Immunohistochemical findings in rectal duplication mimicking rectal prolapse.

Alimentary tract duplications represent rare anomalies, with only 5 % occurring in the rectum. The variety in clinical presentation may lead to a delay in diagnosis or to incorrect and multiple surgical procedures. We report the clinical, histological and immunohistochemical characteristics of a rectal duplication occurring in a 3-month-old male with an unusual clinical presentation. Using routine histology and immunohistochemistry, the rectal duplication showed the diffuse presence of gastric mucosa with a characteristic immunophenotype (i.e., diffuse cytokeratin 7 positivity and scattered chromogranin immunoreactivity). As far as we know, this is the first report showing an immunohistochemical differentiation pattern of gastric lining in a rectal duplication. Our results, showing the presence of gastric mucosa, are suggestive of a possible origin from the embryonic foregut.

Testicular function in men treated in childhood for undescended testes.

PURPOSE: The aim of the study was to evaluate testicular hormones and sperm counts of young men treated in childhood for cryptorchidism METHODS: Testicular volume, serum luteinizing hormone (LH), follicle-stimulating hormone (FSH), and testosterone as well as semen specimens were evaluated in 57 men (mean age, 19 years; range, 18 to 27 years) treated in childhood for unilateral (n = 47) and bilateral (n = 10) cryptorchidism. In 3 unilateral cases monorchidism was found. Thirty-seven patients underwent orchiopexy after hormonal treatment (luteinizing hormone releasing factor, 1.2 mg/d for 28 days followed by human chorionic gonadotropin, 500 IU intramuscularly 3 times a week for 3 weeks). The remainder underwent surgery. Mean age at surgical treatment was 5.4 years (range, 2 to 12 years). These patients were examinated again after a mean period of 13.3 years (range, 10 to 19 years). RESULTS: Reduced testicular volume (<12 mL) was found in 6 of 64 testes (9.3%). LH, FSH, and testosterone levels were found within the normal range in all patients. With linear regression, inverse relations were found between FSH and, respectively, testicular volume (P =.002), sperm concentration (P =.013), sperm motility (P =.023), and normally shaped sperms (P =.019). There were direct relations between testicular volume and sperm concentration (P =.02), sperm motility (P =.000), and normally shaped sperms (P =.001). We did not find any statistical correlation between age at surgery and semen quality. Significantly better results in terms of sperm counts were found in patients directly operated on in comparison to those treated with hormones before orchiopexy. CONCLUSIONS: Presented data indicate tubular impairment in young men operated on in childhood for cryptorchidism; FSH values increase and testicular volume decrease are related to sperm deterioration. Studies on children treated in the first 2 years of life are required to clarify the usefulness of early treatment of cryptorchidism.

Feeding troubles following delayed primary repair of esophageal atresia.

Severe feeding troubles were recorded in five babies with long-gap esophageal atresia who underwent, between 1985 and 1990, a delayed primary anastomosis after spontaneous growth of their esophageal stumps. A comparison with 20 cases of direct esophageal anastomosis, operated on in the same period, was carried out by means of recorded esophagrams, pH monitoring and questionnaires charting the growth pattern and feeding habits of the patients. Bottle feeding, and, later on, the introduction of semi-solid foods was significantly retarded in the group of children with delayed primary anastomosis (labeled as group B) as well as height and weight parameters. Failure to complete feeds, dysphagia, vomiting, coughing, choking and recurrent respiratory symptoms were also significantly more common in this group than in the primary anastomosis group (labeled as group A) even in the absence of stricture. Variable degrees of disordered esophageal motility were present in all patients but pooling of the contrast medium, retrograde flow and delayed clearing of the esophagus were more frequent in group B. No patient was shown to have associated hiatal hernia. A 24 hour pH recording showed severe gastroesophageal reflux in 4 out of 13 cases of group A and in 3 out of 5 cases of group B. Clearing times were significantly delayed in all refluxing children. Our data suggest that the retarded start of oral feeding and swallowing coordination in patients with delayed primary anastomosis add further negative factors to their congenitally impaired esophageal motility, causing protracted dysphagia which represents a major problem for both family and hospital staff.

Non-palpable testes. Orchiopexy in single stage.

