Transient pseudohypoaldosteronism: a potentially severe condition affecting infants with urinary tract malformation.

BACKGROUND: Secondary pseudohypoaldosteronism (S-PHA) is a life-threatening condition affecting young children with urinary tract malformation (UTM). OBJECTIVE: The aim of the study was to highlight the diagnosis of S-PHA in children with UTM and propose appropriate management. STUDY DESIGN: The authors retrospectively reviewed cases of S-PHA related to UTM observed at the institution and searched the PubMed® database to review the literature. RESULTS: A total of 116 cases of S-PHA associated with UTM, including the four cases from the institution, were reviewed. One hundred six cases (92.2%) were younger than 6 months, and 95 cases (81.9%) occurred in boys. Urinary tract infection was associated in 105 cases (90.5%). All types of UTM were observed. In the absence of urinary tract infection, S-PHA was related to bilateral UTM or solitary kidney. In 89 cases (76.5%), S-PHA resolved with medical treatment only. In cases of UTM requiring immediate surgery, electrolyte imbalance related to S-PHA also resolved after surgery. Children with associated urinary tract infection and bilateral UTM are at higher risk of developing S-PHA. DISCUSSION: The pathogenesis of S-PHA has not been fully elucidated. Renal tubular immaturity may be one of the factors involved, in view of the young age of the population being affected. A high rate of bilateral UTM (or UTM on solitary kidney) was observed (50.9%), suggesting an association with S-PHA. In the absence of urinary tract infection (UTI), S-PHA appeared to occur more frequently in the presence of bilateral UTM. Although the indication for early surgery remains unclear, it may have a role in the prevention of UTI and prevention of recurrence of S-PHA. Serum electrolytes should be checked in children with UTM before urological surgery, and/or presenting urinary tract infection, before the age of 6 months. The results of this study must be interpreted cautiously because of its retrospective nature and the fact that data were derived from various articles. Few articles on S-PHA related to UTM have been published in the literature. To the best of the authors' knowledge, the study constitutes the largest series published to date. CONCLUSIONS: S-PHA results in potentially severe electrolyte imbalance and affects children younger than 6 months with UTI and/or UTM. Electrolyte abnormalities related to S-PHA often resolve after administration of appropriate intravenous electrolyte solution and treatment of UTI and/or surgery.

[Isolated gallbladder agenesis mimicking biliary colic: A difficult preoperative diagnosis]. / Coliques hépatiques révélant une agénésie de la vésicule biliaire : un diagnostic préopératoire difficile.

Isolated gallbladder agenesis is a very rare and unrecognized congenital anomaly. Patients are usually asymptomatic, but 23% present with symptoms suggestive of biliary colic. Ultrasound investigation often fails to diagnose this malformation, misinterpreted as scleroatrophic gallbladder, leading to unnecessary and potentially dangerous surgery. We report on a case of a 9-year-old child who complained of biliary colic. Ultrasound showed a possible scleroatrophic gallbladder. This diagnosis was in doubt, however, because the patient had no previous history of cholecystitis. Finally, magnetic resonance cholangiopancreatography failed to show any gallbladder. The absence of the visualization of the gallbladder in a context of right upper quadrant pain should suggest gallbladder agenesis. Pain can be explained by the so-called postcholecystectomy syndrome.

[Giant mesocolic cystic lymphangioma: A treacherous prenatal presentation. Case report]. / Lymphangiome kystique géant du mésocôlon : un diagnostic anténatal difficile. À propos d'un cas.

Cystic lymphangiomas are usually located in the neck region. Less frequently, they can be found in the abdomen. In those cases, pre- and neonatal diagnosis is extremely difficult. We report on the case of a giant mesocolic cystic lymphangioma, diagnosed at birth, in a child who had been monitored during the prenatal period for what was believed to be a digestive dilatation. The progression was marked by excellent tolerance despite a complete lack of regression in the first 10 months of life. The authors discuss the prenatal signs that should suggest this diagnosis and an MRI, as well as management during the 1st year of life.

