Laparoscopic Vascular Hitch for Polar Vessels in Pyeloureteric Junction Obstruction: Medium-Term Follow-up of a Monocentric Experience.

INTRODUCTION: To evaluate medium-term results of the management of patients with pyeloureteric junction obstruction (PUJO) secondary to lower pole vessels managed by a laparoscopic vascular hitch technique. MATERIALS AND METHODS: We performed a retrospective monocentric study of all patients who were operated using this technique, transperitoneal laparoscopy (TL) or robotic surgery (RS), from 2011 to 2018. Description of the initial population, pre- and postoperative data was collected and analyzed. RESULTS: During the study period, 25 patients (11 males and 14 females) with a mean age of 11.1 years were managed. Initial symptoms leading to the diagnosis were present in 21 children (lumbar pain 17, high blood pressure 1, and infection 3). Four patients were diagnosed incidentally (three) or following a prenatal screening (one). The diagnostic has been confirmed in all patients by ultrasound scan completed by computed tomography or magnetic resonance imaging scans. Twenty-one patients were operated by TL and four by RS. Mean operating time was 90.2 minutes. Three immediate postoperative complications were noticed including one urinary tract infection, one urinary tract rupture, and one postoperative massive dilatation. After a mean follow-up of 3 1.5 years, 17 patients remain asymptomatic. Mean pelvic diameter was preoperatively 35 mm and postoperatively 10.5 mm (p < 0.005). Of the eight remaining patients, six required reoperation (pyeloplasty) and two remained symptomatic with recurrent flank pain. CONCLUSION: In our experience, PUJO managed by the vascular hitch technique is associated with a 68% success rate after a 3-year follow-up. These results are different from the current literature which reported success rate of 96% after 52 months. It leads us to question the efficiency of this technique in the long term in our hands both concerning the indications and the surgical procedure itself. Further studies are required to collect data concerning the evolution of our patients during adulthood to compare their evolution to classic pyeloplasty.

Biological Glue as an Alternative to Suction Drainage in Tissue-Expanded Flaps in Children: Cost-Effectiveness Study.

INTRODUCTION: Biological glue is already used as a hemostatic agent and tissue adhesive in plastic surgery. This study evaluates the use of this glue as an alternative to suction drainage for the adhesion of tissue-expanded flaps in pediatric patients. METHODS: This is a retrospective, multicenter case-control study on 48 flap procedures conducted on 42 children (5 months-12 years of age) between 2004 and 2017, comparing a "glue" group (n = 24) with a control group (n = 24), in which a classic redon drain was used. The control patients were matched according to age, etiology, location of the lesion, and the size of the expander. The primary end point was the duration of hospital stay. RESULTS: The conditions were 24 cases of congenital nevus, 14 of cicatricial alopecia, and 10 of sebaceous hamartoma. Twenty-nine lesions were located on the scalp, 15 on the back, 2 on the thigh, and 2 on the buttocks.The average surgical durations (48 ± 24 vs 63 ± 32 minutes, P = 0.13) and average room occupancy time (126 ± 21 vs 139 ± 44 minutes, P = 0.29) were similar between the glue group and the control group. However, the average duration of hospital stay was lower in the glue group (1.5 ± 1.5 days) than in the control group (3.6 ± 1.3 days, P < 0.0001). The complication rates between the groups were similar. CONCLUSIONS: The application of glue on expanded flaps is as reliable as suction drainage with the advantage of reducing the duration of hospital stay and potentially enabling outpatient treatment for certain patients.

Wilms tumor, pleuropulmonary blastoma, and DICER1: case report and literature review.

BACKGROUND: Pleuroblastoma (PPB) is a rare pediatric tumor which, in 30% of cases, is associated with cystic nephroma. It has been recently linked to the DICER1 mutation as part of a predisposition syndrome for various tumors. However, if DICER 1 anomalies have been reported in patients with Wilms tumor (WT), to date, no cases of PPB, WT, and DICER1 mutations have been reported in the same patient. CASE PRESENTATION: We report the case of a 3-year-old patient, initially managed for metastatic WT. During his clinical course, the diagnosis of a PPB was made after detecting the DICER1 mutation and subsequent management was therefore modified. CONCLUSION: This case highlights that in case of simultaneous discovery of a renal tumor and a pulmonary lesion in a child, the DICER 1 mutations should be looked for as these could help adapt management and schedule the surgical procedures.