From 1986 to 1990, 53 non-palpable testes were treated in the department of Pediatric Surgery and Endocrinology at the Regina Margherita Hospital, Turin, Italy. The reported patients were 45, 8 of them were bilateral with both testes non-palpable, 13 of the remaining patients were right cryptorchid and 24 were left. All patients received medical treatment, by LH-RH nasal spray and HCG, before surgery. None of the non-palpable testes descended by hormone therapy only, so that surgical exploration was always required. In the 53 reported cases, 10 gonads were not found, 13 were atrophic and 30 were abdominal. All abdominal gonads were positioned into the scrotum by orchiopexy in a single stage. Half of the patients underwent operation before the age of 2. Therefore, it is to be pointed out that the younger a patient the shorter his inguinal canal, this facilitates orchiopexy for high, undescended testes. In 10 patients a testicular biopsy was made during surgical procedure. Normal morphology and normal spermatogonia content were observed in the abdominal testes of the patients who had reached the age of 1 year. During the follow-up of the reported patients no upward displacement was observed. Eleven patients underwent postoperative testicular ultrasonography which showed that parenchymal structure of the operated testis was normal and the volume of the testis was slightly smaller than normal. Based on their experience, the authors suggest to correct cryptorchidism before the age of one year, surgical procedure follows immediately after ineffective hormone therapy.

Bulky-pedunculated hemolymphangioma of the esophagus: rare case in a two-years old girl.

Benign esophageal masses are rare. The authors present a rare case of bulky pedunculated hemolymphangioma of the esophagus in a two-year-old female. The symptomatology was characterized by acute episodes of dyspnea associated with the protrusion of the mass from the mouth. The mass was removed endoscopically.

[A gastric trichobezoar: a report of a clinical case with anomalous presentation]. / Tricobezoar gastrico: descrizione di un caso clinico a presentazione anomala.

The Authors describe a case of a voluminous trichobezoar in a girl 9 years old. The patient was referred for evaluation of abdominal pain that presented after a banal blunt abdominal trauma occurred some days earlier. On physical examination an epigastric mass was detected. For a few days the girl suffered of inappetence and postprandial vomit. She was noted to have thin and sparse hair, thin eyebrows and onychodysplasia: these findings were considered as features of cutaneous adnexa dystrophy. The blood test and other laboratory findings were within normal limits. Abdominal US and UGI series failed to detect the nature of the mass. Therefore the patient underwent gastroscopy that showed a voluminous trichobezoar, so big and hard that gastrotomy was required for removal. The weight of the mass was 700 g; it occupied the whole stomach from fundus to pylorus. Postoperative recovery was uneventful. After discharge the patient was referred to the Neuropsychiatric Service for treatment trichophagia and prevention of recurrence. The most common site of trichobezoar is stomach. Clinical findings are often aspecific. US and UGI series may not allow the correct diagnosis. A rational diagnostic suspicion can arise when alopecia circumscripta is present. Endoscopy remains the elective technique: this procedure can be contemporarily diagnostic and therapeutic. Surgical approach is required only in case of trichobezoar with peculiar size, shape and hardness. We feel that a neuropsychiatric support is necessary to avoid recurrence.

[A neonatal ovarian cyst associated with transient endocrine anomalies]. / Cisti ovarica neonatale associata ad anomalie endocrine transitorie.

The authors present a case of enormous neonatal ovarian cyst associated with ipoglicemia, ipotiroidism and ipocalcemia. This baby presents also stenosis of the pulmonary artery and congenital dysplasia of the hip. The case is interesting because this type of association is unusual and has not been described before.

Effectiveness of primary inguinal orchiopexy as treatment of non-palpable testes in the first two years of age.

