Treatments for pediatric achalasia: Heller myotomy or pneumatic dilatation?

AIM: The treatment of achalasia consists of reducing distal esophageal obstruction by either Heller myotomy surgery or endoscopic pneumatic dilatation. The aim of the present study was to evaluate the short- and middle-term results of these procedures in children. METHODOLOGY: For technical reasons, children under six years old (n=8) were treated by surgery only, whereas patients over six years old (n=14) were treated by either Heller myotomy or pneumatic dilatation. RESULTS: Of the children aged under six years, 75% were symptom-free at six months and 83% at 24 months of follow-up. Of the patients aged over six years, complete remission was achieved by Heller myotomy in 44.5% vs. 55.5% by pneumatic dilatation after six months, and in 40% vs. 65%, respectively, after 24 months. Both pneumatic dilatation and Heller myotomy showed significant rates of failure. CONCLUSION: These results suggest that pneumatic dilatation may be considered a primary treatment in children over six years old. Also, where necessary, Heller myotomy and pneumatic dilatation may be used as complementary treatments.

Long-term outcome of colon interposition after esophagectomy in children.

BACKGROUND AND OBJECTIVES: Tissues derived from the colon, stomach, and jejunum have been used to replace the esophagus in childhood to cure esophageal atresia or stricture secondary to gastroesophageal reflux or the ingestion of corrosive agents. The outcome in adulthood of colon interposition performed at an early age has yet to be satisfactorily assessed. The aim of this single-center retrospective study was to evaluate the long-term nutritional, digestive, and respiratory outcome of all patients (n = 32) who underwent colon interposition during childhood in our hospital (1970-2001). PATIENTS AND METHODS: Medical records of these subjects were reviewed and their nutritional (weight, height, 24-hour food diary), digestive (questionnaire), and pulmonary function status evaluated. RESULTS: Of the patients, 17 had esophageal atresia (7 males, median age at surgery 11 months, range 0.5-61) and 15 had ingested corrosive substances (10 males, median age at surgery 50 months, range 22-113). Complications occurred less than 1 year postoperatively in 53% and long-term complications (occurring >1 year after surgery) in 84%. Long-term complications were common: digestive symptoms were found in 85% (most frequently observed during the first 5 years of follow-up), abnormal lung function in 7 (58%) of those tested (n = 12), feeding difficulties in 50%, scoliosis in 35%, and nutritional complications in 25%. CONCLUSIONS: Our study showed a high rate of sequelae following esophageal replacement. This highlights the need for multidisciplinary long-term follow-up into adulthood, and research into alternatives to colon interposition as treatment for esophageal strictures.

A case of adrenal haemorrhage after minor trauma in a young child: think of neuroblastoma.

We report a case of neuroblastoma diagnosed after adrenal haemorrhage following a minor trauma in a thirteen-month-old boy. Minor trauma is not commonly described as a cause of AH in the literature. Therefore when no accepted cause for AH can be found in a young child below the age of 5 years, it is important to look for a neuroblastoma and discuss the necessity of surgical exploration.

[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.

Favorable nutritional outcome after isolated liver transplantation for liver failure in a child with short bowel syndrome.

A patient with short gut syndrome presented with end-stage liver disease. She underwent an isolated liver transplantation at the age of 3.5 years. Parenteral nutrition was discontinued 1.5 years after surgery. At 7 years of age, the patient has maintained normal nutritional indices and growth while on a normal oral diet. This observation suggests that liver transplantation alone can be a valuable alternative to the combined small bowel/liver transplantation in short bowel syndrome when intestinal adaptation is expected and if the ileocecal valve is present and that improvement of gut function can occur after successful liver transplantation.

Is the appendix graft suitable for routine biliary surgery in children?