Non-operative Management for Uncomplicated Appendicitis: An Option to Consider.

AIM: The main goal of our study was to assess a 7 days long course of antibiotics for acute uncomplicated appendicitis. MATERIALS AND METHODS: From March 2014 to November 2015, all patients diagnosed with acute appendicitis have been considered to be treated by only antibiotics. Inclusion criteria included clinical (tenderness), biological (C-reactive protein [CRP] < 50), and radiological features (diameter > 6 mm). All patients were treated with intravenous amoxicillin and clavulanic acid (100 mg/kg/day) for 2 days (six doses). At the end of the treatment, clinical and paraclinical examinations included blood samples at day 7 and ultrasound (US) scan at 3 months. RESULTS: A total of 166 patients were treated and followed up prospectively during the study period. Mean age at diagnosis was 10.8 ± 0.6 years. All children, but four were discharged with a clinical improvement after 48 hours and six intravenous antibiotics injection according to our protocol. Four children required surgery during the initial hospitalization period.Initial ultrasound scan showed a mean diameter of 7.85 ± 1.6 mm, with inflamed fat in 124 patients (74.7%). At Day 7, the diameter was 5.2 ± 1.6 mm (p < 0.0001).During a median follow-up of 18.8 months (3.5-18), 22 patients (13.25%) had to be managed for a novel episode of acute appendicitis after a median period of 138 days (13-270). None had to be managed for a complicated appendicitis. CONCLUSION: Non-operative treatment (NOT) is a safe alternative for the management of uncomplicated acute appendicitis in children. Further study should be conducted to determine relapse risk factors.

Comparison between pediatric and adult adipose mesenchymal stromal cells.

BACKGROUND: Adipose-derived mesenchymalstromal cells (ASC) are currently tested in regenerative medicine to promote tissue reconstruction after injury. Regardingautologous purpose, the possible loss of therapeutic function and cell properties during aging have been questioned in adults. To date no reliable information is available concerning ASC from pediatric patients and a better knowledge is required for clinical applications. METHODS: Subcutaneous adipose tissue was collected from 27 donors (0-1 years old) and 50 donors (1-12 years old) and compared with adult ASC for in vitro characteristics. ASC were then tested in a mouse model of limb ischemia. RESULTS: Cells from the stromal vascular fraction (SVF) and subsequent cultured ASC were prepared. Only a greater amount in SVF cell number and ASC proliferative rate were found. Cell phenotype, colony formingunit-fibroblast (CFU-F) content, immunomodulation effect and adipogenic, osteoblastic and angiogenic potentials were not significantly different. In vivo, pediatric ASC induced an increase in microangiographic score in a mouse model of limb ischemia, even though improvement in vascular density was not significantly correlated to limb rescue. Finally messengerRNA (mRNA) analysis using a microarray approach identified that only 305 genes were differentially expressed (217 down- and 88 up-regulated) in pediatric versus adult ASC, confirming that ASC from both age groups shared very close intrinsic properties. CONCLUSION: This is the first study reporting a comparative analysis of ASC from a large number of donors and showing that their in vitro and in vivo properties were similar and maintained during aging.

Prenatal diagnosis of right-sided diaphragmatic hernia and ipsilateral intrathoracic kidney in a female fetus: a rare observation.

Congenital diaphragmatic hernia is a rare congenital malformation, as well as kidney ectopia. Among kidney ectopias, the intrathoracic one is the rarest. Those malformations concern more frequently boys, and affected more the left than the right side. Their association is poorly reported in the literature. We report the rare case of an early sonographic prenatal diagnosis of intrathoracic kidney at 22 weeks of gestation in a female fetus, completed on the follow-up by the diagnosis of an associated diaphragmatic hernia at 33 weeks of gestation. If chest mass is diagnosed prenatally or in neonate, ITK should be considered in a differential diagnosis, all the more if the ipsilateral renal fossa is empty. An associated DH should be searched if ITK is confirmed. Isolated ITK usually requires no specific treatment, in contrast with ITK associated with DH.

