Sildenafil as a therapeutic option for digital ischemic ulceration: case report.

We report the case of a 37-year-old woman who developed critical upper limb ischemia caused by a cervical rib. Because the malformation was initially undiagnosed, a vascular bypass was performed, and failure occurred. Following a 6-month therapy with sildenafil, revascularization of the arm was successful and amputation was avoided. A 6-year follow-up shows a rich collateral network at the compression site and normal values of digital plethysmography. Because hand surgeons often see patients with digital ulcerations and other manifestations of peripheral vascular pathology, therapy of ischemia with sildenafil could be an effective treatment option in patients not responding to classic drugs.

Cardiopathie congénitale: de l'enfance à l'âge adulte [Congenital heart disease: from childhood to adulthood].

Grâce à l'amélioration de la chirurgie cardiaque, les enfants avec une malformation cardiaque congénitale atteignent actuellement en grande majorité l'âge adulte avec une bonne qualité de vie. Un suivi cardiaque régulier est toutefois recommandé. La période de l'adolescence coïncide souvent avec la survenue de complications à moyen et long termes et la nécessité d'une reprise chirurgicale ou par cathétérisme interventionnel, en particulier chez les patients avec cardiopathie complexe. Par conséquent, il est primordial de débuter le processus de transition assez tôt et de la poursuivre jusqu'à l'âge adulte. Nous avons élaboré un programme de transition formel, adapté aux patients avec cardiopathie congénitale. With the improvement of congenital heart surgery, most children with congenital heart disease will survive into adulthood with a good quality of life. Regular cardiac follow-up is recommended for all patients. The adolescent period coincides often with medium and long term consequences and complications and repeat surgery or catheter interventions might be needed. It is therefore of prime importance to begin the transition process early and to pursue it well into adulthood. We have elaborated a formal transition program adapted to youngsters with congenital heart disease.

Epidemiology of hypospadias in Europe: a registry-based study.

BACKGROUND: Hypospadias is a common congenital malformation. The prevalence of hypospadias has a large geographical variation, and recent studies have reported both increasing and decreasing temporal trends. It is unclear whether hypospadias prevalence is associated with maternal age. AIM: To analyze the prevalence and trends of total hypospadias, isolated hypospadias, hypospadias with multiple congenital anomalies, hypospadias with a known cause, and hypospadias severity subtypes in Europe over a 10-year period and to investigate whether maternal age is associated with hypospadias. METHODS: We included all children with hypospadias born from 2001 to 2010 who were registered in 23 EUROCAT registries. Information on the total number of births and maternal age distribution for the registry population was also provided. We analyzed the total prevalence of hypospadias and relative risks by maternal age. RESULTS: From 2001 to 2010, 10,929 hypospadias cases were registered in 5,871,855 births, yielding a total prevalence of 18.61 per 10,000 births. Prevalence varied considerably between different registries, probably due to differences in ascertainment of hypospadias cases. No significant temporal trends were observed with the exceptions of an increasing trend for anterior and posterior hypospadias and a decreasing trend for unspecified hypospadias. After adjusting for registry effects, maternal age was not significantly associated with hypospadias. CONCLUSIONS: Total hypospadias prevalence was stable in 23 EUROCAT registries from 2001 to 2010 and was not significantly influenced by maternal age.

Trigeminal Neuralgia Secondary to Intracranial Lesions: A Prospective Series of 17 Consecutive Cases