BACKGROUND/PURPOSE: The appendix graft (AG) is used widely for urinary tract replacement in children. Biliary tract replacement is less common. The purpose of this retrospective multicentric study was to evaluate the safety of appendix grafting for biliary reconstruction. METHODS: The files of 33 patients treated at 7 European pediatric centers were reviewed. Indications included choledochal cyst (CC) in 5 cases, biliary trauma (BT) in 1, and biliary atresia (BA) in 27. In CC and BT patients, the graft was inserted isoperistaltically between the proximal biliary duct and second duodenum. In all but one of the BA patients, the graft was placed antiperistaltically by patching its cecal end onto the porta hepatis. RESULTS: Postoperatively, all CC and BT patients initially became asymptomatic but developed laboratory evidence of anicteric cholestasis within 1 year. The most common manifestation was increased gamma-glutamyl-transpeptidase level (GGT), whereas histologic findings showed liver damage (mainly fibrosis). Reoperation has been carried out in 4 CC and 1 BT patients within a mean period of 19 months after appendix grafting. The graft procedure was converted to hepaticojejunostomy (HJ) in 4 and to choledocoduodenostomy in 1. Surgical exploration showed kinking in 1 patient and stenosis in 1. In the remaining 3 cases, there was no discernible cause of cholestasis, and appendix histology findings were normal. In all 5 reoperated patients, liver function findings returned to normal within 1 month. Reoperation is scheduled for the remaining CC patient who currently requires ursodesoxycholic medication to maintain normal liver function and presents histologic evidence of "de novo" sclerosing cholangitis. Results of appendix grafting also were poor in the 27 BA patients. Procedure-related perioperative complications occurred in 4 (15%) including 1 early death from graft necrosis. Another early death resulted from intestinal hemorrhage. Jaundice cleared in only 8 (28%). CONCLUSIONS: The findings of this study suggest that the AG is unsuitable for routine biliary repair in children. It should be used only as a salvage technique when conventional HJ repair is contraindicated. Because of the high risk of graft dysfunction, we recommend screening tests to detect biochemical or histologic cholestasis in any patient previously treated with appendix grafting.

Congenital annular constricting band: a case report with pseudarthrosis and review of the literature.

A case of congenital annular constricting band syndrome with an open pseudarthrosis of the tibia and fibula is reported. Ischemia, neurological defect and major lymphoedema were present. The child had an emergency treatment combining an external fixator and a one-stage resection of the constricting band. Bone union was obtained within 4 weeks and complete neurological recovery at 6 months. Long-term result, with realignment and normal function, is satisfactory. A review of the literature is undertaken. Amputations are frequent, however pseudarthroses are rare. One-step treatment of the band has been proposed by certain authors. Neurological recovery is usual. The risk of ischemia favors an early surgical treatment.

Mesenchymal hamartomas of the liver may be associated with increased serum alpha foetoprotein concentrations and mimic hepatoblastomas.

A mesenchymal hamartoma (MH) of the liver in a seven-month-old girl was associated with slightly elevated alpha fetoprotein (AFP) concentrations (320 microg/L, normal < 25 after six months of age). Hepatocytes were abundant at the periphery of the tumour at histopathological examination. These were slightly atypical and expressed AFP immunohistochemically. In two other girls, aged 14 months and two months respectively, with MH and increased serum AFP concentrations (320 microg/L and 7500 microg/L, respectively), hepatoblastomas were misdiagnosed preoperatively. MH of the liver with increased serum AFP concentrations may thus mimic hepatoblastoma radiologically if cysts are lacking, and foetal hepatoblastoma pathologically if a biopsy samples only the peripheral hepatocellular component of the MH.

Use of Ethibloc in the treatment of cystic and venous angiomas in children. 19 cases.

The authors report the use of Ethibloc in the treatment of cystic lymphangiomas and venous angiomas in 19 children. This embolizing product is injected by puncture in situ. Ten cystic lymphangiomas were treated, including 7 of the cervico-facial region. Of these ten, 8 were embolized initially using Ethibloc, and 2 after surgery. The diagnosis was always confirmed by echography and in some cases also by scanner. Six were completely cured and 2 had partial but satisfactory results requiring further injections. The final 2 cases when Ethibloc was administered after surgery, gave very disappointing results as the residual lesion was microcystic and thus difficult to access for the Ethibloc injection. The 9 venous angiomas involved various regions, including 5 facial lesions. These angiomas required a more extensive blood-clotting and radiologic assessment: standard radiography to detect the clot, Doppler echography and M.R.I. precisely clarifying the exact extension in depth. For 6 patients the cure was complete, for 2 others the results were good. Finally, in 1 case Ethibloc embolization was carried out prior to surgery, allowing definitive cure. In all, this technique resulted in a complete cure in 2/3 of the cases and its simplicity makes it a seemingly interesting alternative to surgery.

[Chest injuries in children. Apropos of 93 cases]. / Les traumatismes du thorax chez l'enfant. A propos de quatre-vingt-trieze observations.

UNLABELLED: Ninety-three cases of thoracic trauma (age ranging from 1.1/2 to 15 years) were observed over a 10 year-period (1980-1990): 86 cases of blunt trauma and 7 cases of perforating injuries. Road accidents were the most prevalent (63 cases) followed by home and sport injuries (9), falls (9), perforating trauma (9) and others. Associated lesions were frequent: skeletal (67), abdominal (19), and head injuries (5)--the latter influence the vital prognosis. Rib fractures were observed in 60% of cases of blunt trauma (86). Pulmonary lesions were as follows: pneumothorax (41), hemothorax (21), and lung contusions (29). In 48 cases, several of these pulmonary lesions were associated. Diagnosis and therapy problems were encountered in 21 cases. Results were as follows: 3 deaths, within 24 hours due to hemorrhage (1 case with aortic lesion). FOLLOW-UP: 85 good results, 5 sequelae, without any clinical consequence--either pleural (2), diaphragmatic (2), or lung (1). The treatment was as follows: none (27), pleural aspiration (2), thoraco-synthesis by drain in other cases for 2 to 9 days. Surgery was necessary in 10 cases, with 2 thoracotomies, 1 orthopedic traction, 1 embolization of iliac artery.