Non-contrast-enhanced MR angiography using time-spin labelling inversion pulse technique for detecting crossing renal vessels in children with symptomatic ureteropelvic junction obstruction: comparison with surgical findings.

OBJECTIVES: Investigate the feasibility and evaluate the accuracy of non-contrast-enhanced MR angiography (NC-MRA) using time-spin labelling inversion pulse (time-SLIP)to identify crossing renal vessels (CRVs) in children requiring surgical treatment of ureteropelvic junction (UPJ) obstructionand compare to laparoscopic findings. MATERIALS AND METHODS: Nineteen children ranging from 6 to 16 years of age underwent NC-MRA using the time-SLIP technique before surgery. Two independent readers analysed the MRA images. Number of renal arteries and presence or absence of CRVs were identified and compared with surgicalfindings. Image quality was assessed, as well as the presence of CRVs and measurement of renal pelvis diameter. Intra and inter-reader agreement was calculated using Cohen's kappa coefficient and Bland-Altman plots. RESULTS: The overall image quality was fair or good in 88% of cases. NC-MRA demonstrated CRVs at the level of the obstruction in 10 children and no CRV in 9 children. All were confirmed intra-operatively except in one of the nine children. Sensitivity, specificity, NPV, PPV for predicting CRVs were 92%, 100%, 100% and 87.5%, respectively, for both readers. CONCLUSION: NC-MRA is a good alternative to contrast-enhanced MRA and CT scanning for identifying CRVs in children with symptomatic UPJ. KEY POINTS: • Time-SLIP technique offers acceptable imaging quality for identifying crossing renal vessel. • Time-SLIP technique is easy to apply to the renal MRA examination. • Time-SLIP technique is an alternative to contrast-enhanced MRA and CT scanning.

Impact of obturation of palatal fistulae on speech quality and aerodynamic parameters in patients with cleft palates.

OBJECTIVES: The aim of this prospective study was to measure nasal and oral airflow during speech, before and after obturation. PATIENTS AND METHODS: Included were children aged 3-18 years with nonsyndromic clefts and palatal fistulae. The corpus used was: syllable /pi/; a sentence containing stop consonants and a nasal phoneme; and the description of a picture of a scene. Analysis criteria were: percentage of nasality; value of average flow for the explosion; perceived nasality and intelligibility; and tolerance of the proposed device. RESULTS: Only 5 children were included due to the observation of an increase in the percentage of nasality after obturation. The value of average flow for the explosion increased in all patients. A decrease in perceived nasality was noted in all but 1 patient. An improvement in intelligibility was observed in 3 out of the 5 children. The tolerance of the device was good. CONCLUSION: While the small number of patients studied does not permit firm conclusions concerning the efficiency of the obturation, the method described, as well as the introduction of 'speed of explosion' of stop consonants, offer new perspectives to prospectively study obturator effects on speech.

Conservative management of blunt pancreatic trauma in children: a single center experience.

INTRODUCTION: Blunt trauma of the pancreas represents a significant part of abdomen trauma in children with an incidence estimated at around 10%. If the conservative management is widely accepted concerning the stages I and II, it remains controversial concerning stages III and IV. The aim of our study was to perform a descriptive analysis of the nonoperative management, with a focus on the occurrence of pseudocysts. MATERIALS AND METHODS: The charts of the patients treated in our center for pancreatic trauma from 1990 to 2010 have been reviewed. It was defined by an initial lipase greater than three times the norm and an abnormal computed tomography scan. RESULTS: A total of 36 patients were included, with 26 boys (72%) and 10 girls (28%) with an average age of 8.7 years. The trauma was isolated in 13 cases (36.1%) and in 23 cases, there were other associated lesions (mainly liver [n = 9] and spleen [n = 5]). Pancreatic injuries were graded as follows: I (n = 21), II (n = 2), III (n = 7), and IV (n = 6). Pseudocysts occurred in 11 patients (30.5%) mainly in grades III (n = 3) and IV (n = 7), with an average delay of 17 days. Initial management of pseudocysts was conservative in six patients (54.6%), whereas five patients required mini-invasive procedures. CONCLUSION: Nonoperative management remains a safe way to treat pancreatic injuries despite an average 30% rate of pseudocyst (PC) appearance. It allows a reduction in the number of children who required procedures to less than half of the patients where PC occurred. Furthermore, these procedures were exclusively mini-invasive.

