Esophageal tissue engineering: Current status and perspectives.

Tissue engineering, which consists of the combination and in vivo implantation of elements required for tissue remodeling toward a specific organ phenotype, could be an alternative for classical techniques of esophageal replacement. The current hybrid approach entails creation of an esophageal substitute composed of an acellular matrix and autologous epithelial and muscle cells provides the most successful results. Current research is based on the use of mesenchymal stem cells, whose potential for differentiation and proangioogenic, immune-modulator and anti-inflammatory properties are important assets. In the near future, esophageal substitutes could be constructed from acellular "intelligent matrices" that contain the molecules necessary for tissue regeneration; this should allow circumvention of the implantation step and still obtain standardized in vivo biological responses. At present, tissue engineering applications to esophageal replacement are limited to enlargement plasties with absorbable, non-cellular matrices. Nevertheless, the application of existing clinical techniques for replacement of other organs by tissue engineering in combination with a multiplication of translational research protocols for esophageal replacement in large animals should soon pave the way for health agencies to authorize clinical trials.

[Hyperechogenic fetal bowel: Which fetal and neonatal outcome? A French study of 149 cases]. / Hyperéchogénicité intestinale fœtale : quel bilan proposer et quel pronostic ? À propos d'une série continue de 149 patientes.

OBJECTIVES: In case of hyperechogenic fetal bowel (HFB), invasive procedures such as amniocentesis are often proposed to detect an underlying cause. Our goal is to study etiologies and prognosis of HFB according to antenatal sonographic findings in order to evaluate the relevance of antenatal assessment. MATERIALS AND METHODS: It is a retrospective monocentric study lead from 2008 to 2012, including all patients with a suspicion of HFB on routine sonography. We analysed the antenatal and neonatal results, distinguishing four situations: isolated HFB, HFB+other digestive anomalies, HFB+vascular pathology, HFB+other associated anomalies. RESULTS: For 149 patients, HBF was confirmed. Sixty-nine were isolated HFB, 24 associated with other digestive anomalies, 16 with vascular pathology and 40 with other anomalies. Pregnancy outcomes were different with 92.8, 41.7, 0 and 45.0% of healthy newborns. In the case of isolated HBF, we noted 2.9% cystic fibrosis and 2.9% congenital infection. CONCLUSION: Isolated HBF seems to have a better prognosis than associated forms. However, prenatal investigations to eliminate cystic fibrosis or congenital infection should be offered and may be initially non-invasive, if a larger series confirmed the absence of dyschromosomy in this population.

[Esophageal atresia: prevalence, prenatal diagnosis and prognosis]. / Atrésie de l'œsophage : prévalence, diagnostic anténatal et pronostic.

Esophageal atresia (EA) is a rare congenital malformation (1 in 2,500 to 3,500 births). Prenatal diagnosis (PN) is particularly interesting allowing search for associated malformations related to worse prognosis forms (reference ultrasound, MRI and amniocentesis) and planning the birth in an adapted medico-surgical center. Diagnosis of EA is usually suspected because of indirect and non-specific signs: association of polyhydramnios and absent or small stomach bubble. The visualization in ultrasound or MRI of cervical or thoracic fluid image corresponding to the expansion of the bottom of upper esophageal ("pouch sign") increases the specificity of diagnosis. However, prenatal diagnosis remains difficult and less than 50 % of EA are diagnosed prenatally. Biochemical analysis could improve these results. If EA is confirmed at birth, surgical management consists in a primary end-to-end anastomosis in first days of life, or in two-steps surgery if the defect is too large. Although current prognosis of EA is good, frequency of surgical complications and esophageal lesions secondary to gastroesophageal reflux justify a systematic and multidisciplinary extended follow-up.

[Esophageal atresia]. / Devenir à moyen et long terme des enfants atteints d'atrésie de l'œsophage.

Most of the children operated for esophageal atresia will survive the neonatal period. However, medium-term and late complications are frequent in this population. Gastroesophageal reflux disease is observed in 26 to 75% of the cases and can be responsible for peptic esophagitis, anastomotic stenosis, and Barrett esophagus, which is a risk factor for adenocarcinoma of the esophagus. Dysphagia is frequently observed, sometimes several years after the surgery, affecting up to 45% of children at the age of 5 years. Growth retardation is present in nearly one-third of children at the age of 5 years. Ear, nose, and throat and respiratory complications are also very frequent but tend to improve with time. Tracheomalacia is found in 75% of these children at birth, sometimes responsible for severe complications (malaise, bradycardia). Respiratory symptoms are dominated by chronic cough, wheezing, and infections reported in 29% of the children by the age of 5 years. Restrictive, obstructive syndromes and bronchial hyperactivity can be observed, but usually remain moderate. All these complications can influence the patient's quality of life, which is moderately impaired compared to healthy controls. The high frequency of late sequelae in esophageal atresia justifies regular and multidisciplinary follow-up through adulthood.

