Bronchopulmonary sequestrations in a paediatric centre: ongoing practices and debated management.

Objectives: Bronchopulmonary sequestration (BPS) is the second most common congenital lung malformation, with an estimated incidence ranging from 0.15% to 1.8%.

Twenty-eight years of intestinal transplantation in Paris: experience of the oldest European center.

Our aim was to describe our achievements in pediatric intestinal transplantation (ITx) and define areas for improvement. After a period (1987-1990) of nine isolated small bowel transplants (SBTx) where only one patient survived with her graft, 110 ITx were performed on 101 children from 1994 to 2014: 60 SBTx, 45 liver-small bowel, four multivisceral (three with kidneys), and one modified multivisceral. Indications were short bowel syndrome (36), motility disorders (30), congenital enteropathies (34), and others (1).

Impact of previous cyst-enterostomy on patients' outcome following resection of bile duct cysts.

Aim: To analyze the impact of previous cyst-enterostomy of patients underwent congenital bile duct cysts (BDC) resection.

Methods: A multicenter European retrospective study between 1974 and 2011 were conducted by the French Surgical Association. Only Todani subtypes I and IVb were included.

Congenital bile duct cyst (BDC) is a more indolent disease in children compared to adults, except for Todani type IV-A BDC: results of the European multicenter study of the French Surgical Association.

Aim: To compare clinical presentation, operative management and short- and long-term outcomes of congenital bile duct cysts (BDC) in adults with children.

Methods: Retrospective multi-institutional Association Francaise de Chirurgie study of Todani types I+IVB and IVA BDC.

Results: During the 37-year period to 2011, 33 centers included 314 patients (98 children; 216 adults).

Comparative results of gastric banding in adolescents and young adults.

Background/purpose: Obesity has become a major public health priority. Because of disappointing results obtained with dietary and medical programs, bariatric surgery has been offered to adolescents, although this practice remains controversial. Our aim was to evaluate laparoscopic adjustable gastric banding (LAGB) in adolescents at 2-year follow-up.

Extralobar pulmonary sequestration with combined gastric and intradiaphragmatic locations.

Extralobar pulmonary sequestration is a congenital lung malformation characterized by a non-functional lung segment with systemic feeding vessel. Over 90% of sequestrations are found in the thorax with less than 10% located in the abdomen. We present an unusual case of intra abdominal pulmonary sequestration, located suprarenally, adherent to both the stomach and the diaphragm.

Prenatal intestinal obstruction affects the myenteric plexus and causes functional bowel impairment in fetal rat experimental model of intestinal atresia.

Background: Intestinal atresia is a rare congenital disorder with an incidence of 3/10,000 birth. About one-third of patients have severe intestinal dysfunction after surgical repair. We examined whether prenatal gastrointestinal obstruction might effect on the myenteric plexus and account for subsequent functional disorders.

Intestinal absorption rate in children after small intestinal transplantation.

Background: Small bowel transplantation has now become a recognized treatment of irreversible, permanent, and subtotal intestinal failure.

Objective: The aim of this study was to assess intestinal absorption at the time of weaning from parenteral nutrition in a series of children after intestinal transplantation.

Design: Twenty-four children (age range: 14-115 mo) received intestinal transplantation, together with the liver in 6 children and the colon in 16 children.

Bacteriologic epidemiology and empirical treatment of pediatric complicated appendicitis.

Preoperative samples in the context of complicated appendicitis (CA) are rarely collected, and there is no consensus regarding the optimal antibiotic therapy in children. To help optimize empirical preoperative treatment, we studied clinical and bacteriologic data from a prospective cohort of 93 children with CA in a French hospital. All the bacteria isolated from peritoneal fluids were identified, using phenotypic and/or molecular techniques.

Indications and strategies for intestinal transplantation.

This lecture updates the pediatric surgeon on current indications, techniques, and results of intestinal transplantation in children with intestinal failure with reference to the Paris series.

Loss-of-function of MYO5B is the main cause of microvillus inclusion disease: 15 novel mutations and a CaCo-2 RNAi cell model.

Autosomal recessive microvillus inclusion disease (MVID) is characterized by an intractable diarrhea starting within the first few weeks of life. The hallmarks of MVID are a lack of microvilli on the surface of villous enterocytes, occurrence of intracellular vacuoles lined by microvilli (microvillus inclusions), and the cytoplasmic accumulation of periodic acid-Schiff (PAS)-positive vesicles in enterocytes. Recently, we identified mutations in MYO5B, encoding the unconventional type Vb myosin motor protein, in a first cohort of nine MVID patients.

