Vanishing Gastroschisis with a Favorable Outcome after a 3-Year Follow-Up: A Case Report and Literature Review.

Vanishing gastroschisis (VG) is a severe complication of gastroschisis with a high mortality rate. We report here a case of VG with a favorable outcome after a 3-year follow-up. A 26-year-old primigravida woman was referred to Strasbourg University Hospital because her fetus was diagnosed with an isolated gastroschisis at 13-week gestation. The ultrasound evolution was marked by a progressive closure of the abdominal wall defect from 19-week gestation and the appearance of dilated intra-abdominal loops. The child was born with a closed abdominal wall except a small remnant at the level of the former gastroschisis orifice. Explorative laparotomy revealed extensive midgut atresia with only 50 cm of remaining midgut. A jejunocolic anastomosis was performed. The child is now 3 years old and has a favorable outcome with only 2 nights a week of parenteral nutrition. A total of 39 cases of VG type D from Perrone et al. classification are described in the literature from 1991 to 2019, among which 19 (48.7%) are alive at the time of publication but only 4 cases are described with a long-term follow-up of 3 years or more. This is the fifth case described with a favorable evolution after 3-year follow-up.

[Medium and long-term respiratory outcome in patients operated from congenital diaphragmatic hernia: From a series of 56 patients]. / Évolution respiratoire à moyen et long terme des enfants opérés d'une hernie diaphragmatique congénitale : à partir d'une série de 56 patients.

INTRODUCTION: Recent advances in prenatal diagnosis, high frequency oscillatory ventilation and pulmonary arterial hypertension (PAH) therapies have progressively improved the survival of patients with congenital diaphragmatic hernia (CDH). However, CDH is associated with high morbidity that affects about half of surviving children. The main sequelae observed are respiratory (asthma, persistent PAH [PPAH], bronchopulmonary dysplasia, respiratory infections) and digestive (gastroesophageal reflux disease [GERD]). METHOD: This retrospective study focuses on the medium and long-term respiratory evolution of a cohort of 56 children with CDH and operated at Strasbourg University Hospital between 1999 and 2017. RESULTS: The mean age at assessment was 6,6 years (minimum: 5 months; maximum: 19 years). Seventeen patients (30 %) had asthmatic manifestations. Pulmonary Function Tests (PFT) showed obstructive patterns in 5/11 patients (2 with post-bronchodilator reversibility), and restrictive impairment in 5/11 patients, 3 of whom had thoracic deformity and/or scoliosis. Thirteen patients (23 %) had bronchopulmonary dysplasia. Few patients had recurrent respiratory infections (3.6 %) and chronic respiratory insufficiency (5.3 %). Thirty-nine patients (70 %) presented with an initial PAH, two of whom progressed to PPAH. Sixteen patients (29 %) had thoracic deformity and/or scoliosis and 48 patients (86 %) had GERD, 10 of whom had fundoplication. CONCLUSION: The long-term respiratory outcome of CDH operated patients is characterized by a moderate prevalence of respiratory symptoms and alterations in PFT that are important to consider in their medical follow-up during adolescence and adulthood. PAH is common in the neonatal period but rarely seems to persist.

[Is nephron-sparing surgery relevant for unilateral Wilms tumors?] / Néphrectomie partielle pour tumeur de Wilms unilatérale chez l'enfant : est-ce pertinent ?

