Florent Guérin

Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy.

Objective:To propose an anatomical classification of congenital portosystemic shunts (CPSs) correlating with conservative surgery. Background:CPSs entail a risk of life-threatening complications because of poor portal inflow, which may be prevented or cured by their closure. Current classifications based on portal origin of the shunt are not helpful for planning conservative surgery. Methods:Twenty-three patients who underwent at least 1 surgical procedure to close the CPSs were included in this retrospective study (1997–2012). We designed a classification according to the ending of the shunt in the caval system. We analyzed the results and outcomes of surgery according to this classification. Results:Two patients had an extrahepatic portosystemic shunt, 17 had a portacaval shunt [subdivided in 5 end-to-side–like portal-caval, 7 side-to-side–like portal-caval, and 5 H-shaped (H-type portal-caval)], 2 had portal-to-hepatic vein shunts (portohepatic), and 2 had a persistent ductus venosus. All extrahepatic portosystemic shunts, H-type portal-caval, portohepatic, and patent ductus venosus patients had a successful 1-stage ligation. All 5 end-to-side–like portal-caval patients had a threadlike intrahepatic portal venous system; a 2-stage complete closure was successfully achieved for 4 and a partial closure for 1. The first 2 side-to-side–like portal-caval patients had a successful 2-stage closure whereas the 5 others had a 1-stage longitudinal caval partition. All patients are alive and none needed a liver transplantation. Conclusions:Our classification correlates the anatomy of CPSs and the surgical strategy: outcomes are good provided end-to-side–like portal-caval shunts patients have a 2-stage closure, side-to-side portal-caval shunts patients have a 1-stage caval partition, and the others have a 1-stage ligation.

Outcome of central hepatectomy for hepatoblastomas

BACKGROUND/PURPOSE Central hepatoblastomas (CHBL) involving liver segments (IV + V) or (IV + V + VIII) are in contact with the portal bifurcation. Their resection may be achieved by central hepatectomy (CH) with thin resection margins on both sides of the liver pedicle, by extended right or left hepatectomy with thin resection margins on one side, or by liver transplantation with thick free margins. The aim of this study is to assess the operative and postoperative outcome of CH for hepatoblastoma. METHODS This was a retrospective monocentric study of 9 patients who underwent CH for CHBL between 1996 and 2008. RESULTS The operative time was 4 hours 50 minutes (2 hours 20 minutes to 7 hours), vascular clamping lasted 30 minutes (0-90 minutes), and the amount of blood cell transfusion was 250 mL (0-1800 mL). Two patients had biliary leakage requiring percutaneous drainage. Median follow-up time was 27 months (14-120 months). All of 8 nonmetastatic patients are alive and disease-free; 1 metastatic patient died of recurrent metastases at last follow-up. Although 3 of 9 patients had surgical margins less than 1 mm, none, including the patients who died from metastases, had local recurrence. CONCLUSIONS Our study demonstrates the feasibility of CH for CHBL without operative mortality or local recurrence. Central hepatectomy is an alternative to extensive liver resections in selected patients.

Pancreatic resections for solid or cystic pancreatic masses in children.

Objectives: The aim of the study was to assess the diagnosis and management of solid pancreatic neoplasm in children and the type of surgical treatment, focusing on short- and long-term outcomes. Methods: We retrospectively reviewed the charts of all children who had undergone pancreatic resection for suspicion of pancreatic tumor in Kremlin Bicêtre Hospital, Paris, between 1986 and 2008. We studied the symptoms at diagnosis, the type of surgery, and the short- and long-term morbidity and mortality. Results: Of 18 patients identified, there were 7 pseudopapillary tumors, 3 neuroblastomas, 2 rhabdomyosarcomas, 1 acinar cell carcinoma, 1 endocrine cell carcinoma, 1 renal angiomyolipoma, and 3 pancreatic cysts. Symptoms at diagnosis were abdominal trauma, abdominal mass, and jaundice. Operative procedures were duodenopancreatectomy (11), mid-pancreatic resections (2), splenopancreatectomy (2), distal pancreatectomy (1), and tumorectomy (2). There were no deaths related to surgery. The postoperative morbidity rate was 45%, including 2 cases of fistula (11%) occurring after a mid-pancreatic resection and a pancreaticoduodenectomy. The median follow-up was 4.2 years (range 2–11). There was no diabetes mellitus, but there was 1 case of fat diet intolerance requiring pancreatic enzyme substitution. All of the children had a growth curve within normal limits. Conclusions: In this experience, pancreatic resections have proven to be a safe and efficient procedure, with low long-term morbidity, for the treatment of tumoral and selected nontumoral pancreatic masses.