[Congenital mesenteric band simulating a small-bowel volvulus in a mesenteric rotation anomaly]. / Bride mésentérique congénitale simulant un volvulus de l'intestin grêle sur anomalie de rotation mésentérique.

A congenital mesenteric band is a rare cause of intestinal obstruction. We describe a case of upper gastrointestinal obstruction on a jejunal congenital band in a 2-year-old child. The challenge is to make the diagnosis in a patient with no history of previous surgery.

[Complete resolution of inflammatory myofibroblastic tumor of the bladder after antibiotic therapy]. / Régression complète d'une tumeur myofibroblastique inflammatoire de vessie après antibiothérapie.

Inflammatory myofibroblastic tumors (IMT) are rare benign tumors, most commonly arising in the lungs and urinary bladder. Many etiologic factors are suspected in their development, but none have been formally demonstrated. Conventional treatment for bladder IMT is complete surgical resection by partial cystectomy or transurethral resection. We report the case of an 8-year-old girl with documented bladder IMT that resolved completely after antibiotic therapy.

[Chronic intestinal pseudo-obstruction complicated by an eating disorder]. / Pseudo-obstruction intestinale chronique compliquée d'un trouble des conduites alimentaires chez une adolescente.

Chronic idiopathic intestinal pseudo-obstruction is a rare and serious chronic disease starting in childhood, which can affect the entire digestive tract. It is caused by a peristalsis intestinal disorder that leads to occlusions without any obvious obstruction. Few studies have been carried out regarding the prognosis of this illness. This disease is often diagnosed by a process of elimination, but some histological anomalies have been present in the digestive wall of certain patients. This clinical case concerns a 17-year-old girl affected by CIPO and eating disorders. It seems difficult to discriminate between digestive disorders and eating disorders. What psychological effects can this severe pathology have? Are eating disorders induced by CIPO? These questions are raised in this article through the example of this patient's somatopsychic complexity and the ensuing difficulties in her overall care.

[A bulky lateral cervical mass]. / Une volumineuse tuméfaction cervicale latérale.

Branchial cysts are rare, benign cervical lesions that can mimic thyroid goiters, in particular in areas where goiters are endemic. This case describes an Ivorian patient who presented with what appeared to be a voluminous thyroid goiter. At surgery, it proved to be a cyst of the second pharyngeal arch.

[Does prenatal diagnosis modify neonatal management and early outcome of children with esophageal atresia type III?]. / Le diagnostic anténatal modifie-t-il la prise en charge néonatale et le devenir à 1 an des enfants suivis pour atrésie de l'œsophage de type III ?

OBJECTIVE: Evaluate neonatal management and outcome of neonates with either a prenatal or a post-natal diagnosis of EA type III. STUDY DESIGN: Population-based study using data from the French National Register for EA from 2008 to 2010. We compared children with prenatal versus post-natal diagnosis in regards to prenatal, maternal and neonatal characteristics. We define a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and mortality at 1 year. RESULTS: Four hundred and eight live births with EA type III were recorded with a prenatal diagnosis rate of 18.1%. Transfer after birth was lower in prenatal subset (32.4% versus 81.5%, P<0.001). Delay between birth and first intervention was not significantly different. Defect size (2cm vs 1.4cm, P<0.001), gastrostomy (21.6% versus 8.7%, P<0.001) and length in neonatal unit care were higher in prenatal subset (47.9 days versus 33.6 days, P<0.001). The composite variables were higher in prenatal diagnosis subset (38.7% vs 26.1%, P=0.044). CONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity related to the EA type (longer gap). Even if it does not modify neonatal management and 1-year outcome, prenatal diagnosis allows antenatal parental counseling and avoids post-natal transfer.

Actifit synthetic meniscal substitute: experience with 18 patients in Brest, France.

