Jean Stephane Valla

Laparoscopic renal surgery via a retroperitoneal approach in children

PURPOSE Laparoscopic procedures are still not widely performed by pediatric urologists due to operative time and costs as well as the lack of indications and, thus, the lack of sufficient surgeon experience. We report our experience with the retroperitoneal approach using special inexpensive methods for various indications in children. MATERIALS AND METHODS From 1994 to 1997 we performed 42 retroperitoneal laparoscopic procedures in 41 children with a mean age of 4 years, including 31 nephrectomies, 8 partial nephrectomies, 2 renal cystectomies and 1 pyelolithotomy. A total of 13 patients were younger than 1 year. Followup ranged from 6 months to 3 years. RESULTS Mean operative time was 104 minutes (range 35 to 150) for nephrectomy, 153 (range 90 to 210) for upper pole nephroureterectomy, 135 for pyelolithotomy and 60 for renal cystectomy. Average postoperative stay was 2 days. Conversion was required in 2 cases of partial nephrectomy due to unidentified polar vessels, including 1 involving duodenal perforation. CONCLUSIONS The indications for retroperitoneal laparoscopy are expanding with the experience of the surgical team. The retroperitoneal approach provides a technique comparable to that of conventional renal surgery.

Laparoscopic versus open appendectomy in children : A retrospective comparative study of 2,332 cases

AimThe laparoscopic treatment of paediatric appendicitis remains controversial, especially in the presence of complications. This study evaluated the outcomes of open appendectomy (OA) and laparoscopic appendectomy (LA) by analysing the data from a multicentre study.MethodsThe authors retrospectively reviewed a series of 2,332 appendectomies (1,506 LA and 826 OA) performed in children and adolescents (median age 8 years) in 9 different centres of paediatric surgery. For the patients operated using laparoscopy, an IN procedure was employed in 921 (61.2%), an OUT procedure in 571 (37.9%) and a MIXED procedure in 14 (0.9%). In the open surgery, a McBurney incision was adopted in 795 patients (96.4%).ResultsMedian duration of surgery was 40 minutes for LA and 45 minutes for OA. Median hospital stay was 3 days (LA) and 4.3 days (OA) in case of simple appendicitis and 5.2 days (LA) and 8.3 days (OA) in case of peritonitis. Complications were recorded in 124 LA cases (8.2%) and 65 OA cases (7.9%). The conversion rate in laparoscopy was only 1.6% (25 cases). The statistical analysis was performed using the Mann–Whitney test, and the main significant difference that emerged was the length of hospital stay, which was in favour of laparoscopy compared with open surgery (P < 0.0001).ConclusionsWe conclude that in clinical settings where laparoscopic surgical expertise and equipment are available and affordable, LA seems to be an effective and safe alternative to OA. Three out 9 centres participating in our survey perform LA in all patients with a suspicion of appendicitis. Our study shows that laparoscopy significantly reduces hospital stay in case of appendicitis and peritonitis and presents an extremely low conversion rate (1.6%) to open surgery. Laparoscopic transumbilical appendectomy (37.9%) in our series seems to be a simple option, even for less-skilled laparoscopic surgeons.

Thirteen cases of isolated tubal torsions associated with hydrosalpinx in children and adolescents, proposal for conservative management: retrospective review and literature survey

BACKGROUND/PURPOSE Isolated tubal torsion associated with hydrosalpinx is a rare pathology. Our goal was to analyze the clinical and imaging features and discuss the different treatment options available. METHODS We retrospectively reviewed all the cases of adnexal torsion treated in our department of pediatric surgery over a 10-year period. We searched 2 electronic databases (Medline and Sciencedirect) and targeted reports published during the same period using the key words tubal torsion and hydrosalpinx. RESULTS A total of 13 cases, 6 from our hospital and 7 in the medical literature, were identified and analyzed. In 9 (69%) of 13 cases (n = 9/13), torsion and hydrosalpinx occurred on the left fallopian tube. Salpingectomy was performed in 11 of the patients. The resected tubes showed the persistence of ciliated cells associated with signs of moderate ischemic infarction in 50% (n = 3/6) of the cases. CONCLUSIONS Isolated tubal torsion associated with hydrosalpinx is too often misdiagnosed and treated by salpingectomy regardless of the negative impact on the future reproductive potential of our young patients. As is commonly advocated for ovarian salvage in adnexal torsions, tubal conservation should be favored when possible.

