Morgagni hernia: transabdominal or transthoracic approach?

BACKGROUND: Morgagni hernias are rare and constitute less than 2% of all diaphragmatic hernias. Treatment is primarily surgical and transthoracic or transabdominal route approach methods are amenable. In this study, we compared the results of our Morgagni hernia cases, which underwent either transabdominal or transthoracic method of surgery. METHODS: We retrospectively analyzed the records of 20 patients we operated on for Morgagni hernias between 1997 and 2011 in our clinic. Age, sex, presenting symptoms, lesion location, diagnoses, applied surgical method, duration of the hospital stay, morbidity and mortality rates were reviewed. Six of the cases were (30%) approached via thoracotomy and 14 (70%) were laparotomy. The hernial sac was resected in all cases. Diaphragmatic defects were repaired using nonabsorbable sutures in all cases except in one case where prolen mesh used. RESULTS: Thirteen cases (65%) were female and seven (35%) were male. Mean age was 44.1 +/- 25.3 years (1-73 years). Hernias were located on the right side in 18 cases, the left side in one, and bilaterally located in one case. Herniated organs were: omentum in 19 (95%), transverse colon in 18 (90%), small bowel in 4 (20%), stomach in 3 (15%), and left lobe of the liver in one (5%) case. No complication was observed in patients who underwent laparotomy, and wound infection occurred in one patient who underwent thoracotomy. Hospital stays in thoracotomy and laparotomy groups are 7 and 6.2 days, respectively. There were no mortalities observed. There was no recurrence during the follow-up of 36.4 months (10-116 months). CONCLUSION: Our findings showed that both surgical methods have similar and satisfactory results. Although transthoracic approach was preferred in previous cases, the transabdominal approach was preferred in later ones because we assumed that the later procedure is less invasive for the patient. We prefer and propose the abdominal approach for the surgical management of Morgagni hernias.

Colon interposition for esophageal stenosis in a patient with epidermolysis bullosa.

Epidermolysis bullosa (EB) is a disease with 3 forms, most hereditary, characterized by spontaneous blistering lesions. The autosomally inherited form, epidermolysis bullosa dystrophica recessive (EBDR), is responsible for esophageal lesions consisting of web or stenosis. The authors could find only 9 cases treated by various esophageal replacement procedures in the literature, and the experience with 1 case treated by colon interposition is presented. J Pediatr Surg 36:1861-1863.

Rapidly progressive bronchiectasis complicating ulcerative colitis in a child.

Patients with ulcerative colitis may have a presentation dominated by extraintestinal manifestations. These manifestations, particularly bronchiectasis, are very rarely seen in pediatric patients. A 13-year-old boy with ulcerative colitis who was diagnosed by colonic mucosa biopsy is presented. He developed unexplained productive cough after the appearance of colonic disease. He was treated and followed up at his primary care hospital with the sole diagnosis of ulcerative colitis, with little attention given to the chest symptoms. The relation of the bronchial involvement to the ulcerative colitis was not established until two years after the onset of disease. Thoracal computed tomography (CT) examination after this period showed evidence of bronchiectasis and pulmonary involvement. Despite prophylactic inhaled corticosteroid treatment, no clinical or radiographic improvement was observed and widespread bronchial destruction developed very rapidly. More effective treatment with oral steroids was probably necessary in this patient, if the early chest symptoms were related to the ulcerative colitis.

Pattern of human leukocyte antigens in Turkish children with celiac disease.

BACKGROUND: Regional variations in the human leukocyte antigen (HLA) distribution patterns of celiac disease (CD) have been reported. The aim of the present study was to assess the distribution of HLA class I and class II in Turkish children with CD and to compare the findings with a control group. METHODS: Human leukocyte antigen typing was performed in 33 children with CD and in 77 healthy individuals, who served as controls, by using standard National Institutes of Health lymphocytotoxicity techniques. RESULTS: A positive association was found between HLA A2 (42 vs 19% for sick subjects compared with healthy controls, respectively), B8 (39 vs. 9% for sick subjects compared with healthy controls, respectively), CW7 (45 vs. 25% for sick subjects compared with healthy controls, respectively), DR3 (70 vs. 17% for sick subjects compared with healthy controls, respectively), DR7 (30 vs. 13% for sick subjects compared with healthy controls, respectively) and DQ2 (52 vs. 34% for sick subjects compared with healthy controls, respectively). The combinations of DR3-DQ2 (30 vs. 12% for sick subjects compared with healthy controls, respectively), DR3-DR4 (21 vs. 1% for sick subjects compared with healthy controls, respectively) and DR7-DQ2 (21 vs. 6% for sick subjects compared with healthy controls, respectively) were also found to be significantly important in children with CD. The highest relative risk (RR) was for HLA B8 in class I (RR 6.50), for DR3 (RR 11.30) in class II and for combination of DR3-DR4 (RR 20.46). The highest etiologic fraction (EF) was for the DR3 antigen (EF 0.55). CONCLUSIONS: The present study emphasizes that HLA genotypes are an important background to CD development, but some additional susceptibility factors remain to be identified.