BACKGROUND: Wilms tumors (WTs) are the most frequent renal tumors in children. Radical nephrectomy (RN) remains the gold-standard surgical treatment for this type of cancer. Excellent results in overall survival (>90%) make it possible to consider nephronic preservation. The objective of this systematic review is to evaluate the relevance of nephron-sparing surgery (NSS) for the treatment of nonsyndromic unilateral Wilms tumor (UWT) in children. METHODS: Articles in English related to "unilateral Wilms tumor, unilateral nephroblastoma, partial nephrectomy, nephron-sparing surgery, renal function" identified in the Medline library were screened and data were extracted to perform a qualitative systematic review. RESULTS: We identified 377 articles, 14 of which were integrated into the analysis. Data on 4288 children were included, 3994 (93.1%) underwent RN, whereas 294 (6.8%) underwent NSS. Stage I anatomopathology resulted in 55.1% RN and 79% NSS. Overall survival and event-free survival were similar: respectively 95.7% and 92.8% after RN and 96 and 90.5% after NSS. Positive margin status was higher after NSS (8.5% vs 0.5%), but tumor rupture and local tumor recurrences were similar. The rate of mild to moderate renal function was higher after RN (42% vs 10% after NSS). DISCUSSION: NSS is regularly performed for WT in case of bilateral or syndromic tumors, but the literature considering UWT does not show consensus. The superiority of NSS for renal outcomes has now been fully evaluated, but the main problem of this surgery in case of UWT is to ensure oncologic outcomes as good as outcomes after RN. WTs are usually massive tumors for which partial nephrectomy is contraindicated, but studies showed that chemotherapy before surgery could reduce tumor volume and make NSS possible. This review shows that NSS results seem to be as good as RN results and that preoperative chemotherapy should be highlighted for its participation in the reduction of the positive margin status. Although radiotherapy is used with caution because of its side effects, some studies showed that it gave excellent results for oncologic salvage after local recurrence. Constant progress in medical imaging and detection systems has led to the emergence of a new type of assistance for surgeons such as image reconstruction and vessel or urinary tract system segmentation. Virtual simulation of the operation based on a real case should help evaluate the feasibility of complex procedures in the near future. CONCLUSION: NSS for UWT seems to be a credible therapeutic alternative. New technologies such as 3D reconstruction should help surgeons define the best parameters to select ideal tumors for this surgery in the near future. For the moment, small tumors (<4cm), distant from the renal hilum (ideally on the upper pole) that respect at least 50% of the renal parenchyma (ideally superficial with exophytic development) seem to be the perfect indication for NSS.

Minimally invasive repair of pectus excavatum in children: Results of a modified Nuss procedure.

INTRODUCTION: Pectus excavatum (PE) is the most common deformity of the anterior thoracic wall. The Nuss technique allows the thorax to be reshaped with the aid of a retrosternal metallic bar. The aim of this study is to evaluate and compare the complication rate between the original Nuss technique and a lightly modified approach. MATERIAL AND METHOD: We performed a retrospective single-center observational study based on the medical files of patients operated for PE in the Pediatric Surgery Unit between July 2004 and July 2015. We divided two patient groups according to the operating technique employed: the Nuss group (NG) and the modified Nuss group (MNG) with supplementary subxiphoid incision and bilateral thoracoscopy. RESULTS: Twenty-seven patients were included: sixteen in the NG and eleven in the MNG. No significant differences were found between the two groups for all kinds of complications: total complication rate (50% for the NG versus 54% for the MNG, P>0.05), early (31% vs 46%, P>0.05), late (19% vs 9%, P>0.05), non-serious (37% vs 36%, P>0.05) or serious (13 vs 18%, P>0.05). There was no life threatening complication in the MNG, contrary to the NG. In the two groups, a significant difference was found (P=0.029) regarding the operating time: longer operating times (80±25min) were correlated with a higher complication rate. CONCLUSION: The modified Nuss technique does not cause more complications than the original technique described by Nuss and it has the advantage to minimize the risk of heart damage.

[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.

Fetal intervention for severe lower urinary tract obstruction: a multicenter case-control study comparing fetal cystoscopy with vesicoamniotic shunting.

OBJECTIVE: To evaluate the efficacy of fetal intervention using fetal cystoscopy or vesicoamniotic shunting in the treatment of severe lower urinary obstruction (LUTO). METHODS: A cohort of 111 fetuses with severe LUTO attending two centers between January 1990 and August 2013 were included retrospectively. Fetuses were categorized into three groups based on the method of intervention: (1) fetal cystoscopy, (2) vesicoamniotic shunting or (3) no intervention. Multivariate analyses were performed to determine the probability of survival and normal renal function until 6 months of age by comparing fetal cystoscopy and vesicoamniotic shunting to no fetal intervention. RESULTS: Of the 111 fetuses with severe LUTO that were included in the analysis, fetal cystoscopy was performed in 34, vesicoamniotic shunting was performed in 16 and there was no fetal intervention in 61. Gestational age at diagnosis, method of fetal intervention and cause of bladder obstruction were associated with prognosis. In multivariate analysis and after adjustment for potential confounders (considering all causes of LUTO) the overall probability of survival was significantly higher with fetal cystoscopy and vesicoamniotic shunting when compared to no intervention (adjusted relative risk (ARR), 1.86 (95% CI, 1.01-3.42; P = 0.048) and ARR, 1.73 (95% CI, 1.01-3.08; P = 0.04) respectively). A clear trend for normal renal function was present in the fetal cystoscopy group (ARR, 1.73 (95% CI, 0.97-3.08; P = 0.06)) but was not observed in the vesicoamniotic shunt group (ARR, 1.16 (95% CI, 0.86-1.55; P = 0.33)). In cases in which there was a postnatal diagnosis of posterior urethral valves, fetal cystoscopy was effective in improving both the 6-month survival rate and renal function (ARR, 4.10 (95% CI, 1.75-9.62; P < 0.01) and 2.66 (95% CI, 1.25-5.70; P = 0.01) respectively) while vesicoamniotic shunting was associated only with an improvement in the 6-month survival rate (ARR, 3.76 (95% CI, 1.42-9.97; P < 0.01)) with no effect on renal function (ARR, 1.03 (95% CI, 0.49-2.17, P = 0.93)). CONCLUSION: Fetal cystoscopy and vesicoamniotic shunting improve the 6-month survival rate in cases of severe LUTO. However, only fetal cystoscopy may prevent impairment of renal function in fetuses with posterior urethral valves. Our data support the idea of performing a subsequent randomized controlled trial to compare the effectiveness of fetal cystoscopy vs vesicoamniotic shunting for severe fetal LUTO.