Minor Hypospadias: The “Tip of the Iceberg” of the Partial Androgen Insensitivity Syndrome

Background Androgens are critical in male external genital development. Alterations in the androgen sensitivity pathway have been identified in severely undermasculinized boys, and mutations of the androgen receptor gene (AR) are usually found in partial or complete androgen insensitivity syndrome (AIS). Objective The aim of this study was to determine whether even the most minor forms of isolated hypospadias are associated with AR mutations and thus whether all types of hypospadias warrant molecular analysis of the AR. Materials and Methods Two hundred and ninety-two Caucasian children presenting with isolated hypospadias without micropenis or cryptorchidism and 345 controls were included prospectively. Mutational analysis of the AR through direct sequencing (exons 1–8) was performed. In silico and luciferase functional assays were performed for unreported variants. Results Five missense mutations of the AR were identified in 9 patients with glandular or penile anterior (n = 5), penile midshaft (n = 2) and penile posterior (n = 2) hypospadias, i.e., 3%: p.Q58L (c.173A>T), 4 cases of p.P392S (c.1174C>T), 2 cases of p.A475V (c.1424C>T), p.D551H (c.1651G>C) and p.Q799E (c.2395C>G). None of these mutations was present in the control group. One mutation has never been reported to date (p.D551H). It was predicted to be damaging based on 6 in silico models, and in vitro functional studies confirmed the lowered transactivation function of the mutated protein. Three mutations have never been reported in patients with genital malformation but only in isolated infertility: p.Q58L, p.P392S, and p.A475V. It is notable that micropenis, a cardinal sign of AIS, was not present in any patient. Conclusion AR mutations may play a role in the cause of isolated hypospadias, even in the most minor forms. Identification of this underlying genetic alteration may be important for proper diagnosis and longer follow-up is necessary to find out if the mutations cause differences in sexual function and fertility later in life.

Adrenal masses in children: the role of minimally invasive surgery.

The development of laparoscopic surgery has extended its uses to include adrenalectomy in children and in adults. Because conventional adrenalectomy requires a large incision, minimally invasive surgery offers a less aggressive solution in some selected cases. Twenty-nine adrenal masses in 26 children were treated using adrenalectomy between 1994 and 2004 (12 were treated laparoscopically, the remaining 17 with open surgery). Minimally invasive procedures were limited to the removal of small localized adrenal tumors and to biopsies. Although this approach must be limited to operations on lesions presumed to be benign, preoperative criteria for nonmalignancy are often difficult to define. Indications can be expanded to include to stage I neuroblastoma. There seems to be no age and weight limit. The technique applied varies in accordance with anatomy and the surgeons experience: minimally invasive adrenalectomy, in our experience, was preferentially performed through a lateral retroperitoneal approach. Laparoscopic adrenalectomy can be used if the selection of cases is rigorous and the operations are performed by well-trained laparoscopic surgeons.

Thoracoscopic Thymectomy in Children: Our Preliminary Experience

INTRODUCTION The aim of this study is to present a preliminary series of six children affected by an anterior mediastinal mass, resulting in a thymoma. We treated this pediatric population by a video-assisted thoracoscopic thymectomy. PATIENTS AND METHODS We performed a retrospective study from January 2000 to January 2012 of all children affected by an anterior mediastinal mass, resulting in a thymoma. Data included sex, age at surgery, clinical and radiological features, surgical procedure, follow-up, and complications. RESULTS Video-assisted thoracoscopic thymectomy was performed in six children (five boys and one girl). Four patients presented with dyspnea and/or thoracic pain treated with analgesic and corticosteroid therapy without any benefits. Two patients were asymptomatic. Preoperatively, exams included radiological imaging, blood, and immunological test. All 6 patients were treated with a thoracoscopic approach; of these patients, 5 were treated with a thoracoscopic right-side thymectomy and 1 with a left-side approach. Patients were treated by a three-trocar technique, and thymectomy was performed using Valleylab (now Covidien) (Boulder, CO) LigaSure™. The mean operative time was 120 minutes. The mean hospital stay was 5 days. In all cases histological findings revealed no malignancy. The mean follow-up was 38 months. DISCUSSION Thoracoscopic thymectomy facilitated the goal of early thymectomy. The right-side approach is preferred because it gains a good visualization of the venous anatomy for dissection. The left side allows a good extended resection of both the thymus and perithymic fat. Thoracoscopic thymectomy should be consider a valid, less invasive alternative to the most radical open approach.