Can zinc deficiency be used as a marker for the diagnosis of celiac disease in Turkish children with short stature?

BACKGROUND: It is generally accepted that celiac disease (CD) must always be considered when dealing with growth failure in children. Therefore, it is important to develop screening tests for detecting patients that need an intestinal biopsy. The aim of the present study was to investigate the value of plasma zinc levels for the diagnosis of monosymptomatic CD in short-statured children. METHODS: Fourty-nine children with a short stature and 34 healthy controls were investigated. Plasma zinc levels were assayed by atomic absorption spectrophotometry in short-statured children and controls. All patients with short stature underwent endoscopic small intestinal biopsy. RESULTS: Duodenal mucosal histopathology was normal in 25 children. Low plasma zinc values were observed in 54.2% of patients with CD, 32.0% of patients with idiopathic short stature and 14.8% of controls. The mean values of plasma zinc levels were not significantly different among the three groups. Sensitivity, specificity and the positive and negative predictive values for plasma zinc were 45.8, 76.0, 64.7 and 59.4%, respectively. CONCLUSIONS: These results indicate that zinc deficiency is an important problem in CD children with short stature; however, plasma zinc levels are not useful as a screening test for selecting patients for jejunal biopsy.

Mutation analysis in patients of Mediterranean descent with Wilson disease: identification of 19 novel mutations.

In this study, we report further results of mutation analysis of the ATP7B gene in Wilson disease (WD) patients of Mediterranean origin. A total of 136 WD chromosomes, 73 of which were of Italian, 43 of Turkish, 18 of Sardinian, and two of Spanish origin, were analysed and the mutation characterised in 84.5% of them. We found 50 different mutations of which 19 are novel, including three nonsense, one frameshift, and 15 missense mutations. The mutations detected were rare and mostly found in the compound heterozygous state together with other mutations and only rarely in homozygosity. Most of these mutations lie in the transmembrane and ATP binding loop regions. These data expand our knowledge of both the structure-function relationships of the WD protein and the molecular pathology of WD, thus improving our capability of prevention and genetic counselling.

Presinusoidal portal hypertension does not affect hepatic artery resistance index.

We aimed to determine the mean hepatic artery resistance index (RI) in presinusoidal portal hypertension and to compare the values with those in sinusoidal portal hypertension. The hepatic artery RIs of 11 patients with presinusoidal portal hypertension, 12 patients with sinusoidal portal hypertension and 16 healthy subjects were examined with duplex Doppler ultrasound. Mean hepatic artery RIs of three groups were compared. In patients with presinusoidal portal hypertension, mean RI in the hepatic artery (0.63 +/- 0.06) was significantly lower (p < 0.05) than that in the patients with sinusoidal portal hypertension (0.73 +/- 0.03). There was no significant difference (p > 0.05) in the mean RIs of the hepatic artery between the patients with presinusoidal portal hypertension (0.63 +/- 0.06) and the controls (0.67 +/- 0.05). Mean RI value in patients with sinusoidal portal hypertension (0.73 +/- 0.03) was significantly higher (p < 0.05) than the mean values in the control group (0.67 +/- 0.05). Hepatic arterial resistance does not change in presinusoidal portal hypertension, whereas it increases in sinusoidal portal hypertension. However, there are some overlaps in the RI values which raise difficulties in the differentiation of these two forms of portal hypertension.

Etiology of chronic diarrhea.

With worldwide use of oral rehydration solutions, the treatment of acute diarrhea does not pose much of a problem. However, chronic diarrhea is still harmful, especially for the growth and development status of the children. Between January 1993 to December 1996, patients who suffered from chronic diarrhea for more than one month duration and admitted to Dr. Sami Ulus Children's Hospital were evaluated for epidemiological and etiologic factors. Seventy consecutive patients were evaluated. The mean age was 40.8 months and 52% were males. Malnutrition was detected in 80% of cases. Etiologic factors included celiac disease 30%, cow milk allergy 17%, bacterial and parasitic factors 26%, cystic fibrosis 10% and postinfectious gastroenteritis 10%. Eosinophilic gastroenteritis, chronic nonspecific diarrhea, pseudo-obstruction, neurofibromatosis and inflammatory bowel disease were rarely detected. Celiac disease and cow milk allergy were implicated as the most common causes of chronic diarrhea. The vicious cycle of faulty nutrition, malnutrition and infection and postinfectious enteropathy were also significant factors in the etiology of chronic diarrhea. It may be considered that cow milk protein prick test, sweat test, immunologic tests and mucosal biopsies should be performed for the definite diagnosis of chronic diarrhea.

Celiac disease in Turkish short-statured children and the value of antigliadin antibody in diagnosis.