Mechanical occlusion of the inferior vena cava: an early complication after repair of pectus excavatum using the Nuss procedure.

The Nuss procedure is the most widely used surgical procedure to correct pectus excavatum. Although it is a minimally invasive approach, a number of major early complications, such as heart perforation, have been reported. We describe a 15-year-old boy in whom acute occlusion of the inferior vena cava developed after a Nuss repair. The diagnosis was confirmed by emergency postoperative CT examination, and treatment consisted of immediate removal of the Nuss bar.

Pulmonary and intestinal congenital anomalies masquerading as cystic suprarenal masses.

OBJECTIVES: The prenatal finding of a cystic suprarenal mass (CSM) presents a wide differential diagnosis. The aim of this study was to present the natural course and outcome of antenatally diagnosed CSMs. METHODS: We reviewed the medical records of patients with prenatally detected CSMs that were assessed between January 1998 and December 2011. Retrospective data collection included the age at the time of diagnosis, the size of the mass, and the sonographic evolution of the mass. Surgical treatment was indicated in cases of malignant tumors and symptoms or when size increased. RESULTS: The observation period ranged from 1 month to 10 years. The data of 21 patients were analyzed. The median length of follow-up was 32 months (ranging from 2 to 131 months). A total of 13 masses were detected on the left side and 8 on the right side. In 13 patients, the lesions disappeared after a median of 7 months (ranging from 0 to 37 months). Surgery was performed in two neonates: one for a teratoma at 10 days postpartum, and one for a neuroblastoma at 17 days postpartum. Six patients had an extralobar pulmonary sequestration (ELPS), and in four patients, surgical resection was performed at an average of 9.2 months (ranging from 1 to 20 months) postpartum, because of an infection or increase in ELPS size. Histological examination confirmed the pulmonary sequestration. An association with congenital cystic adenomatoid malformation (CCAM) was found in one patient, and ectopic pancreatic tissue was discovered in another patient. Two patients conservatively managed remain under observation. CONCLUSIONS: During the neonatal period, the accurate assessment of CSMs is necessary to exclude the presence of malignant tumors. Most patients can be conservatively managed, with close follow-up, including radiological assessment. If the mass persists after 1 year of age, ELPS should be considered as a probable diagnosis, and minimally invasive laparoscopic surgery can be performed, as it provides both definitive diagnosis and treatment.

Unusual duplicate bladder exstrophy in a female newborn: a case report.

The authors report a rare variant of exstrophy-epispadias complex, a duplicate bladder with normal bladder communicating with an exstrophic bladder by a fistula, in a girl with no genital malformation except for a duplicated clitoris. This variant could be a hybrid form of duplicate bladder exstrophy and superior vesical fistula. It seems easier to repair and has a better prognosis than classic bladder exstrophy.

Complex communicating bronchopulmonary foregut malformation with pancreatic heterotopy depicted with fetal magnetic resonance imaging: a case report.

Bronchopulmonary foregut malformation (BPFM) is a group of rare congenital anomalies that affect the respiratory and upper gastrointestinal tracts. We report here the first case of prenatal depiction of communicating BPFM, including extrapulmonary sequestration and foregut cyst, both with pancreatic differentiation. Magnetic resonance imaging on the fetus confirmed a polymalformative syndrome with right-lung and gastric cysts and allowed the detection of respiratory and alimentary tracts communication and aberrant feeding vessels. Diagnosis was confirmed after birth by multidetector computed tomography and postnatal pathologic findings. The patient, a boy, underwent surgery for respiratory distress at 4 weeks of life. This singular case confirms the benefits of early surgery for communicating BPFM.

Branchi-oculo-facial syndrome: a case report to highlight recent genetic considerations.