Urological fistulas after fetal cystoscopic laser ablation of posterior urethral valves: surgical technical aspects.

OBJECTIVE: To describe the surgical technical aspects associated with the development of urological fistulas after fetal antegrade cystoscopic laser fulguration of the posterior urethral valves (PUV). METHODS: The perioperative data for all fetal cystoscopies performed between January 2004 and August 2013 at three institutions in the USA, France and Brazil were reviewed, with particular emphasis on surgical technical aspects of the procedure and the complications encountered. RESULTS: A total of 40 fetal cystoscopies were performed at the three institutions. Laser fulguration of the PUV was performed in 23 of these cases, with a survival rate of 60.9% (14/23) and normal renal function in 85.7% (12/14) of these infants. Urological fistulas were diagnosed postnatally in four (10%) newborns. The presence of fistulas was associated with a higher gestational age at diagnosis of PUV (P < 0.01) and with the use of semi-curved rather than curved sheaths (P < 0.01), the use of a diode laser (P < 0.01) and the use of higher laser power and energy (P < 0.01 and P < 0.01, respectively), as well as with less operator experience (P < 0.01) and with absence of fetal anesthesia/immobilization (P = 0.02). CONCLUSION: Urological fistulas are a severe complication of fetal cystoscopic laser fulguration of PUV and are associated with type, energy and power settings of the laser and instrumentation. The use of appropriate technique and proper training of the operator are necessary to perform this fetal intervention safely.

Lower pole vessels in children with pelviureteric junction obstruction: laparoscopic vascular hitch or dismembered pyeloplasty?

OBJECTIVE: To choose between laparoscopic "vascular hitch" (VH) and dismembered pyeloplasty (DP) in treatment of aberrant lower pole crossing vessels potentially responsible for pelviureteric junction obstruction (PUJO) in older children. PATIENTS AND METHODS: Retrospective study of 19 patients treated laparoscopically for PUJO. Based on videos of the procedures, we studied the anatomical relationship between the renal pelvis, the pelviureteric junction, and the aberrant vessels. RESULTS: Eight patients had laparoscopic VH and 11 had DP. All patients with DP needed drainage. In the VH group, 7/8 patients were asymptomatic and had decreased pelvic dilation. Half of them accepted MAG3 scintigraphy, and in these patients the obstructive syndrome disappeared completely. The last patient in this group was lost to follow-up. We observed three anatomical variations in the location of polar vessels: type 1 (in front of the dilated pelvis), type 2 (in front of the pelviureteric junction), type 3 (under the pelviureteric junction, resulting in ureteral kinking). CONCLUSION: Laparoscopic VH is a simple technique involving no urinary anastomosis or drainage, but we cannot guarantee that the crossing vessels are the sole etiology for PUJO. Following our experience, only patients with type 3 anatomical variations and with a normal pelviureteric junction should be proposed for VH.

[Liver torsion leading to death in a 16-month-old infant treated neonatally for an omphalocele]. / Volvulus du foie d'évolution fatale 16 mois après la cure d'une omphalocèle géante.

We report the case of an infant who underwent surgery on the first day of life for a giant omphalocele. At the age of 16 months, he presented an acute abdominal syndrome and died a few hours later. Autopsy revealed a twisted left liver lobe (LL) including a suprahepatic vein. To our knowledge, this is the first case of giant omphalocele complicated by twisted liver lobe and fatal outcome.