BACKGROUND: The management of post-meniscectomy pain is poorly standardised. Allogeneic transplantation may be appropriate in some patients after total meniscectomy. After partial meniscectomy, the synthetic meniscal substitute Actifit may constitute a valid option if the knee is stable or stabilised and aligned or re-aligned. The interconnected pore structure of Actifit promotes tissue regeneration from the meniscal wall. Arthroscopy is used to position the implant, which is then sutured to the remaining native meniscus using horizontal stitches and to the meniscal wall using vertical stitches. However, a burdensome programme of rigorous rehabilitation is required after Actifit implantation. HYPOTHESIS: We hypothesised that implantation of a meniscal substitute effectively alleviated pain without adversely affecting the knee. OBJECTIVES: To assess the intra-articular behaviour of Actifit and the outcomes of Actifit implantation in a prospective case-series of patients monitored using arthroscopy, pathology, and imaging studies, as well as the Lysholm score to assess clinical benefits on daily activities. MATERIALS AND METHODS: Between October 2009 and April 2012, 18 patients underwent Actifit implantation at the military hospital in Brest, France. All procedures were performed by the same surgeon, who had extensive experience with meniscal suturing. There were 13 males and 5 females aged 20 to 46 years. The medial meniscus was involved in 13 patients and the lateral meniscus in 5 patients. Actifit implantation was used alone in 6 patients and in combination with anterior cruciate ligament reconstruction and/or realignment osteotomy in 12 patients. All patients were followed-up for at least 2 years. RESULTS: The mean Lysholm score after 1 year was 92%, indicating excellent outcomes. Magnetic resonance imaging showed no damage to the implant or degeneration of the neighbouring cartilage. Histological examination of meniscal substitute biopsies taken 1 year after implantation showed polymer ingrowth by normal chondrocytes and fibrochondrocytes. The clinical and radiographic outcomes compared favourably with those seen after isolated procedures on bone or ligaments. DISCUSSION: Actifit has no deleterious effects on patients. The implant induces and promotes meniscal regeneration. Actifit constitutes a major addition to our therapeutic armamentarium. We provide convincing evidence that meniscal reconstruction can be highly beneficial in decreasing the risk of progression to knee osteoarthritis. LEVEL OF EVIDENCE: IV.

Successful early surgical treatment in neonatal compartment syndrome: case report.

Neonatal compartment syndrome is rare, and the diagnosis is often missed or delayed because other ischemic diseases can mimic clinical signs observed on the skin. A premature newborn infant presented with skin lesions during the first hours of life that were recognized as the sentinel finding in compartment syndrome of the newborn. We restored normal function by emergency surgery. The authors highlight the importance of effective collaboration between pediatricians and surgeons to improve the management of this neonatal condition.

[Congenital mesoblastic nephroma: diagnosis and treatment of one case]. / Le néphrome mésoblastique congénital : diagnostic et prise en charge à partir d'un cas.

The congenital mesoblastic nephroma (CMN) described by Bolande et al. in 1967 is a renal tumor often discovered in neonatal period and early childhood. It's usually considered as a benign tumor with good prognostic for which nephrectomy is the reference treatment. But some cases of local recidives and metastatic sites had been described in the literature. For these reasons histologic analysis and quality of follow up are very important. In this observation we describe a neonatal kind of CMN and we discuss this pathology.

Painful medial knee compartment syndrome in over-45 year-olds: I--medical or surgical management: a series of 174 patients.

INTRODUCTION: There is at present no consensus on the management of degenerative medial meniscus lesions in patients aged over 45 years without proven osteoarthritis, especially given that the causal relation between degenerative meniscal lesion and osteoarthritis remains controversial. A prospective multicenter non randomized study was therefore performed. The principal objective was to assess surgeons' practice in the management of degenerative medial meniscus lesions. The secondary objectives were to identify predictive and prognostic factors and to compare medical versus surgical attitudes so as to draw up an adapted treatment strategy. PATIENTS AND METHOD: One hundred and seventy-four patients were included between September 2008 and February 2010, and distributed between a surgical (n=104) and a medical group (n=70). Minimum follow-up was 6 months. Patient satisfaction and health-related quality of life on the SF-36 questionnaire were assessed at 6 months. RESULTS: No difference emerged between the surgical and medical groups. However, predictive factors for poor results were identified: overweight (p=0.005), cartilage lesions (p=0.035) and meniscus extrusion (p=0.006). DISCUSSION: Results clarified the relation between degenerative meniscus lesions and osteoarthritis, in terms of meniscal incompetence. Meniscal extrusion should be seen as an arthrogenic degenerative meniscus lesion. We recommend a management strategy based on terrain and imaging data (X-ray and MRI), with the aim of providing patient relief while conserving cartilage.