AIM: The best treatment of non-palpable testes is currently argument of debate. The aim of present study was to describe authors' experience in surgical treatment with inguinal standard orchidopexy of non-palpable testes. METHODS: In the last 17 years we have treated 2002 cryptorchid testes, among these 327 (16.33%) were non palpable. Age and distribution of cryptorchid testes was: 0-1 y (165 NPT), 1-2 y (84 NPT), 2-5 y (43 NPT), 5-10 y (16 NPT) and >10 y (19 NPT). RESULTS: Non-palpable testes were diagnosed and treated earlier (76.14% in the first two years). At surgical examination 204 (62.38%) were intrabdominal, 80 (24.46%) were atrophic and 43 (13.14%) vanishing. Among atrophic testes 54 (67.5%) were intracanicular, 21 (26.5%) were at the external inguinal ring, 4 (5%) were intrabdominal and 1 (1.25%) ectopic; among vanishing testes 22 (51.16%) were intrabdominal, 14 (32.55%) intracanicular and 7 (16.27%) at the external ring of inguinal canal. CONCLUSION: Atrophic and vanishing testes were in intrabdominal location in 26 cases: only in these cases (7.95% of all non palpable testes) laparoscopy should have avoided inguinal surgery. Inguinal standard orchiopexy performed as day-surgery with general anaesthesia associated to caudal analgesia should be considered effective and less invasive than laparoscopic approach.

[A rare case of esophageal atresia type I]. / Eccezionale caso di atresia esofagea di tipo I.

An extremely rare case of type A esophageal atresia is reported. The baby girl patient born spontaneously after a 38-week pregnancy, was diagnosed prenatally with suspected type A esophageal atresia. Diagnosis was confirmed at birth by chest and abdominal X-ray. As per protocol, a naso-esophageal tube was positioned in aspiration and a Stamm gastrostomy made for nutritional purposes. Evaluation of the distance between blind pouches at one month of life showed they were overlapping. At intervention the pouches were found to be united by a fibrous bridge about 1.5 cm long. Anastomosis was carried out with ease. The postoperative course was trouble-free. On the X day the baby girl was being fed completely per os. Histolo-gical examination of the fibrous residue excluded the presence of a mucosa-lined lumen. X-ray examination of the esophageal-gastric passage, one month after the operation, showed the smooth transit of the contrast medium and an adequate anastomotic lumen. At follow-up, at the age of 9 months, the baby was growing normally and being fed per os with a diet appropriate for her age; no oesophageal dilatation was necessary. Type A oesophageal atresias are long-gap forms: they are treated with direct anastomosis after the blind pouches come together spontaneously in the first four months of life. Stress is laid on the rarity of the case. According to Kluth's classification of 1976, this form was described by Mason in 1855 and Jlott in 1905 on the basis of autopsy findings. A review of the literature did not show any similar clinical cases.

The value of lung scintigraphy in the management of airways foreign bodies in children.

AIM: A retrospective analysis was made to evaluate our experience in the management of tracheobronchial foreign bodies (TFB) in children (age

Sperm count of young men surgically treated for cryptorchidism in the first and second year of life: fertility is better in children treated at a younger age.

INTRODUCTION: Recent data has indicated the usefulness of performing orchiopexy in the first years of life. In this study, we evaluated testicular function in young men operated on for cryptorchidism in the first year of life. To our knowledge, this is the first report on the effects of such an early treatment. MATERIALS AND METHODS: Testicular function was assessed in a group of young men operated for cryptorchidism during the first year of life (Group A, n=13) and during the second year of life (Group B, n=16). RESULTS: Total sperm counts were clearly higher in Group A (52.3+/-14.3 million/ml vs. 30.4+/-23.5 million/ml, p=0.005) as was sperm motility (36.2+/-8.7 vs. 23.1+/-15.7%, p=0.009). A clear inverse relationship was found between age at orchiopexy and total sperm count (r=-0.394, p=0.034) and sperm motility (r=-0.382, p=0.041). The relationship between volume of testes, position at surgery, uni/bilaterality of cryptorchidism, evidence of Ad spermatogonia at biopsy performed during surgery and treatment with LHRH and hCG performed before surgery and fertility was not significant. The latter findings may be partially explained by the low number of patients participating in the study and need further investigation. CONCLUSIONS: We obtained, for the first time, results showing the benefit of treating cryptorchidism during the first year of life rather than in the second year or even later.

[Conservative treatment of children constipation with Macrogol 4,000]. / Il trattamento conservativo della stipsi del bambino con macrogol 4000.