Retroperitoneoscopic Heminephrectomy in Duplex Kidney in Infants and Children: Results of a Multicentric Survey

PURPOSE Retroperitoneoscopic partial nephrectomy (RPN) in children is considered a complex technique with limited diffusion among pediatric surgeons and urologists. We aimed to report the outcome of this technique in infants and children with duplex kidney in a 5-year retrospective multicentric international survey. MATERIALS AND METHODS Data on 50 children who underwent RPN (41 upper-pole nephrectomies and 9 lower-pole nephrectomies) were retrospectively collected in this six-institution survey. Median age at surgery was 3.3 years. There were 35 girls and 15 boys. The left side was affected in 28 patients, versus the right side in 22 patients. We assessed intraoperative and postoperative morbidity. Follow-up (median, 2.5 years; range, 12 months-5 years) was based on clinical controls and echo color Doppler renal ultrasound scans. RESULTS Median duration of surgery was 255 minutes. Surgery was always performed with the patient in a lateral position. Special hemostatic devices were used for dissection and parenchymal section in all centers. Three patients from two centers (6%) required conversion to open surgery. We recorded seven complications (six peritoneal perforations, one opening of the remaining calyxes) in the 50 cases. Re-operation rate was 0%. Average length of hospital stay was 4.1 days. CONCLUSIONS Our survey shows that RPN remains a challenging procedure with a long learning curve, performed only in pediatric centers with huge experience in this field. In our survey operative time was longer than 4 hours. The complication rate remains high (7/50, or 14%), with complications classified as Grade II according to the Clavien-Dindo classification. They did not require further surgery, but they were associated with a prolonged hospital stay.

Family History is Underestimated in Children with Isolated Hypospadias: A French Multicenter Report of 88 Families

Purpose: While familial forms of complex disorders/differences of sex development have been widely reported, data regarding isolated hypospadias are sparse and a family history is thought to be less frequent. We aimed to determine the frequency of hypospadias in families of boys with hypospadias, to establish whether these familial forms exhibit a particular phenotype and to evaluate the prevalence of genetic defects of the main candidate genes. Materials and Methods: A total of 395 boys with hypospadias were prospectively screened for a family history with a standardized questionnaire, extensive clinical description, family tree and sequencing of AR, SF1, SRD5A2 and MAMLD1. Results: Family history of hypospadias was more frequent than expected (88 patients, 22.3%). In 17 instances (19.3%) familial hypospadias cases were multiple. Familial hypospadias was related to the paternal side in 59.1% of cases, consisting of the father himself (30.7%) as well as paternal uncles and cousins. Premature birth, assisted reproductive techniques, other congenital abnormalities and growth retardation were not more frequent in familial hypospadias than in sporadic cases. The severity of phenotype was similar in both groups. The results of genetic analysis combined with previous data on androgen receptor sequencing revealed that familial cases more frequently tend to demonstrate genetic defects than sporadic cases (5.68% vs 1.63%, p = 0.048). Conclusions: Familial forms of hypospadias are far more frequent than previously reported. Even minor and isolated forms justify a full clinical investigation of the family history. Detecting these hereditary forms may help to determine the underlying genetic defects, and may improve followup and counseling of these patients.

Early thoracoscopic resection of an atypical upper extralobar sequestration

We report a very rare case of complex upper extralobar pulmonary sequestration, detected during pregnancy and resected early because the diagnosis was uncertain (possibility of a teratoma). At 2 months, the upper left pulmonary lesion was completely removed by thoracoscopy. Pathology concluded an extralobar sequestration associated with a duplication cyst of mixed bronchogenic and esophageal type. The postoperative course was uneventful. The early thoracoscopic approach in this type of antenatally diagnosed lesion was a good option.