Object: The purpose of the study was to assess the role of Gamma Knife surgery (GKS) in secondary trigeminal neuralgia (TN) caused by space-occupying lesions. Methods: From July 2010 till January 2015, 17 patients had GKS for secondary TN caused by intracranial lesions. The primary outcome was tumor control. The secondary outcomes were the alleviation of pain and the eventual secondary effects. Covariates were the age, duration of symptoms, duration till alleviation etc. Results: The mean age in this series was 63.3 years (range 39-79). The mean follow-up period was 1.85 years (range 0.5-3). Nine (52.9%) were meningiomas, five (29.4%) trigeminal schwannomas, two (11.8%) brain metastases and one (5.9%) arteriovenous malformation (AVM). Eight were located on the right side and nine on the left side. The mean duration of TN was 13.5 months (range 0.5-160). Follow-up was available for 16 patients (94.1%). Pain alleviation appeared after a mean time of 4.6 months (1-11) in 15 patients (88.2%). Five (29.4%) patients completely stopped medication in a mean time of 7 months (range 1-13) and three (17.6%) decreased it at half of the initial doses. No patient developed new hypoesthesia or other cranial nerve complication. The marginal doses for meningiomas and trigeminal schwannomas were 12 Gy (12-14), for metastasis 20 (20-20) and for AVM 24 Gy. The mean target volume was 1.84 cc (range 0.12-8.10). The mean prescription isodose volume was 2.65 cc (0.19-11.90). The mean maximal diameter was 19.9 (range 9-36). The mean number of isocenters was 14.2 (4-27). The mean duration was 76.9 minutes (range 25-172). The mean conformity, selectivity, Paddick and gradient index were: 0.99 (range 0.955-1), 0.701 (range 0.525-0.885), 0.694 (range 0.525-0.885) and 2.904 (range 2.654-3.371). At last follow-up, tumor decreased in 10 (58.8%) patients, was stable in 6 (35.3%) and increased in one (5.9%), the latest at 6 months. Conclusions: Gamma Knife surgery is safe and effective in treating intracranial lesions presenting with secondary TN. The initial pain freedom response was close to 90%, while having no secondary effect. Pain alleviation is achieved even in the absence of a volume variation of the lesions.

Poland 's syndrome: radiologic findings

Poland's syndrome is a rare non-inherited congenital anomaly. The authors describe the classic radiologic findings of Poland's syndrome by reporting the case of a male four-year old patient with asymmetry of hands and chest, illustrating the fundamental imaging criteria for a conclusive diagnosis.

Congenital lobar emphysema presenting as an airway foreign body.

We report here the case of a 15 months old girl presenting with clinical signs and radiological exams highly suggestive of a foreign body (FB) aspiration. Diagnostic endoscopy revealed an overlooked bronchial malformation responsible for a congenital lobar emphysema (CLE). CLE presenting after one year of age is rare and can easily be misdiagnosed. Therefore, the possibility of an overlooked CLE needs to be known and evoked as an alternative diagnosis when dealing with children presenting with suspected FB aspirations. We report on this unexpected, yet misleading presentation of CLE and review the available literature on the subject.

Portal cholangiopathy: case report

The present report describes the case of a child that after blunt abdominal trauma presented with portal thrombosis followed by progressive splenomegaly and jaundice. Ultrasonography and percutaneous cholangiography revealed biliary dilatation secondary to choledochal stenosis caused by dilated peribiliary veins, characterizing a case of portal biliopathy. The present case report is aimed at presenting an uncommon cause of this condition.

Imaging findings of bronchial atresia in fetuses, neonates and infants.

Congenital lung malformations are increasingly detected before birth. However, bronchial atresia is rarely identified in utero and not always recognized in neonates. There are two types of atresia: 1) proximal, located at the level of the mainstem or the proximal lobar bronchi, which is extremely rare and usually lethal during pregnancy, causing a tremendous volume increase of the distal involved lung with secondary hypoplasia of the normal lung, and 2) peripheral, located at the segmental/subsegmental bronchial level, which may present as an isolated lesion or as part of a complex congenital malformation. Prenatal findings are mostly nonspecific. Postnatal exams show overinflated lung areas and focal bronchial dilations. The typical fluid-filled bronchoceles are not always observed in neonates but develop progressively in the first months of life. This pictorial essay describes the spectrum of imaging findings of bronchial atresia in fetuses, neonates and infants.

Risk of hypoglycemia in newborns from mothers with gestacional diabetes

There are not enough previous publications which are focused on mothers with well-controlled gestational diabetes mellitus (GDM) as a risk factor that determines the occurrence of neonatal hypoglycemia. In addition, approaches to blood glucose monitoring have been inconsistent and poorly defined. Our objective is to determine if being a newborn from a mother with well-controlled gestational diabetes (regardless insulin treatment) have a higher risk to develop hypoglycemia than a healthy newborn, using a defined and strict protocol. The project will take place in a regional hospital of Girona. We will recruit from 2014 to 2015 a cohort of 623 infants born in this center without any malformation or any perinatal pathology or complication, selected with a consecutive sampling. We will record sex, ethnicity and gestational age information. We will measure blood glucose levels and anthropometric measurements in newborns always taking into account the presence of well-controlled maternal gestational diabetes or not. Patients will be followed up during 24 hours to determine the incidence of hypoglycemia. We will analyze the contribution between exposure factors that we have studied and the incidence of the outcome using a multivariate analysis