[Intussusception after congenital diaphragmatic hernia repair]. / Invagination intestinale après réparation d'une hernie diaphragmatique congénitale.

INTRODUCTION: Bowel intussusception is a common complication of abdominal surgery. However, the literature on intussusception after congenital diaphragmatic hernia (CDH) repair is scarce. CASE REPORT: A 24-month-old female was admitted with vomiting, crying and leukocytosis, with no objective abdominal signs. The chest x-ray showed the presence of bowel in the left hemithorax. Surgical exposure reduced a hernia across a Bochdalek defect, involving part of the left colon and the transverse colon. On the 7th postoperative day, the patient had symptoms of intestinal obstruction with worsening of her general condition. The explorative laparotomy evidenced an ileoileal intussusception, 15 cm from the ileocecal valve, in absence of a leading point. CONCLUSION: A postoperative intussusception in a similar case could be explained by atony of the herniated bowel, possibly a functional leading point in the postoperative phase, when the peristalsis is reactivated.

Modified Spitz procedure using a Collis gastroplasty for the repair of long-gap esophageal atresia.

OBJECTIVE: We report here on our experience with a modified Spitz procedure using a Collis gastroplasty for the repair of long-gap esophageal atresia (EA). PATIENTS AND METHODS: The authors reviewed 5 cases of children with long-gap (5-6 vertebral bodies) EA. The repair was carried out after 3 months of permanent monitoring, enteral nutrition through a gastrostomy tube and permanent aspiration of the proximal esophagus. The Collis gastroplasty was performed to create a gastric tube along the gastric lesser curvature using an Endo-GIA linear stapler. The gastric tube can be easily mobilized into the thorax after ligation of the left gastric vessels and anastomosed to the proximal esophageal segment. RESULTS: There were no intraoperative complications. The median postoperative hospital stay was 39 days (range: 30-60) with a median follow-up of 20 months (range: 16-29). During the early follow-up period, a digestive occlusion occurred in one patient, and several months later, she suffered from hiatal herniation. Other postoperative complications included anastomotic stricture (n = 2) treated by dilatations, gastroesophageal reflux (GER) (n = 3), and weight delay (n = 3) requiring additional enteral nutrition in 2 cases. No mortality was seen in our series. CONCLUSIONS: We propose a modified Spitz procedure using a Collis gastroplasty in the primary repair of long-gap EA as an alternative option to esophageal replacement or elongation techniques. The complications noted in our series are common complications of EA surgical repair, such as GER, anastomotic stricture and ponderal stagnation.

Surgical management of congenital chylothorax in children.

PURPOSE: Aim of the study was to determine the role of surgery in the management of congenital chylothorax (CC). METHODS: We retrospectively reviewed the data of patients with CC requiring medical or surgical treatment postnatally in our institution between January 2001 and March 2009. RESULTS: Ten patients were treated for CC. We divided our population into 2 groups: group A consisted of patients in whom CC healed after conservative medical treatment (thoracocentesis, pleural drainage, total parental nutrition, somatostatin, intrapleural injections of povidone-iodine), and group B of patients who needed both medical and surgical treatment (pleural abrasion and/or pleurectomy). Conservative postnatal therapy was successful in 50% of cases. Of the 3 patients treated preoperatively with intrapleural injections of povidone-iodine, 2 presented with severe complications. Surgical treatment was successful in all cases, with no surgical complications. Patients in group B had a significantly lower birth term (p=0.0254) and birth weight (p=0.0021) compared to patients in group A. Patients with a massive chylothorax (≥50 mL/kg/day) needed surgery significantly more often than those with chylothorax <50 mL/kg/day (p=0.0119). CONCLUSION: The initial postnatal medical management of CC should consist of thoracocentesis, drainage by tube thoracostomy, and total parenteral nutrition. If this treatment fails after 10 days, we propose using alternative therapies such as somatostatin (although its efficacy is not clear) and surgery. Chemical pleurodesis by intrapleural injection of povidone-iodine must be avoided in infants and small babies. Surgical management by pleural abrasion and/or pleurectomy appears to be safe and effective. Early surgical management is proposed for babies with low birth term, birth weight and massive chylothorax >50 mL/kg/day. Long-term follow-up is needed to evaluate the potential consequences of this therapy.