Painful medial knee compartment syndrome in over-45-year-olds. II--technical note: Biomechanical considerations: theoretic analysis of load distribution in the knee according to plane and to gait phase.

Varus deviation as defined by Thomine is the basis for understanding load distribution between the medial and lateral tibial plateau; it was originally defined in the frontal plane. Analysis in the sagittal and horizontal planes leads to a concept of varizing area. Varizing area sheds light on how, during gait, load predominates in the medial and posteromedial knee. It also accounts for the following two findings: (a) degenerative lesions are mainly medial, even when the mid-knee axis is normal; (b) degenerative lesions are mainly posterior.

[Cystinuria in children: A report of 4 cases]. / Cystinurie chez l'enfant : à propos de 4 observations.

Cystinuria is an inherited autosomal-recessive disorder of renal reabsorption of the dibasic amino acids. It is the cause of about 6% of all kidney stones observed in children. Cystine is relatively insoluble at the physiological pH of urine. Cystine stones are characteristic and frequent recurrences are observed. We report on 4 cases and describe the initial presentation (obstructive renal failure, urinary sepsis, familial screening) and the medical and surgical management. Medical management is mainly based on hyperhydration and urine alkalinization. Long-term therapy with sulfhydryl agents to prevent formation of renal stones seems to be effective but adverse side effects are frequent, requiring the withdrawal of treatment. Urological management has evolved from surgical stone removal to minimally invasive procedures (extracorporeal shock wave lithotripsy, ureteroscopy).

Impact of colostomy on intestinal microflora and bacterial translocation in young rats fed with heat-killed Lactobacillus acidophilus strain LB.

A rat animal model of left colostomy was found to significantly impair the growth curve of rats. Assessment of the intestinal flora showed that colostomy mostly affects the cecal but not colonic microflora. Generally, the number of enterococci was increased in both ileum and cecum; cecal lactobacilli also rose, accounting for a promotion of lactic acid bacteria in colostomised rats. No significant differences between colostomised, laparotomised and control rats could be observed for the translocation of intestinal bacteria to internal organs of rats (i.e. spleen, kidneys, lungs or liver), whatever their diet. Heat-killed Lactobacillus acidophilus strain LB administration (dead probiotic bacteria) tended to exhibit a stimulatory effect on bifidobacteria, probably affecting the culture-medium fermentation substances included in the pharmaceutical product. This effect was abolished by laparotomy and colostomy. A trend towards a probiotic-like effect, not susceptible to colostomy, was also witnessed as counts of lactobacilli tended to increase in both cecum and colon of all animals fed with L. acidophilus LB.

[Urodynamic investigations in children]. / Place des investigations urodynamiques chez l'enfant.

Performing urodynamic investigations in children presents some difficulty due to the lack of any normogram, and due to the results that vary with age. Such investigation is therefore carried out only when clinical examination and radiological assessment fail to explain a voiding dysfunction. The procedure should be performed in a urodynamic unit that has paediatric expertise. A specific paediatric procedure is to be respected when performing uroflowmetry and cystometry in children. Assessing the urethral pressure profile is very difficult since moving a catheter along the urethra causes a reflex activity of the pelvic floor muscles. Main indications are: neuropathic bladders, voiding dysfunctions, urinary infections, anorectal malformations and pelvic tumours. As in adults, urodynamic investigations are useful when selecting a therapeutic strategy.