Branchio-oculo-facial syndrome (BOFS) is a rare entity described during the last century which has been recently linked to mutations of the gene encoding for the transcription factor named 'TFAPA2'. We report here a sporadic case of BOFS with a partial phenotype caused by a de novo mutation of this gene and discuss recent genetic findings.

The surgical management and outcome of congenital mediastinal malformations.

We reviewed our institutional experience with congenital mediastinal masses and compared the postnatal management and outcome of patients with or without prenatal diagnosis. Between January 1997 and August 2011, 24 patients underwent surgical procedures for congenital mediastinal mass. For eight patients, the mass was detected by prenatal ultrasonography at 27 weeks of gestation (range 22-35). Postnatal management consisted in open surgery for seven patients at a mean age of 9 months (range 1 day-20 months) and sclerotherapy for one lymphangioma at 5 months of life. Sixteen patients had postnatal diagnosis at 137 months (±194) of median age. Eight bronchogenic cysts, seven bronchopulmonary foregut malformations, five teratomas, three lymphangiomas and one haemangioma were operated on. The median age at resection was 28 months (1 day-15 years). There were four emergency procedures and no surgical mortality. The median follow-up was 45 months (3-144). The duration of mechanical ventilation and hospital stay was, respectively, 4.6 h and 7.5 days for antenatal patients and 24.3 h and 14.3 days for postnatal diagnosed patients. Prenatal diagnosis allows early management of congenital mediastinal malformations. Early resection can be performed prior to the occurrence of symptoms ∼1 year of life and is associated with an excellent outcome and less morbidity.

Ovarian germ cell tumors in children. Management, survival and ovarian prognosis. A report of 75 cases.

BACKGROUND/PURPOSE: The aims of this study were to evaluate survival and ovarian prognosis in patients treated for ovarian germ cell tumor (OGCT) and to propose a decision-making protocol. METHODS: Charts of girls operated on for OGCT from 1976 up to 2009 were reviewed retrospectively. Tumor characteristics were assessed by tumor markers, imaging, and pathology. RESULTS: Charts were available in 71 children presenting 75 OGCT. Tumors were benign in 58 cases and malignant in 17 cases. The average of the largest diameter of benign OGCT was significantly lower than that of malignant OGCT (76.5 +/- 49 mm versus 169 +/- 54 mm, P < .0001). Ovarian-sparing tumorectomy was carried out in 27 benign OGCT; 23 (85%) preserved ovaries were follicular. Malignant OGCTs were managed according to the protocols of the French Society for Pediatric Oncology. Bilateral oophorectomy had to be performed in 2 children. One patient presented a recurrence and 1 died. CONCLUSIONS: In our series, both benign and malignant OGCTs have a good prognosis. A 75-mm cutoff size is proposed as an important criterion to preoperatively differentiate between benign and malignant tumors. In benign OGCT, ovarian-sparing tumorectomy leads to preserve ovaries in approximately 85% of cases, and in malignant OGCT, high survival rate has been obtained.

Ovarian torsion. Management and ovarian prognosis: a report of 45 cases.

BACKGROUND/PURPOSE: Ovarian torsion in childhood and adolescence is a rare entity. Traditionally, treatment is oophorectomy. The aim of this study was to evaluate ovarian outcome and to propose a decision-making protocol for suspected ovarian torsion. METHODS: Between January 1986 and December 2007, 45 ovarian torsion cases in 40 girls were operated on. In all the cases, when the ovary was preserved, patients were clinically and ultrasonographically followed up for several months. RESULTS: Median age was 11 years. Median delay between the first symptoms and surgical procedure was 3 days. There was a statistical difference (P = .0003) between the mean of the largest diameter of twisted normal ovary and the mean of the largest diameter of twisted diseased ovary. Underlying pathology was benign in 22 cases and low-grade malignancy in 2 (one grade II immature teratoma and one steroid cell tumor). Conservative management was performed in 26 cases. At follow-up, 17 ovaries were follicular, 7 being black-bluish during surgery. CONCLUSIONS: Conservative approach after detorsion of black-bluish ovaries is safe and effective in children. Although very unlikely, the fear of missing malignancy must incite to proceed with caution and can lead, when the size of the twisted ovary is greater than 75 mm, to prefer laparotomy to laparoscopy.