[Intractable diarrhoea revealing a neuroblastoma hypersecreting the vasoactive intestinal peptide]. / Diarrhée profuse révélatrice d'un neuroblastome hypersécrétant le vasoactive intestinal peptide.

In children, the watery diarrhoea-hypokalemia-achlorhydria (WDHA) syndrome is uncommon and usually due to a neuroblastic tumour hypersecreting the vasoactive intestinal peptide (VIP). We report a case of WDHA syndrome secondary to hypersecretion of VIP that revealed a neuroblastoma in a 13-month-old girl. A secretory diarrhoea, characterised by the persistence of diarrhoea despite the cessation of oral feeding, led to the search of a neuroblastic tumour in the patient. The serum concentration of VIP decreased to normal values soon after the surgical excision of the tumour.

[Small intestine atresia and abnormal insertion of the umbilicus in a child with fetal alcohol syndrome]. / Atrésie de l'intestin grêle et implantation anormale de l'ombilic chez un enfant présentant une foetopathie alcoolique.

BACKGROUND: Fetal alcohol syndrome is quite common in our region (incidence: 1/700 live births). It usually associates facial dysmorphism, intra-uterine growth retardation and mental delay. CASE REPORT: Jonathan, born to an alcoholic mother, presented a typical dysmorphy of fetal alcohol syndrome and a low inserted umbilicus. A small bowel atresia was discovered at the third day of life and operated on. CONCLUSION: Although never been previously described, this association suggests a common embryological origin between fetal alcohol syndrome, small bowel atresia, and umbilical abnormality.

[Children who swallow air]. / Les enfants avaleurs d'air.

UNLABELLED: Gas in the gut derives from swallowed air, intra-luminal production and diffusion from the blood. Excessive swallowed air may cause pathologic aerophagy. CASE REPORTS: This report describes four children aged from 3 to 12 years with pathologic aerophagy due to excessive air swallowing. One of them had a "Gas Bloat Syndrome". Clinical presentation was very incongruous. However, the clinical features were characteristic: abdominal distention increasing throughout the day, associated with visible and often audible air swallowing and excessive flatus. The questioning and clinical examination helped for diagnosing. Medical symptomatic treatment was associated with a psychotherapy approach. CONCLUSION: A better recognition of this condition might contribute towards earlier diagnosis.

[Trichobezoars in children and adolescents]. / Trichobézoards de l'enfant et de l'adolescent.

BACKGROUND: Trichobezoars are composed of hair or fibres. The typical patient presenting with a trichobezoar is an adolescent girl experiencing mental disturbance or retardation. CASE REPORT: Three girls with trichobezoars were seen. Two of them, 4 and 15 years old, had serious digestive antecedents (corrosive oesophagogastritis and oesophageal atresia, respectively) and had undergone previous surgery. The third was a 13-year-old mentally retarded girl. In two cases, an enzymatic dissolution trial was unsuccessful, requiring secondary surgical removal after, in one case, ineffective gastroscopic removal complicated by an oesophageal perforation. The third child had an initial operative removal. DISCUSSION: Serious digestive antecedents may favour bezoars, a diagnosis that must be evoked in such patients presenting with chronic digestive symptoms. Enzymatic dissolution is ineffective for large bezoars, and gastroscopic removal carries some risks such as perforation or intestinal obstruction. Operative removal is usually indicated. Psychiatric follow-up is needed to prevent this complication in children with serious digestive antecedents and to reduce the risk of recurrences.

[Anastomotic stenosis after surgical treatment of esophageal atresia: frequency, risk factors and effectiveness of esophageal dilatations]. / Sténose anastomotique après traitement chirurgical de l'atrésie de l'oesophage: fréquence, facteurs de risque et efficacité des dilatations oesophagiennes.