Postoperative intestinal obstruction after laparoscopic versus open surgery in the pediatric population: A 15-year review.

PURPOSE: The aim of the study was to determine the possible advantages of a laparoscopic approach versus open surgery in infants and children with regard to the development of postoperative intestinal obstruction (PIO). MATERIAL AND METHODS: A retrospective study was performed. The medical records of all infants and children who underwent an abdominal operation in our department between 1992 and 2007 were included. Data included initial diagnosis, age at initial operation, initial surgical approach, time to PIO, and type of definitive treatment. RESULTS: From 1992 to 2007, a total of 3 600 abdominal operations were performed in our institution. As not all types of operations can be performed using a laparoscopic approach, in order to obtain a more exact result we compared only operations of the same type performed either by laparoscopy or laparotomy in this period. A total of 2 243 comparable abdominal operations were performed, of which 1 558 (69.46%) were performed using a laparoscopic approach and 685 (30.54%) were performed by laparotomy. Postoperative intestinal obstruction (PIO) was diagnosed in 36 cases. In 14 of these patients (38.88%), the initial operation was performed via a laparoscopic approach, while the remaining 22 (61.12%) were initially operated using laparotomy. Overall, 0.89% of the patients initially managed by laparoscopy and 3.21% of the patients initially treated by laparotomy were treated for postoperative intestinal obstruction (p<0.0001). CONCLUSION: Abdominal surgery via a laparoscopic approach reduces the likelihood of PIO development. The risk for re-operation after development of PIO in infants and children is significantly higher in patients initially treated by laparotomy than for patients treated by laparoscopy.

Outcome of an adult granulosa cell tumour in an adolescent initially managed by laparoscopy.

Adult granulosa cell tumour is rarely encountered in adolescents. Tumours in their early stages have a favourable prognosis whereas those in advanced stages have a poor outcome. Documented series of tumours at advanced stages are very rare and follow-up is difficult because of the frequently prolonged intervals before progression of the tumour. That may explain why it is very difficult to define a consensual management strategy with or without adjuvant therapy such as chemotherapy or radiation therapy. We describe a case of adult granulosa cell tumour in a 14-year-old girl who initially underwent laparoscopy for an "unsuspicious" cyst. Management was then modified according to oncological guidelines because of the histological nature of this cyst.

[Laparoscopic treatment of congenital inguinal hernias in children (212 patients)]. / Traitement par coelioscopie des hernies inguinales de l'enfant: 212 patients.

OBJECTIVES: Laparoscopic treatment of inguinal hernias in children remains controversial. The inguinal approach is the only recommended procedure nowadays. The aim of our series was to precise the results of this technique and its advantages. POPULATION AND METHODS: We report our preliminary experience in laparoscopy for inguinal hernias in 212 children. RESULTS: Laparoscopy allowed us to discover 26 controlateral hernias not previously diagnosed, 3 direct hernias with 1 of them associated with a patent process vaginalis, 2 femoral hernias associated with a patent process vaginalis, 1 double pouch hernia and 1 volvulus of a cyst developed from a patent process vaginalis. In case of recurrence after an inguinal approach, laparoscopy seemed to be helpful to evaluate the anatomical lesion. We observed only 1 recurrence. DISCUSSION: Indications for laparoscopy in case of inguinal hernia in children are reviewed and discussed in the literature. Laparoscopic diagnosis for a precise diagnosis is no more discussed. Various techniques were proposed in case of laparoscopic treatment of inguinal hernias in children: most of surgeons realized only a suture of the sac. We prefer to dissect all the sac in order to avoid any recurrence. Advantages of laparoscopic approach become more obvious after the age of 2 years when the opening of the parietal wall is required in case of an inguinal approach and when local anaesthesia is no more recommended. The opportunity to diagnose a bilateral hernia when non diagnosed before surgery or to discover a femoral or a direct hernia, or in case of recurrence after an inguinal approach, the atraumatic dissection of the vas and spermatic vessels at the level of the internal inguinal ring, are the main advantages for laparoscopy.

[Surgical thoracoscopy in children]. / Thoracoscopie chirurgicale chez l'enfant.

This article is a state-of-the-art about the main diagnostic and therapeutic indications of paediatric surgical thoracoscopy. The authors stress the features of this surgery in children, in terms of anatomy, anaesthesia and postoperative analgesia. For experienced teams, almost any pleural, mediastinal or pulmonary lesion can be approached thoracoscopically.