Quality of life and long-term results in patients operated on for Esophageal Atresia.

BACKGROUND: Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) represents the most severe entity among the foregut malformations, with patients at risk of chronic morbidity. This study aims to investigate on health-related quality of life (QoL) and long-term results in patients with EA. METHODS: 50 patients (30M, 20F, mean age: 14-year-old), among 82 patients operated from January 1995 to December 2005, answered the questionnaire. Two groups where compared: 8-12 years (A), 12-18 years (B). The survey investigated on 5 itmes: dysphagia; GERD; pulmonary affections (asthma, bronchitis, episodes of chronic cough during a year); growth and nutritional status (BMI); QoL scores. A control group of 50 healthy children was used. RESULTS: 42 type III AE (3 long-gap and 1 VACTER association). Median BMI was 18,72 (SD 3,38) (range: 30,52-12,80). In 22 pts BMI < 18,5. 19 pts were positive for dysphagia (EAT-10 score > = 3). A correlation was found between dysphagia and low BMI (Pearson 0,37). Dysphagia and low BMI were more diffuse in the younger population, while decreased in group B. 15 pts treated in their life for GERD (8 with medical therapy; 7 fundoplication). GerdQ test data showed only 4 pts with score > 6. GERD and dysphagia: 11 pts with GERD presented EAT-10 test > 3 (Pearson value confirmed this correlation: 0,59). Respiratory pathologies: 26 pts with positive anamnesis for chronic pulmonary affections (recurrent bronchitis in 21 pts; chronic cough in 15; the association of both in 12; 11 pts with asthma). PedsQL 4.0 median value was 15,4 (SD 10,1), control group value was 15,6 (SD 7,6); difference between the 2 groups was not statistically significant (p-value: 0,11). Correlation test for comparing high value of PedsQL and long-term complication in AE: the strongest association was with the dysphagia (Pearson: 0,55). CONCLUSIONS: Dysphagia resulted the most disabling symptom in group A but swallowing function slightly improved with the growth. Generally, AE seems not heavily influence patients' QoL.

Infantile Abdominal and Pelvic Lipoblastomas: A Case Series.

Lipoblastomas are rare benign mesenchymal tumors that arise from embryonal fat cells. They are usually discovered in infants and children under 3 years of age, and mostly occur in the trunk (from 10 to 60%, depending on the study) and extremities (from 40 to 45%), while head and neck localizations are rare, with only five cases described to date. We report on three cases of lipoblastomas in infants younger than 4 years, with unusual localizations: one intra-abdominal, discovered during a laparotomy for an intussusception; one pelvic, misdiagnosed as an ovarian mass; and one gluteal with a pelvic extension. All children underwent magnetic resonance imaging as preoperative workup. All tumors were completely resected with free surgical margins and ultrasonographic follow-up was uneventful for all patients.

Cystic intra-abdominal masses in children.

Benign intra-abdominal cystic masses in infancy are fairly uncommon and their etiopathogenesis, histology and clinical presentation differ significantly. Our aim is to report our experience in their treatment in order to discuss the best diagnostic and treatment modality. The medical records of 5 children (2M, 3F) with cystic intraabdominal masses referred to our hospital between November 2012 and September 2016, were retrospectively reviewed. All patients underwent open surgery and subsequent histopathologic analysis. Different clinical presentations, localizations of the masses, diagnostic tools, surgical approaches, histological examinations and outcomes were reviewed. Patients mean age was 5.4 years (range: 8 months-9 years). Two patients presented recurrent abdominal pain and abdominal distension; 1 patient had a palpable mass discovered incidentally and 2 complained acute abdominal pain. Routine laboratory tests, tumor markers and abdominal ultrasound were immediately done in all patients. Three patients underwent MRI and 1 abdominal CT. At laparotomy 2 hepatic cysts, 2 mesenteric cyst and 1 retroperitoneal cyst were discovered. Histology reports described: 1 hepatobiliary cystadenoma, 1 benign hepatic hamartoma and 3 cystic lymphangiomas (1 retroperitoneal and 2 mesenteric). There were no major postoperative complications, deaths, or recurrences in our series (follow-up 3-24 months). Despite the rarity of these lesions, benign cystic abdominal masses in children are not so uncommon and should be considered as causes of acute abdominal pain. The differential diagnosis is not always possible preoperatively. In our series, radical excision of the lesions was possible in all cases, allowing reliable histological results and avoiding recurrences.

Pyogenic Granuloma of the Sigmoid Colon causing Intussusception in an Infant.

Pyogenic granuloma is a benign vascular tumor that may affect the gastrointestinal tract. This report describes a rare case of sigmoid-colon pyogenic granuloma in a 4-month-old boy causing intussusception. Resection and anastomosis were curative. The mother had history of high dose of progesterone exposure during initial weeks of conception for vaginal bleeding. This may point towards etiology of the lesion.

Improved sperm count and motility in young men surgically treated for cryptorchidism in the first year of life.

INTRODUCTION: The timing of surgery in cryptorchidism has been debated for a long time. Reports on histology suggest better fertility outcomes with early surgery, whereas evidence of long-term improved fertility still lacks sound data. The aim of this study is to analyze sperm count and motility in a cohort of young men operated on during the first 2 years of life for cryptorchidism. PATIENTS AND METHODS: A total of 78 young men (age, 18-26 years) surgically treated for cryptorchidism in the second year of life were recalled to evaluate testicular volume and sperm count and motility. Of the 78 young men, 51 accepted to participate to clinical and sperm evaluation. Relationship between total sperm count (TSC), sperm motility (SM), and age at surgery was investigated by Student t-test and Fisher test. Patients were divided into two groups: those patients who were submitted to surgery in their first year of life (Group A) and those patients who were submitted to surgery in their first and the second year of life (Group B). We investigated the ratio of those patients with normal sperm count to those patients with abnormal sperm count (we defined as normal TSC > 15 million and SM > 15%) and compared the mean TSC and SM in the two groups. RESULTS: TSC were slightly but not significantly higher in the first group (45.5 ± 15.5 million/mL vs. 36.5 ± 23.6 million/mL, p = 0.107) and SM (30.5% ± 11.3% vs. 26.5% ± 15.4%, p = 0.341). The percentage of patients with normal sperm count and motility were significantly higher in the first group: normal TSC was found in 26 of 27 patients (96.3%) in Group A versus 18 of 24 patients (75.0%) in Group B (p = 0.042), normal SM was found in 26 of 27 patients (96.3%) versus 16 of 24 patients (66.7%), respectively (p = 0.008). In the two groups, no statistically significant difference was found neither in the proportion of patients with bilateral cryptorchidism, in the position of the testes, nor in the ratio of subjects treated with hormonal therapy before being operated on. CONCLUSIONS: In formerly cryptorchid subjects submitted to surgery in the first 2 years of life, the percentage of patients with normal sperm count and motility is higher than 95%, with even better fertility prognosis if orchiopexy is performed in the first year of life.