Liver nodules after portal systemic shunt surgery for extrahepatic portal vein obstruction in children

BACKGROUND Liver nodules have been reported after portal systemic shunt surgery (PSSS) in animal experiments or in humans with liver cirrhosis. The aim of our study was to assess the incidence of liver nodules after surgery for extrahepatic portal vein obstruction (EHPVO) in children without associated liver disease. METHODS We retrospectively reviewed the charts of 45 children who had surgery from 1979 to 2005 for EHPVO in our institution, consisting of 38 PSSS and 7 portal reperfusion procedures (PRPs). We assessed the presence of liver nodules on ultrasonography. RESULTS Of 45 patients, 7 (15%) had liver nodules during a median of 80 months of follow-up. All the nodules occurred after PSSS. Five nodules were subjected to biopsy; we found 2 liver cell adenomas and 3 focal nodular hyperplasias. CONCLUSIONS In this study, liver nodules occurred in 18% of cases after PSSS for EHPVO in children and not after PRP. As many children have undergone PSSS throughout the world, the presence of liver nodules should be considered during the follow-up of those patients.

Congenital portosystemic vascular malformations

Congenital portosystemic shunts are developmental abnormalities of the portal venous system resulting in the diversion of portal blood away from the liver to the systemic venous system. Such malformations are believed to come from an insult occurring between the fourth and eighth week of gestation during the development of hepatic and systemic venous systems, and could explain their frequent association with cardiac and other vascular anomalies. They are currently categorized into end-to-side shunts (type I) or side-to-side shunts (type II). This article aims to review the common symptoms and complications encountered in congenital portosystemic shunts, the surgical and endovascular treatment, and the role of liver transplantation in this disease. We will also focus on the current controversies and the areas where there is potential for future studies.

Meso‐Rex bypass for extrahepatic portal vein obstruction in children

Meso‐Rex bypass (MRB) and portosystemic surgical shunt (PSS) are both used to treat extrahepatic portal vein obstruction (EHPVO) in children. The aim of this study was to analyse the outcome of MRB and PSS to select patients who could benefit from a prophylactic MRB.

Prenatal and postnatal Ciliated Hepatic Foregut Cysts in infants.

PURPOSE Ciliated Hepatic Foregut Cyst (CHFC) is a rare congenital lesion arising from the embryonic foregut. Since squamous cell carcinomas arising from CHFC have been reported in adults, complete resection should be considered. We report our experience with CHFC. METHODS We reviewed the charts of 2 patients who had surgery after prenatal detection of a CHFC and 2 patients with postnatal diagnosis. RESULTS Two patients had antenatally detected liver cyst. Postnatal ultrasonography showed a cyst in segment IV, with wall calcifications and sediments. Bile ducts were encased in the wall of the cyst. They underwent central hepatectomy with double biliary diversion and uneventful post operative course. The two other patients underwent non anatomical resection of a cyst on the left lobe and in segment IV, found prior or during liver surgery. Pathology examination showed cysts filled with mucinous fluid, surrounded by an epithelium composed of ciliated cells. One case had a squamous metaplasia. CONCLUSION In infants, CHFC are found antenatally or incidentally. A solitary uni or mutilocular cyst with wall calcifications, sediments, located in the central liver segments should raise the diagnosis. Resection of large cysts in the central segments of the liver is challenging and biliary diversion should be considered.