UNLABELLED: Anastomotic stricture is the most common complication following the surgical repair of esophageal atresia, and is usually treated by esophageal dilation. OBJECTIVES: The aims of this study were to assess in an infant population operated on at birth for type III or IV esophageal atresia: 1) the frequency of esophageal stenosis following the repair of esophageal atresia, and associated factors; 2) the efficacy of esophageal dilation by the Savary-Gaillard bougie technique. MATERIALS AND METHODS: The medical records of 52 children presenting with esophageal atresia over a 5-year period were retrospectively reviewed. Gestional age and birth weight, duration of mediastinal and transanastomotic drainage, and anastomotic complications including leakage, stricture, and the presence of gastroesophageal reflux were recorded and analysed. Patients presenting with anastomotic stricture were compared with a group of children without stricture. The number of esophageal dilations, their efficacy and the complication rate were analyzed. RESULTS: Anastomotic stricture developed in 20 (40%) of the 50 patients undergoing primary repair for esophageal atresia. The occurrence of anastomotic stricture was related to anastomotic tension during esophageal surgical repair (p < 0.03). Young children required esophageal dilation at a mean age of 142 days (24-930 days). Stricture resolution occurred after a mean of 3.2 dilations (1-15) over an average period of 7.9 months (range: 0-30 months). Dilation was successful in 90% of the 20 patients. Seven patients required only one dilation. Perforation of the esophagus occurred in one case, and this severe complication led to the death of the child. Esophageal dilation was unsuccessful in two patients, who presented prolonged severe dysphagia. CONCLUSION: Anastomotic stricture following repair of esophageal atresia is connected with the length of the gap that has to be repaired, and tension during suture. Esophageal dilation by the Savary-Gaillard bougie technique is an effective method for treating esophageal stricture. Several dilations are usually needed before the disappearance of dysphagia.

[Analysis of delay in diagnosis of extrahepatic biliary atresia]. / Analyse du retard au diagnostic de l'atrésie des voies biliaires.

UNLABELLED: Prognosis of extrahepatic biliary atresia depends on an early surgical treatment. This survey studied the delay to surgery of infants treated for biliary atresia and analysed the causes of late diagnosis and referral. MATERIAL AND METHODS: Medical files of 21 infants treated for biliary atresia between 1988 and 1998 were retrospectively analysed. RESULTS: Median age at biliary operation was 57 days and did not change during time. In only 3/21 cases, surgery was performed before 45 days of age. The first clinical or biological sign of cholestasis was noted at a median age of 12 days. In eight cases, the first medical visit for cholestasis (median age of 21 days) was not followed by a blood test. In 11 cases, infants presenting with biological cholestasis were referred to hospital more than seven days later. False diagnosis was noted in seven cases and delayed significantly the operation. CONCLUSION: The delay to surgical treatment is too long and does not decrease whereas a majority of infants visit their physician early. Medical information is mandatory for all physicians taking care of infants.

[Liver transplantation in an adolescent with cystic fibrosis]. / Transplantation hépatique chez une adolescente atteinte de mucoviscidose.

BACKGROUND: Orthotopic liver transplantation (OLT) is an effective treatment for patients with cystic fibrosis end stage liver disease, especially those with only mild pulmonary involvement. Long-term follow-up in such transplanted patients is still lacking. CASE REPORT: A 15-year-old girl with cystic fibrosis received an OLT because of severe decompensated cirrhosis. She had been colonized by Pseudomonas aeruginosa for 3 years and had pancreatic insufficiency; she also had mild glucose intolerance. Postoperatively she developed diabetes mellitus requiring insulin therapy for 9 months. Oral cyclosporin was poorly absorbed so that she was given a new emulsion of cyclosporin (Neoral) that was better absorbed. A rapid pubertal catch-up was obtained but the patient remained colonized by Pseudomonas aeruginosa. CONCLUSION: This 3-year postoperative follow-up confirms that OLT can represent a good alternative in those patients with severe liver disease and mild pulmonary involvement.

[The long-term follow-up of patients with a congenital diaphragmatic hernia: review of the literature]. / Hernie de coupole diaphragmatique: devenir à long terme.

The increased survival rate reached in infants with congenital diaphragmatic hernia (CDH) has shown a concomitant increase in late morbidity. Many complications including pulmonary damage, cardiovascular disease, gastro-intestinal disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities have been described. Long-term pulmonary morbidity in CDH consists of obstructive and restrictive lung function impairments due to altered lung structure and prolonged ventilatory support. Long-term consequences of pulmonary hypertension are unknown. Gastro-esophageal reflux disease (GERD) is also an important contributor to overall morbidity. Failure to thrive may be caused by GERD, insufficient intake due to oral aversion and increased caloric requirements due to pulmonary morbidity. Neurological outcome is determined by an increased risk of perinatal and neonatal hypoxemia in the first days of life of CDH patients. Many studies have addressed the substantial impact of the health problems described above, on the overall well-being of CDH patients, but only a few studies focus on the health-related quality of life. Considering the scattered data indicating substantial morbidity in long-term survivors of CDH, 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.

[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.