[Congenital diaphragmatic hernia: respiratory and vascular outcomes]. / Hernie de coupole diaphragmatique : devenir respiratoire et vasculaire pulmonaire.

Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly associated with a variable degree of pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Despite remarkable advances in neonatal resuscitation and intensive care, and new postnatal treatment strategies, the rates of mortality and morbidity in the newborn with CDH remain high as the result of severe respiratory failure secondary to PH and PPH. Later, lung function assessments show obstructive and restrictive impairments due to altered lung structure and lung damage due to prolonged ventilatory support. The long-term consequences of pulmonary hypertension are unknown. Other problems include chronic pulmonary aspiration caused by gastro-oesophageal reflux and respiratory manifestations of allergy such as asthma or rhinitis. Finally, failure to thrive may be caused by increased caloric requirements due to pulmonary morbidity. Follow-up studies that systematically assess long-term sequelae are needed. Based on such studies, a more focused approach for routine multidisciplinary follow-up programs could be established. It is the goal of the French Collaborative Network to promote exchange of knowledge, future research and development of treatment protocols.

[Pyeloplasty for pyelo-ureteric junction syndrome in children: lombo-assisted procedure versus lombotomy]. / Pyéloplastie pour syndrome de la jonction pyélo-urétérale chez l'enfant : voie lombo-assistée versus lombotomie.

Surgical treatment of pyelo-ureteric junction syndromes was classically at the child's, a pyeloplasty by posterior way or by lombotomy. For several years, assisted video techniques are proposed for this gesture. The purpose of our study was to compare the lombo-assisted pyeloplasty procedure with the lombotomy procedure, within the framework of this coverage. We made a retrospective study of procedures performed from January 2000 to December 2005, based on a file review of children operated for pyelo-ureteric junction syndrome. Children under the age of 2 years were excluded. Fifty-two children were divided in 2 groups: group 1: 24 children, 7 girls and 17 boys, average age of 86 months (extremes: 27-172) benefited from a lombo-assisted pyeloplasty. Group 2: 28 children, 12 girls and 16 boys, average age of 69 months (extremes: 24-129) benefited from a pyeloplasty by lombotomy. Operating times were significantly shorter in opened surgery than with the lombo-assisted procedure. There was no significant difference in terms of per- or post-operating complication, use of analgesic and hospitalization duration. On the other hand, the lombo-assisted procedure provided the same undisputable aesthetic benefit and the same muscular preservation than pure lomboscopy procedure. As a consequence, this procedure could be set up at no risk for the patient, even if it had lead to longer operating times. However, operating times stayed shorter than with the pure lomboscopy or the celioscopy procedures, compared to the literature.

[Outcome of children with repaired oesophageal atresia]. / Atrésie de l'oesophage : devenir des enfants opérés.

Although initial prognosis of oesophageal atresia is nowadays excellent with more than 95% of survival, the long-term complications are frequent. A gastro-oesophageal reflux is found in 26 to 75% of the cases, responsible for peptic oesophagitis, anastomotic stenosis and Barrett's oesophagus, risk factor of adenocarcinoma of the oesophagus. A dysphagia is frequently observed on these patients, sometimes several years after the surgery, observed in almost 45% of five-year-old children. Growth retardation is found in nearly a third of these children. Respiratory symptoms are particularly frequent, especially in the first years, associating tracheomalacia facilitating the bronchopulmonary infectious episodes (found in about 30% of 5-year-old children). Esotracheal fistula recurrence is very rare. A deformation of the rib cage is reported in 20%, and a scoliosis in 10% of the patients. However, the quality of life of these patients in the adulthood is good, and influenced by the existence of associated malformations. Even if the current prognosis of oesophageal atresia is good altogether, the frequency of the complications (digestive, respiratory, nutritional, orthopaedic) far from the initial intervention, and the necessity of a surveillance of the secondary oesophageal damages, justifies a systematic and multidisciplinary follow-up until adulthood.

[Necrotizing enterocolitis in two infants with gastroschisis]. / Entérocolite sévère chez deux nourrissons présentant un laparoschisis.

Necrotizing enterocolitis is seldom observed in full-term, or near term newborns. We report on two infants born after 36 weeks of gestation who presented with necrotizing enterocolitis after surgery for gastroschisis. Both patients had been formerly weaned from parenteral nutrition and fed thereafter with hydrolyzed cows' milk protein formula. At the age of 1.5 and 4.5 months respectively, the patients presented clinical and radiological signs of necrotizing enterocolitis. Evolution was favourable under medical treatment (parenteral nutrition, progressive refeeding). Post-neonatal digestive symptoms in a child with gastroschisis should prompt the diagnosis of necrotizing enterocolitis in order to allow an early treatment.