Retrospective French nationwide survey of childhood aggressive vascular anomalies of bone, 1988-2009.

Objective: To document the epidemiological, clinical, histological and radiological characteristics of aggressive vascular abnormalities of bone in children.

Study Design: Correspondents of the French Society of Childhood Malignancies were asked to notify all cases of aggressive vascular abnormalities of bone diagnosed between January 1988 and September 2009.

Results: 21 cases were identified; 62% of the patients were boys.

Intestinal transplantation for total intestinal aganglionosis: a series of 12 consecutive children.

Background: Management of patients with total intestinal aganglionosis (TIA) is a medical challenge because of their dependency on parenteral nutrition (PN). Intestinal transplantation (ITx) represents the only alternative treatment for patients with irreversible intestinal failure for achieving intestinal autonomy.

Methods: Among 66 patients who underwent ITx in our center, 12 had TIA.

Lung resection in cystic fibrosis: a survival analysis.

Lung resection may be considered for cystic fibrosis (CF) patients showing localized severe chronic atelectasis and/or bronchiectasis. Nonetheless, literature on survival after surgery is scarce. This study was carried out to assess survival time after partial lung resection.

Paraesophageal bronchogenic cyst: first case reports in pediatric.

We described for the first time a paraesophageal intra-abdominal bronchogenic cyst, first considered as hepatic tumor, in a young boy. Resection was successfully realized by laparoscopy. Abdominal bronchogenic cyst and differential diagnosis are discussed.

Surgical treatment of an unusual case of pelvic extramedullary hematopoiesis.

Extramedullary hematopoiesis affects about 15% of the patients treated for thalassemia intermedia. Usually seen in adulthood, the most common location is the paraspinal region. Diagnosis and treatment of extramedullary hematopoiesis located in the pelvis of a young 15-year-old girl is discussed.

Grand mal seizures: an unusual and puzzling primary presentation of ruptured hepatic hydatid cyst.

We report a case of hepatic hydatidosis where the first clinical manifestations, generalized seizures after minor head and abdominal trauma, and delayed anaphylaxis, made the primary diagnosis difficult. Severe anaphylaxis has been reported as initial presentation of quiescent hepatic hydatidosis. In endemic areas, the diagnosis must be carefully ruled out in patients experiencing abrupt anaphylactic shock of uncertain etiology.

Study of the impact of liver transplantation on the outcome of intestinal grafts in children.

Background: Successful small bowel transplantation remains a challenge due to the septic and immune content of the gut. The possible beneficial role of the liver was assessed in pediatric recipients of isolated intestinal and liver intestinal combined transplantation, receiving the same immunosuppressive therapy.

Methods: Fifteen children who underwent small bowel transplantation (seven SbTx) or combined liver-small bowel transplantation (eight LSbTx) at a single center between 1994 and 1998 were retrospectively reviewed and compared with fifteen controls (eight normal and seven appendicitis as inflammatory control).

New clinical and therapeutic perspectives in Currarino syndrome (study of 29 cases).

Purpose: The aim of the study was to clearly define the anomalies that compose the Currarino syndrome (CS). We highlight the frequency of associated malformations of the spinal cord and the possibility of a communication between the presacral tumor and the spinal canal, leading to neurological complications.

Methods: We studied 29 patients with CS, including 12 familial cases; histological examination of the presacral tumor was performed, and cytogenetic and molecular biology studies of the HLXB9 locus were carried out.

The transmanubrial approach: a new operative approach to cervicothoracic neuroblastoma in children.

Background: Cervicothoracic neuroblastoma originates from the cervical sympathetic nerves and ganglia and thus presents a problem when dissecting the vascular and nervous elements of the subclavian region. The standard operation is based on thoracotomy or dual cervicotomy/thoracotomy, but these approaches do not provide optimal control of the subclavian vessels. We report our experience in children with cervicothoracic neuroblastoma by using a technique usually performed for apical lung cancer.

Inflammatory myofibroblastic tumor in children: clinical review with anaplastic lymphoma kinase, Epstein-Barr virus, and human herpesvirus 8 detection analysis.

Background/purpose: Inflammatory myofibroblastic tumor (IMT) is considered as an intermediate neoplasm that may present malignant features. Differential diagnosis with other tumor processes is sometimes difficult. Similar anaplastic lymphoma kinase (ALK) gene abnormalities as in anaplastic large cell lymphoma have been reported.