[Laparoscopic surgery in children]. / Chirurgie coelioscopique chez l'enfant.

In the last two decades, laparoscopy surgery has been progressively adopted to children. Cardiorespiratory changes induced have been understood and controlled. Abdominal and urological surgery have widely benefited from this technique. Immediate postoperative period is simpler. The risk of small bowel obstruction by bands and adhesions is limited. Nevertheless, laparoscopy is not indicated for all pathologies. In neonatal surgery, more studies are necessary. Maybe the future is robotic surgery.

[Outcome at 2 years of very premature infants cared at the hospital of Cayenne in 1998]. / Devenir médicosocial à l'âge de 2 ans des grands prématurés pris en charge au centre hospitalier de Cayenne en 1998.

UNLABELLED: Perinatal mortality in French Guyana is twice as high as the national rates. Within a global project rallying all the people working around the birth, a catching up program benefited the neonatal unit of the Cayenne hospital in order to make up for the important lack of modern structures and means. OBJECTIVES: To establish some quality care for the less than 33 weeks preterm infants born in 1998, and to estimate the medical and familial future for these infants after they have reached 2 years of age. METHODS: All less than 33 weeks old infants born alive and admitted at the neonatology unit of the CHC were included in the study. The obstetrical and neonatal data were recorded from the medical files. A survey was conducted through a questionnaire including the familial and medical outcome and was led from december 1999 to july 2001. Results were analysed with Epi-info 6.0 software (CDC, Atlanta, USA). RESULTS: Eighty-eight infants issue from 78 pregnancies were included. Among them, 29 were transferred in utero and 12 were transferred after birth. The mothers' situations were mainly characterized by precariousness, a poor follow-up of the pregnancy (27% non-declared), a higher morbidity of their newborns. Neonatal results did not differ from national results from Epipage cohort in terms of gestational age (29.9 weeks), weight at birth (1411 g), mortality (90.9 alive when released from the hospital), morbidity, growth at 2. However, 26% were hypotrophic (15% in Epipage cohort) in relation with the 29% pregnancies complicated with hypertension. Four infants were suffering from acquisition retardation due to motor handicaps; 2 infants had severe socio-educative difficulties. The postnatal follow-up was mainly performed by the Mother and Children Health Centers. A third of medical exams were not recorded in the health book, sensory screening exams were not performed. The opening of a medicosocial actions center in 2001, will allow a prospective follow-up of this population. CONCLUSION: Early neonatal mortality decreased from 10.3 for 1000 births to 4.4 in 1998. This reflects the improvement of the new-born caring possibilities. However, an improvement of mortality rates will not be possible without a better pregnancy follow-up.

[Change in management of ureteropelvic junction obstruction]. / Evolution de la prise en charge du syndrome de la jonction pyélo-urétérale.

PURPOSE: The aim of this study was to evaluate the change in ureteropelvic junction management, from surgical correction to observation of the hydronephrosis. We checked wether or not it was deleterious for kidney. MATERIALS AND METHODS: We retrospectively reviewed 96 charts between 1988 and 1998. Initial ultrasonography, and voiding cystourethrogram were available for all cases. Intravenous urography and diuretic renography were studied when available. Minimal follow-up of patients was one year. Patients were divided into three groups: surgery right away, surgery after observation, and observation only. RESULTS: Later was the diagnosis, more significant were the hydronephrosis and impairment of renal function (p < 0.01). In group operated on right away (69 cases), drainage improved, hydronephrosis decreased, but renal function did not improve significantly (p = 0.37). Sixteen patients were operated on after observation without deleterious effect for kidney. Eleven patients were only observed: hydronephrosis progressively decreased. Initial radiological results were not different between the last two groups (p > 0.05), and were not predictive of their course. CONCLUSION: Initial non operative management of hydronephrosis was not dangerous for renal function. It is advisable to detect at the earliest all signs of obstruction, because surgery improves renal drainage but not renal function.