Technical problems and complications of a transanal pull-through for Hirschsprung's disease.

BACKGROUND: The purpose of this study was to evaluate the technical difficulties and complications of a transanal pull-through for Hirschsprung's disease. MATERIAL AND METHODS: This report was based on a multicentric retrospective study of 65 cases. Pull-through procedures were transanal Swenson or Soave procedures in 26 and 39 cases, respectively. RESULTS: Evaluation of the aganglionic level, peri-rectal dissection, and anastomosis were the three steps in the procedure where surgeons encountered difficulties. Such difficulties led to serious complications in 3 cases. A patient with a colon biopsy before the pull-through procedure had a postoperative pneumoperitoneum requiring a second laparoscopy for suture and washing. Another patient had peritonitis due to anastomotic leakage. Finally, a difficult rectal dissection in a neonate led to a urethral injury requiring secondary urethral repair. Only 41 of the 65 patients had no abdominal scars (63 %). CONCLUSION: We considered the transanal pull-through for Hirschsprung's disease to be a reliable technique. Nevertheless, it requires an urethral stent, precise dissection, careful anastomosis and selected indications in order to avoid major complications.

[Diagnosis and treatment of congenital bronchopulmonary malformations. A review of 32 cases]. / Diagnostic et traitement des malformations bronchopulmonaires congénitales : analyse de 32 observations.

OBJECTIVES: Analysis of our experience and propositions on the diagnosis and treatment of congenital bronchopulmonary malformations (BPM). POPULATION AND METHODS: Retrospective study of BPM diagnosed between 1997 and 2001. Analysis of clinical spectrum, diagnosis tools, treatment, and clinical outcome. RESULTS: Thirty-two cases of BPM have been investigated (11 cystic adenomatoid malformations, 7 pulmonary sequestrations, 7 bronchogenic cysts, 4 congenital lobar emphysema, and 3 complex emphysematous malformations). Nineteen patients had a prenatal diagnosis. For 9 others, symptoms occurred before 4 years of age. Evaluation included a CT-scan in all patients (BPM involution in one). Surgical treatment was performed in 30 patients (lobectomy in 18), with a mean age of 7 months for asymptomatic patients. During the follow-up (mean: 3 years), respiratory symptoms were reported in 10 cases, 3 of them were related to the BPM. DISCUSSION: Improvement in prenatal ultrasound diagnosis modified the management strategy. Considering the risk of pulmonary complications, surgical treatment is required during the first months of life. For congenital lobar emphysema, and some pulmonary sequestrations or small cystic adenomatoid malformations (<3 cm), conservative attitude may be preferred. BPM justify a multidisciplinary management.

[Cow's milk protein allergy after neonatal intestinal surgery]. / Allergie aux protéines du lait de vache après chirurgie digestive néonatale.

UNLABELLED: Cases of cow's milk protein allergy have been occasionally reported after neonatal intestinal surgery. AIM OF THE STUDY: To measure the prevalence of cow's milk protein allergy (CMPA) following neonatal intestinal surgery. PATIENTS AND METHODS: The files of all children who underwent intestinal surgery in the neonatal period over a four-year-period were reviewed. The diagnosis of CMPA was made on the association of one or several symptoms suggesting food allergy after the introduction of cow's milk protein in the diet, the disappearance of the symptoms after exclusion of cow's milk protein from the diet and their reappearance after reintroduction of cow's milk protein. RESULTS: During the study period, 251 neonates underwent an intestinal surgery. Among them, 11 babies (4.3%) developed CMPA. None of them had a medical history of family atopy. Moreover, while 5 children were fed with a diet containing cow's milk protein before surgery: none of them presented initially with symptoms suggesting CMPA before intestinal surgery. Small intestine suffering was observed during operation in seven of 11 patients. No specific neonatal digestive disease or malformation was associated with CMPA. The signs revealing CMPA were primarily digestive : diarrhoea (N =3), vomiting (N =4), abdominal distension (N =2), colic or anorexia (N =2). Casein specific immunoglobulin E were present in nine of ten cases, alpha lactalbumin and/or beta lactoglobulin specific immunoglobulin E were present in six of ten cases. Prick test were performed in three children and were positive. CONCLUSION: The high prevalence of CMPA among these patients with no risk factors of allergy raises the question of the role of neonatal intestinal surgery in developing food allergy. These data should be confirmed by prospective case-control studies. They underline the interest to evoke the diagnosis of CMPA when digestive symptoms occur after milk protein introduction in children undergoing neonatal intestinal surgery. Breast feeding or milk protein hydrolysate formula should be used for refeeding these patients.

Successful management of severe chylothorax with argon plasma fulguration and fibrin glue in a premature infant.