Three new pathogens infecting antilles cherry in the State of Pará

When grown in monoculture, Antilles cherry (Malpighia glabra) plants have been affected by diseases which cause fruits malformation and spotting, reducing their value for market. From 1999 on, three new diseases characterised by leaf spot and fall of leaves have been observed in plantations located in Santa Izabel do Pará and Igarapé Açu counties. After isolation and pathogenicity tests on leaves of Antilles cherry plants, the isolates were identified as Calonectria ilicicola (anamorph: Cylindrocladium parasiticum) which causes large leaf spots reaching up to 7 cm long, brownish in colour, coalescent, scorching large leaf areas and causing 50% of leaf fall; Corynespora cassiicola, which provokes irregularly shaped, necrotic leaf spots with dark brown margins and white centers, surrounded by a yellow halo; and Myrothecium roridum which causes greyish target spots. Corynespora cassiicola has been reported causing leaf spots on different hosts in the Amazon region, while C. cassiicola has been recorded infecting Antilles cherry besides other hosts in the States of Maranhão and Pará.

Hemosuccus pancreaticus: causa rara de hemorragia digestiva maciça

Hemosuccus pancreaticus is a unusual syndrome manifested by hemorrhage into the pancreatic duct and by blood loss through the ampulla of Vater: It may be caused by tumors, arteriovenous malformation, pancreatic lithiasis, aneurism rupture from adjacent vessels, or erosion of pancreatic and peripancreatic vessels due to chronic pancreatitis. The authors describe a case of massive and recurrent gastrointestinal upper hemorrhage in a 26-year-old man without known risk factors for pancreatitis. This man underwent urgent surgery due to gastrointestinal bleeding during the ínvestigation. During the procedure, blood was found in the intestinal lumen and a tumor in the head of pancreas with two centimeters of diameter: A gastroduodenopancreatectomy was performed. Histological study showed chronic pancreatitis with a fistula from the pancreatic vessels to the Wirsung duct. The patient was discharged without postoperative complications and after months, remains assymptomatic.

Cisto de colédoco em adulto

Choledochal cyst is a rare congenital malformation of the biliary tree, and aproximately 25% of them are diagnosed in adults. Appropriate surgical management of these lesions depends on the anatomic site and extension of the cystic process. The recognized association of the bile duct cysts with hepatobiliary malignant disease has important surgical implications. Total cyst removal and cholecistectomy with Roux-en- Y hepaticojejunostomy was performed in a 47 year old female with a common bile duct cyst tipe 1 postoperative period was uneventful. A review of literature is presented.

Linfangioma cístico do fígado

Hepatic lymphangioma developed from a malformation that blocks the lymphatic system of a given area of the liver is a rare benign tumor observed most often in children and adolescents. The authors report and discuss a case of cystic lymphangioma of the liver in a 65 years old female patient, from a region where hydatid disease is endemic, with a complaint of epigastric pain, nausea, and vomiting. The patient presented a non-pulsatile mass, with severe pain at palpation in the epigastric region, The diagnostic investigation revealed a large cystic lesion in the left lobe of the liver; and she was submitted to bisegmentectomy II and III, which showed a hepatic lymphangioma. Considering the progressive character of this lesion, it should be carefully evaluated, since the removal of the lymphangioma is a safe, curative procedure.

Linfangioma cístico do pâncreas

Lymphangiomas are benign neoplasias of lymphatic tissue, extremely rare in the pancreas. Three lymphangioma types exist: capillary, cavernous and cystic. The authors report a case of cystic lymphangioma of the pancreas, with incidental diagnosis to the abdominal ultrasonography on investigation of urinary symptoms in male patient. To the laparotomy upper cystic mass was identified in the pancreas, that was totally resected. The histological examination demonstrated a cystic lymphangioma of the pancreas. The cystic lymphangioma of the pancreas was discovered accidentally, being assintomatic in most of the cases. Treatment of choice is complete resection, otherwise recidive is higher than 50%.

Hérnia de bochdalek em adulto

Bochdalek´s hernia is a congenital malformation of the posterolateral diaphragm region. It is more common on the left and more frequently seen in newborns and rare in adults, with over a few 100 reported cases. We present a case of Bochdalek´s hernia in a 49-year-old patient with long term dyspeptic symptoms. The upper endoscopy showed a gastric fundus herniation sliding into the chest through the diaphragmatic defect. The patient also presented with a rare pulmonary malformation diagnosed during surgery. It was corrected through thoracic approach with no other complications.