Gastroesophageal reflux and related pathology in adults who were born with esophageal atresia: a long-term follow-up study.

BACKGROUND & AIMS: Esophageal atresia (EA) is the most common congenital anomaly of the esophagus. There are few long-term follow-up data on adults who had surgery for EA as infants. The primary aims were to evaluate the prevalence of esophageal symptoms and pathology and second to develop recommendations for follow-up. METHODS: This is a descriptive study of individuals attending a clinic in an adult tertiary referral hospital, established to provide care for adults who had surgery for EA as infants. Individuals aged 20 years or older were identified from an existing database and invited by telephone to attend the clinic. One hundred thirty-two patients attended the clinic from 2000-2003. Individuals were assessed by using a structured questionnaire. Endoscopy was performed in 62 patients because of symptoms. RESULTS: Reflux symptoms were reported by 83 (63%), including 25 (19%) with severe symptoms. Dysphagia was reported by 68 patients (52%). Of those who underwent endoscopy, reflux esophagitis was present in 36 patients (58%), Barrett's esophagus in 7 (11%), and strictures in 26 (42%) patients. One patient was diagnosed with esophageal squamous cell carcinoma. Men who were 35 years or older and individuals with severe reflux symptoms were at high risk of having severe esophagitis or Barrett's metaplasia. CONCLUSIONS: Reflux symptoms, esophagitis, and Barrett's esophagus are common in these individuals. We recommend clinical assessment as adults and upper endoscopy for reflux symptoms or dysphagia. Transition of young adults from pediatric care to an adult gastroenterology clinic with expertise in EA appears to be highly beneficial.

Intussusception: trends in clinical presentation and management.

BACKGROUND: The association of a rotavirus vaccine and intussusception has renewed interest in understanding the incidence, clinical presentation and outcome of intussusception. METHODS: A retrospective chart review of all patients diagnosed with intussusception at Royal Children's Hospital, Melbourne over a 6.5-year period (1 January 1995-30 June 2001) was conducted using patients identified by a medical record database (ICD-9-CM code 560.0 1993-1997; ICD-10-CM code 56.1 1998-2001). Patient profile, clinical presentation, diagnosis methods, treatment and outcome were analyzed and compared to data previously reported on children with intussusception at the same hospital during 1962-1968. RESULTS: The hospitalization rate for primary idiopathic intussusception increased marginally from 0.19 to 0.27 per 1000 live births during the period 1962-1968 to 1995-2001. Most patients (80%) were <12 months of age (median age 7 months, range 2-72 months). The combination of abdominal pain, lethargy and vomiting was reported in 78% of infants. Air enema confirmed the diagnosis of intussusception in 186 of 191 cases (97%) and air reduction was successful in most cases (82%). Factors associated with increased risk of intestinal resection included abdominal distension (32%), bowel obstruction on abdominal X-ray (27%) and hypovolemic shock (40%). No mortality was observed in the present study. CONCLUSIONS: Over the past 40 years at Royal Children's Hospital, Melbourne the hospitalization rate due to primary idiopathic intussusception has marginally increased from 0.19 to 0.27 per 1000 live births. Diagnosis and treatment using air enema has been highly successful, resulting in a reduction in patients requiring surgery and reduced hospital stays.

Developmental haemostasis. Impact for clinical haemostasis laboratories.

Developmental haemostasis is a concept, now universally accepted, introduced by Andrew et al. in the late 1980's. However, coagulation analysers and reagents have changed significantly over the past 15 years. Coagulation testing is known to be sensitive to changes in individual reagents and analysers. We hypothesised that the reference ranges developed by Andrew et al. may not be appropriate for use in a modern coagulation laboratory. Our study was designed to determine whether a current day coagulation testing system (STA Compact analyser and Diagnostica Stago reagent system) was sensitive to age-related changes in coagulation assays. This is the first large scale study since Andrew et al. to determine the age associated numerical changes in coagulation proteins. Our results confirm the concepts of developmental haemostasis elucidated by Andrew et al. However, our results clearly demonstrate that the absolute values of reference ranges for coagulation assays in neonates and children vary with analyser and reagent systems. The results confirm the need for coagulation laboratories to develop age-related reference ranges specific to their own testing systems. Without this, accurate diagnosis and management of neonates and children with suspected bleeding or clotting disorders is not possible. Finally we present age related reference ranges for D-dimers, TFPI, and endogenous thrombin potential, previously not described.

Surgical management of a pancreaticopleural fistula in a child with chronic pancreatitis.

Chronic pancreatitis in children rarely results in the development of a recalcitrant pleural effusion, secondary to a connection between the pleural cavity and the pancreas. We describe such a case and the curative surgical therapy and include a brief discussion of the relevant medical literature as it pertains to this complication in the pediatric population.

The learning curve in learning the curve: a review of Nuss procedure in teenagers.

BACKGROUND: The Nuss procedure is a new minimally invasive repair for pectus excavatum that was first published in 1998. Modifications in technique are constantly evolving to minimize complications, especially bar displacement, which are higher in adolescents and adults. The present study reviews our early experience with this procedure in a much older group of children than previously reported and suggests an alternative method of avoiding bar displacement. METHODS: Retrospective chart review was carried out on 78 consecutive patients who underwent the Nuss procedure between December 1999 and January 2004. All patients underwent a uniform technique using bilateral lateral stabilisers and thoracoscopy was not used. Operative details, subjective cosmetic results and complication rates were assessed. RESULTS: The mean age was 15.4 +/- 3.2 years. Single bars were used in 95%, double bars in 5%. The defect was asymmetrical in 26%. The defect was mild in 7%, moderate in 59% and severe in 34%. The median length of stay was 6.0 days (range 4-11). Total operating time was 58 min (range 35-95). Patient controlled analgesia (morphine) for pain relief was used for 105 h (range 61-169) or 4.4 days; the epidural infusion was stopped 1 day earlier. Cosmetic results were excellent in 80.3%. There was residual asymmetry in 75% of the asymmetrical defects although all were improved. Complications included eight (10%) reoperations for bar displacement. This was related to the learning curve as seven of these occurred in the first 2 years of the series. Removal of the bar has been accomplished in 31 (40%) patients. This was elective in all but four patients (three early removals for pain and one for infection). CONCLUSIONS: The Nuss procedure gives good results even in teenagers. Modification of technique and increased experience has reduced complications. The use of bilateral lateral stabilisers without additional wire fixation is an alternative method to avoid bar displacement.