BACKGROUND: It is generally accepted that celiac disease (CD) must always be taken into consideration when dealing with children manifesting growth failure. It is, therefore, important to have laboratory tests capable of detecting patients who should undergo intestinal biopsy. In this study, we have prospectively evaluated clinical characteristics, gliadin antibody measurements and duodenal biopsies in 47 children with short stature and without gastrointestinal symptoms, in order to determine the incidence of CD and the diagnostic value of immunoglobulin (Ig)A and IgG antigliadin antibodies (AGA) for CD. METHODS: Anthropometric parameters and IgA- and IgG AGA were evaluated in 47 children with short stature. Antigliadin antibodies were measured by enzyme-linked immunosorbent assay (Euroimmun kit). Endoscopic intestinal biopsies were taken from all children. RESULTS: On the basis of intestinal biopsy, 26 (55.3%) patients were found to be probable CD. Sensitivity, specificity, positive predictive (PPV) and negative predictive value (NPV) for IgA AGA was found to be 23, 90, 75 and 48%, respectively. Sensitivity, specificity and PPV for IgG AGA was 100, 0 and 55%, respectively. The NPV for IgG AGA was not determined. CONCLUSIONS: The results of our study demonstrated that because of their incomplete sensitivity, specificity, PPV and NPV, intestinal biopsy can not be replaced by these tests.

Severe portal hypertension due to congenital hepatoportal arteriovenous fistula associated with intrahepatic portal vein aneurysm.

A 13-year-old girl was referred for assessment of severe gastrointestinal tract bleeding. Her liver function tests were normal, and she had no evidence of chronic liver disease or history of significant trauma. Clinical and sonographic findings suggested the presence of a portal vein aneurysm associated with a hepatoportal arteriovenous fistula. Abdominal angiography confirmed the diagnosis. The arteriovenous fistula was congenital, and the associated portal vein aneurysm was either congenital or secondary to hemodynamic changes in the portal venous system.

Hepatic damage in gluten sensitive enteropathy.

BACKGROUND: Gluten sensitive enteropathy has been reported to occur concomitantly with liver abnormalities, such as primary biliary cirrhosis, chronic active hepatitis and primary sclerosing cholangitis. METHODS: Duodenal biopsy was performed in nine children (all with short stature, five with chronic diarrhea and three with hepatosplenomegaly of unknown etiology) with a possible diagnosis of gluten sensitive enteropathy. All of the patients had different abnormalities in serum aminotransferase levels. With the help of laboratory investigations, viral, autoimmune, metabolic and toxic etiologies which cause hepatic damage were excluded. Liver biopsy was performed in five of these patients, two showing fibrosis and three nonspecific reactive changes. Gliadin antibodies were measured in six cases. RESULTS: Intestinal mucosal histopathology was compatible with gluten sensitive enteropathy in all patients. While immunoglobulin (Ig) G gliadin antibodies were positive in all cases, only three cases were found to have positive IgA gliadin antibodies. After a gluten-free diet, levels of transaminases fell to normal within 3 months and remained so in seven of these patients. A second intestinal biopsy, which was performed after 1 year of gluten-free diet revealed normal intestinal mucosa in all patients. CONCLUSION: Gluten sensitive enteropathy should be considered when evaluating a child with elevated levels of serum transaminase and in cases with cryptogenic liver disease.

Budd-Chiari syndrome in a child secondary to membranous obstruction of the hepatic vein treated by percutaneous transluminal angioplasty. Report of a case.

Budd-Chiari syndrome (BCS) due to membranous obstruction of the hepatic vein and the inferior vena cava is rare in children. We report a child with BCS that had a membranous obstruction at the level of the hepatic veins. The web was successfully dilated percutaneously by balloon catheters. Symptoms and signs of obstruction improved without any complication. As percutaneous catheterization is an effective, safe and repatable procedure, we recommend this technique for treatment of children and adults with BCS due to membranous obstruction of the hepatic veins.

Left ventricular dysfunction due to hypocalcemia in a neonate.

Hypocalcemia is a relatively uncommon but reversible cause of left ventricular dysfunction in infants and children. A 30-day-old boy with idiopathic hypocalcemia presented with congestive heart failure and convulsive seizures. He had no evidence of underlying cardiac disease. The cardiac failure responded to calcium therapy. It is suggested that hypocalcemia should be considered as a possible cause of left ventricular dysfunction in infants.

Oral rehydration of infants with hypernatremic dehydration due to acute gastroenteritis.

Twenty-five infants with hypernatremic dehydration due to acute gastroenteritis were given oral rehydration therapy (ORT). The patients received a glucose-electrolyte solution (such as that recommended by the World Health Organization) over six hours (2:1 rotating method). Twenty-three patients were successfully rehydrated within 48 hours after onset of therapy, while the two remaining patients attained normal serum Na+ levels within 72 hours. Acidosis was noted in 10 patients which disappeared in 24 hours.