peritoneal needle suction (PNS) alone as first treatment for intestinal perforations in the premature neonate. MATERIALS AND METHOD: The charts of 6 consecutive premature infants presenting with intestinal perforations treated initially by PNS alone were reviewed. RESULTS: The patients' median birth weight was 1030 g, with a median gestational age of 27 weeks. In 5 out of 6 infants (83 %), PNS achieved complete exsufflation without recurrence of the pneumoperitoneum and complete intestinal healing, allowing complete enteral feeding 30 to 71 days after perforation. One infant with recurrent pneumoperitoneum after 3 PNS and peritoneal drainage was operated. All infants survived. CONCLUSION: We believe that for early perforations of the premature neonate, the poor diffusion of the infection and the frequent capacity of the perforation to close and subsequently heal without scars, favour a minimally invasive management using PNS.