A new surgical approach of temporary ovarian transposition for children undergoing brachytherapy: Technical assessment and dose evaluation

PURPOSE We developed a new technique of temporary ovarian transposition (OT) for prepubertal girls undergoing brachytherapy. The aim of this study was to describe it, assess its feasibility and safety and calculate the dose delivered to the ovary in order to prove its efficacy. METHODS Sixteen prepubertal patients underwent temporary OT for brachytherapy at our center from March 2001 to December 2012. OT was done either by laparotomy or by laparoscopy. In all patients, the ovaries were grasped with an atraumatic forceps and mobilized above the iliac crest level as high as possible without any dissection or division of the ovarian ligaments or of the fallopian tube. They were sutured to the anterior abdominal wall by a transfixing stitch of non-dissolvable suture knotted on the outside of the patient on a pledget. RESULTS Median age at surgery was 3 years (range: 2-9 years). The integrity of the fallopian tube was respected and not a single ligament was dissected or divided. None of the patients had intraoperative or postoperative complications. The stitches were retrieved after completion of irradiation and the ovaries in all the patients fell back into the pelvis. The calculated median radiation dose to the ovary was 1.4 Gy (range: 0.4-2.4 Gy). CONCLUSIONS This surgical technique is simple and safe, either by laparotomy or by laparoscopy. It meets the radiation and physical constraints in prepubertal girls with vaginal or bladder RMS. However, longer follow-up is required to assess the ovarian function.

Partial Unilateral Ureteral Obstruction in Newborn Mice: Magnetic Resonance Imaging and Pathology Studies

PURPOSE The mechanism underlying the evolution of congenital obstructive hydronephrosis in humans is still unclear. Although partial unilateral ureteral obstruction has been extensively explored in rats, studies in neonatal mice may lead to new insights into underlying cellular mechanisms, especially with the availability of mutant mice. We developed a model of partial unilateral ureteral obstruction in newborn mice. MATERIALS AND METHODS Mice were operated on by the fifth day of life. We created 3 groups, namely partial unilateral ureteral obstruction (embedding the ureter in the psoas muscle), complete unilateral ureteral obstruction (ligating the ureter) and sham (exposing the ureter). We studied pelvis diameter and kidney length on magnetic resonance imaging, and kidney weight, inflammation, apoptosis and fibrosis on histological assessment during the second (early) and fourth weeks (late) postoperatively. RESULTS Magnetic resonance imaging showed enlarged pelvis diameter in late partial unilateral ureteral obstruction, and in early and late complete unilateral ureteral obstruction. Pathological studies revealed parenchyma atrophy in early and late partial unilateral ureteral obstruction. Apoptosis occurred early in partial unilateral ureteral obstruction and decreased later. Macrophage infiltration was enhanced in early and late partial unilateral ureteral obstruction. Fibrosis increased in late partial unilateral ureteral obstruction. All of these results were significantly intermediate between mice with complete unilateral ureteral obstruction and sham operated mice. CONCLUSIONS It is possible to create renal lesions in newborn mice specific to partial ureteral obstruction. Magnetic resonance imaging of morphological changes demonstrated specific features of partial unilateral ureteral obstruction. Being noninvasive, this approach opens the way for further studies to investigate prognostic parameters after partial ureteral obstruction. In the future this model could be used in knockout mice to study the pathogenesis of renal lesions secondary to obstruction.

La préservation de la fertilité dans les cancers de l’enfant

Preserving fertility is a requisite for any child undergoing gonadotoxic treatment for cancer. Techniques vary depending on the age, sex of the patient and nature of the treatment. Boys undergoing irradiation have testicular protection or transposition. Post-pubertal boys undergoing chemotherapy can have semen cryopreservation like adults. However, fertility preservation for pre-pubertal boys undergoing chemotherapy implies testicular tissue cryopreservation. Research concentrates on germ cell transplantation, autotransplantation of testicular tissue or in vitro spermatogonial maturation in order to later restore spermatogenesis. Ovarian transposition can be undergone in case of brachytherapy or external beam radiotherapy in girls and has published success rates of 80%. Ovarian cryopreservation has been developed since 1995 for pre-pubertal and post-pubertal girls undergoing chemotherapy. The ovary is retrieved surgically and ovarian cortical segments are frozen. Thawing and transplantation of gonadal tissue are the next steps to restoring fertility. However, immature oocytes will have to be matured either in vivo or in vitro in order to restore fertility. Fertility preservation must be offered to children with cancer even if maturation of immature germ cells is uncertain for research 20 to 30 years from now will probably enable fertility restoration.