AIM: Macrogol 4,000 is one of the new generation's osmotic laxatives. It is constituted by a heavy molecular weight polymer without additional salts. In most of patients Macrogol 4,000 shows its efficacy in 48 hours from the beginning of treatment. Daily evacuations has been reported after first week therapy with an improvement in quality of life. The aim of this observational study was to demonstrate the efficacy of Macrogol 4,000 in the treatment of constipation in children. METHODS: The effect of Macrogol 4,000 was assessed in 120 children affected by constipation treated with therapeutic doses of Macrogol 4,000. The study period ranged from September 2006 to March 2007. The data analysis was collected with the evaluations concerning clinical examination and the consultation of daily diaries assembled by parents, tracking stooling pattern; in case of loosing contacts with the patient, a telephonic survey has been carried out. The child with symptoms improvement, i.e. reduction of the pain during defecation, daily spontaneous evacuations and better stool consistence, were considered ''normal''. RESULTS: Of the 120 patients, 89 returned to the clinical follow-up, while the remaining 31, who missed the follow-up, has been contacted by telephone. To the final analysis, 103 patients (85.8%) have had a normalization of the clinical symptoms, 9 patients (7.5%) have refused Macrogol 4,000 for the unpleasant flavour, 8 patients (6.7%) did not have any improvement from the treatment. CONCLUSION: The results of this observational study suggest that Macrogol 4,000 could be very useful to treat pediatric patients with constipation, also those affected by neuronal intestinal displasia (NID) type B.

Surgical treatment of cryptorchidism.

In the last 12 years, 916 cryptorchid testes have been treated sequentially with therapeutic and surgical procedures. Although the age of the boys varied considerably at the time that corrective procedures commenced, the best results were obtained when they were in the first year of life. The testes of the boys at this age who had been treated had a normal histology; this was not the case of those who were treated at an age older than one year.

Association between multiple intestinal atresia and omphalocele: a case report.

Multiple intestinal atresia is a rare disorder with vascular or hereditary etiology. So far, the occurrence of this malformation along with omphalocele has not been reported. We describe a boy born from a nonconsanguineous gypsy couple with intrauterine growth retardation, omphalocele, and multiple intestinal atresia from the pylorus to the rectum. The microscopic examination of the intestine shows multiple small lumina with a sieve-like appearance. This is characteristic of the hereditary atresias and suggests development of a defect in (re)canalization during embryogenesis. The association with omphalocele indicates a common developmental defect may be present.

A rare anorectal malformation: a very large H-type fistula.

A very large H-type rectovaginal fistula was observed in a 9-year-old girl. The diameter of the abnormal opening was about 15 mm. The fistulous tract originated from the stenotic segment 1 cm above the pectinate line of the anus and was connected to the back wall of the vagina. The patient underwent posterior sagittal transrectal surgery with a protecting colostomy. At present, she has good bowel function and sphincter control without recurrence of the fistula.

Cryptorchidism: medical and surgical treatment in the 1st year of life.

Since cryptorchidism can cause infertility and early orchiopexy can improve fertility, we tried to determine whether medical and surgical treatment in the 1st year of life can improve testicular fertility. We concluded that this is the best time to treat cryptorchid tests.

Early hormonal and surgical treatment of cryptorchidism.

PURPOSE: We investigated the efficacy of early gonadotropin treatment of cryptorchidism for promoting testicular descent and ameliorating testicular histology. MATERIALS AND METHODS: We treated 319 cryptorchid testes in 281 boys 4 months to 3 years old with luteinizing hormone-releasing hormone and human chorionic gonadotropin sequential therapy. Surgery was done on the 207 testes that did not respond to medical treatment. Microscopic biopsies were performed in 134 of these 207 testes. Histological findings were compared to those of 30 cryptorchid testes in boys younger than 1 year who underwent surgery without previous hormonal treatment. RESULTS: Combined luteinizing hormone-releasing hormone and human chorionic gonadotropin treatment induced scrotal descent of a percentage of cryptorchid testes depending on clinical position. Therapeutic success was greater when testes were in a lower position and results were not age dependent. Hormonal treatment of cryptorchidism during the first year of life stimulated spermatogonia maturation. CONCLUSIONS: When administered at the end of age 6 months, hormonal treatment can be considered an effective and timely substitution for gonadotropin and testosterone insufficiency in cryptorchid infants. Therefore, we recommend this therapeutic procedure combined with surgery in the first year of life.