Post-traumatic neonatal chylothorax is a rare entity. Management includes medical and surgical treatment. We describe here a newborn who developed a severe chylothorax after repair of an oesophageal atresia. The chylothorax was treated successfully by a combination of argon beam coagulation of the mediastinum and fibrin glue application. However, the patient developed complete thrombosis of the left femoral vein with clot extension to the inferior vena cava which resolved after infusion of recombinant tissue plasminogen activator (rt-PA). The use of argon plasma coagulation and mediastinal fibrin glue application for treating postoperative chylothorax appears to be attractive and is easy to perform even in small premature infants and may replace a more extensive surgical procedure. However, the coagulation profile should be monitored and special care should be taken to prevent vascular thrombosis after surgery.

[Endoscopic diagnosis of a gastric heterotopic pancreas and esophageal atresia: an incidental association?]. / Diagnostic endoscopique d'une hétérotopie pancréatique de l'estomac et atrésie de l'oesophage: une association fortuite?

UNLABELLED: Heterotopic or aberrant pancreas is a rare congenital anomaly, usually asymptomatic. The preoperative diagnosis is difficult but most cases of gastric ectopic pancreas have a unique appearance that often makes possible a diagnosis in the absence of histologic confirmation. A well-delineated submucosal, firm mass with central umbilication is characteristic of gastric ectopic pancreas. To the best of our knowledge, the association of esophageal atresia and gastric heterotopic pancreas has never been reported previously. CASE REPORTS: We report two cases of heterotropic pancreas associated with esophageal atresia. Both diagnoses were incidental findings and concerned children operated on at birth for esophageal atresia. Although a definitive diagnosis is histologic, the endoscopic appearance made it possible to maintain this diagnosis. CONCLUSION: Some symptoms have been attributed to ectopic pancreas, and malignant degeneration has been reported in adults. Management continues to be debated: some authors recommend surgical resection while others, as we do, prefer initial therapeutic abstention.

Gastrostomy as a decompression technique in children with chronic gastrointestinal obstruction.

BACKGROUND: Percutaneous or surgical insertion of gastrostomy tubes for feeding children has been well described. However, there is no report of percutaneous gastrostomy for chronic decompression in children with chronic gastrointestinal obstruction. The objective of this study was to evaluate this technique. METHODS: Eighteen gastrostomies were performed for gastrointestinal decompression in children. The patients ranged in age from 2 to 125 months (median: 15.5), and all had prolonged hospital stays for severe disease: severe gastroparesis and/or duodenal dysmotility (n = 8), duodenal stenosis (n = 2), chronic intestinal pseudo-obstruction (n = 4), enterocolitis (n = 2), and metastatic abdominal carcinomatosis (n = 2). The duration of symptoms before gastrostomy placement ranged from 0.5 to 44 months (median: 2), with major symptoms including epigastric pain, early satiety, nausea, vomiting, and bloating. RESULTS: The goals of gastric decompression and removal of the nasogastric tube were achieved in all patients, and all had significant relief of both nausea and emesis. Oral intake of liquids and soft foods was possible in 17 of 18 of the children. The tubes were kept in place for a median of 22.5 months (range, 2-73). There was neither long-term morbidity nor mortality associated with the presence of the tube. Seventeen patients returned home with gastric decompression and cyclic parenteral nutrition, two of them for terminal care. Six patients died. In all patients, gastrostomy was used throughout and did not contribute to the death of the patients. For seven children, tubes were removed because of resolution of small bowel or gastric outlet obstruction. CONCLUSIONS: Gastrostomy is an efficient and well-tolerated method of achieving long-term gastric decompression in children with abdominal obstruction not amenable to surgery and/ or resistant to medical treatment.

Congenital pyloric stenosis: a modified umbilical incision for pyloromyotomy

Thirty-two patients underwent a pyloromyotomy via an umbilical incision; in 11 a modified umbilical approach was used to facilitate delivery of the pyloric mass. Incisions are made in the skin fold of the upper half of the umbilicus and at the midline, joining the two at the top. The skin incision is closed by upper umbilical translocation with a very good cosmetic result. This incision allows easy access to the pylorus and provides more convenient exposure. The absence of traction on the retractors avoids tissue ischemia, which leads to wound abscess development.

Congenital pyloric stenosis: a modified umbilical incision for pyloromyotomy.

Thirty-two patients underwent a pyloromyotomy via an umbilical incision; in 11 a modified umbilical approach was used to facilitate delivery of the pyloric mass. Incisions are made in the skin fold of the upper half of the umbilicus and at the midline, joining the two at the top. The skin incision is closed by upper umbilical translocation with a very good cosmetic result. This incision allows easy access to the pylorus and provides more convenient exposure. The absence of traction on the retractors avoids tissue ischemia, which leads to wound abscess development.