Ruptured Sinus of Valsalva Aneurysm Associated with Situs Ambiguus, Isolated Levocardia, and Polysplenia.

Aneurysm of the sinus of Valsalva, a rare cardiac condition, results from dilation of an aortic sinus. Sudden aneurysm rupture can trigger rapidly progressive heart failure. We discuss the case of a 57-year-old woman with situs ambiguus, isolated levocardia, and polysplenia who presented with acute-onset heart failure. Transesophageal echocardiograms revealed an aneurysm of the right coronary sinus of Valsalva that had ruptured into the right atrial cavity. The patient underwent successful surgical repair. To our knowledge, this is the first report of a sinus of Valsalva aneurysm in a patient with this combination of congenital abnormalities. We briefly review the association between congenital heart disease, situs ambiguus, and ciliary dysfunction.

Ultrasound of congenital and inherited disorders of the pediatric hepatobiliary system, pancreas and spleen.

Ultrasound is often the initial imaging examination performed of the solid organs of the pediatric abdomen. The sonographic appearance of the hepatobiliary system, pancreas and spleen changes with growth and development. This article reviews the normal US appearance of these organs in children and illustrates, through case examples, congenital and inherited conditions that affect them.

Jarcho-Levin Syndrome with Splenic Herniation: A Rare Presentation.

BACKGROUND Jarcho-Levin syndrome, also known as spondylothoracic dysplasia and spondylocostal dysplasia, is characterized by varieties of vertebrae and rib anomalies. Jarcho-Levin syndrome is a clinical-radiological diagnosis with clinical evidence of short neck, short trunk, normal-sized limbs, or increased arm span, and vertebral and rib defects on the skeletal survey. CASE REPORT About 400 cases have been reported in world literature and 18 in our Indian literature. We report the case of a one-day-old female baby with a short trunk, short neck, low hairline, apparently long limbs, protuberant abdomen, mild midfacial dysmorphism, low-set ears, and a high-arched palate. There was one cystic swelling over the lateral side of the left hypochondrium sized about 3×3 centimeters, nonpusatile; the skin over the swelling was normal color and free. Radiological findings showed crowding of ribs with pebble-like appearance of the vertebrae and diastematomyelia of the spinal cord (type 2). We report here the first case of Jarcho-Levin syndrome with splenic herniation. To the best of our knowledge there have been no case reports of Jarcho-Levin syndrome with splenic herniation in the literature. CONCLUSIONS Jarcho-Levin syndrome can be easily diagnosed by clinical-radiological findings in newborns, with short trunk having a high index of suspicion. Prenatal diagnosis using level 2 ultrasonography can make it easier to manage the baby after delivery. Management should be from the basic neonatal care to prevention and immediate treatment of recurrent respiratory infections. Spinal surgical intervention to improve the thoracic volume and hence decrease the pulmonary restriction has been tried.

Ultrasound-guided percutaneous sclerosis of congenital splenic cysts using ethyl alcohol 96% and minocycline hydrochloride 10%: A pediatric series.

INTRODUCTION: The management of congenital splenic cysts continues to evolve. In the past the standard treatment was splenectomy, but increased knowledge about the spleen's immunologic function has led most pediatric surgeons to preserve splenic tissue. A great number of studies using sclerosing substances have been published, but to date reports in children have been limited. Our study concerns a group of 15 children with congenital splenic cysts treated with percutaneous drainage and sclerosis with alcohol. We performed the procedure under general anesthesia and checked radiologically for possible leakage. METHODS: In 2000 our group started managing pediatric patients with splenic cysts. During the first eight years surgery was the treatment of choice. From April 2008 to December 2014, a prospective study was conducted on 15 consecutive patients treated with percutaneous sclerotherapy. The outcomes regarding cystic dimensional variations before and after treatment were analyzed. RESULTS: In 20% of patients complete disappearance of the cystic lesion was achieved. In 67% of the patients the maximum diameter of the cyst was reduced to below 50mm. CONCLUSION: Our results should encourage the use of this treatment because it is a valid and safe option in childhood. The high success rate achieved with percutaneous drainage and sclerotherapy of cystic lesions supports our results.

Reference ranges of fetal spleen biometric parameters and volume assessed by three-dimensional ultrasound and their applicability in spleen malformations.

AIM: The aims of this article are to establish three-dimensional ultrasonographic nomograms of normal fetal spleen size and to evaluate the clinical application value. METHODS: An observational, cross-sectional study was performed on 455 women with a normal singleton pregnancy between 18 and 38 weeks' gestational age (GA). Fetal spleen volume was measured using three-dimensional ultrasound equipped with virtual organ computer-aided analysis, and biometric parameters were assessed in multiplanar mode to create reference ranges to GA. Thirty cases were randomly selected to conduct reliability analyses via intraobserver and interobserver ultrasonographic measurement. Moreover, 50 cases of suspected splenic malformations were evaluated by the newly established nomograms and followed up subsequently. RESULTS: Using regression formulas, we found that fetal spleen size increased with GA. We observed strong reliability in intraobserver and interobserver volume measurements with intraclass correlation coefficients of 0.994 and 0.962. Bland-Altman analyses showed narrow limits of agreement [intraobserver: (-3.2 to 3.5)%; interobserver: (-3.2 to 4.3)%]. Of the 50 cases with suspected splenic malformations, six cases of splenomegaly and one case of splenic cyst were diagnosed. CONCLUSION: Three-dimensional ultrasound nomograms of normal fetal spleen size across a range of GA have a strong diagnostic value. Volume measurements with good reliability were optimal in clinical practice.

Splenogonadal fusion mimicking a testis tumor.

The presence of ectopic splenic tissue in the scrotum is attributed to splenogonadal fusion, a rare congenital anomaly. This ectopic splenic tissue can be an incidental finding or less often present as a scrotal mass later in adult life. Given the rarity of splenogonadal fusion, especially in the adult population, this case highlights the clinical characteristics of the condition, with a special focus on the signs and findings that might help prevent unnecessary orchiectomy.

Continuous-type splenogonadal fusion: report of a rare case.

Splenogonadal fusion is a rare congenital anomaly. It has two subtypes, discontinuous and continuous. Up to one-third of continuous-type fusion is associated with other congenital anomalies. We present a continuous-type splenogonadal fusion case that was found incidentally during indirect hernia repair; the testicle was preserved during excision. Laparoscopic exploration was helpful in identifying the isolated polysplenia as the origin of continuous-type splenogonadal fusion, and in excising the cord-like attachment proximally. The patient had no other associated anomaly.

[Prenatal ultrasonic diagnosis and follow-up of fetal splenic cysts: report of two cases. Review of the literature]. / Diagnóstico ecográfico prenatal y seguimiento de quistes esplénicos fetales: a propósito de dos casos. Revisión de la bibliografía.

Congenital splenic cysts are rare entities wich are not frequently diagnosed in prenatal sonographies and there is only a few literature wich report these medical cases. The aim of this article is to introduce this sonographic discovery and study whether there are impacts for the newborn, association with cormosomopathy or fetal pathology. We describe two medical cases of fetal splenic cysts wich were diagnosed in our service by ultrasonography at 29 and 32 weeks of gestation, their antenatal monitoring and postnatal evolution. Besides theses, it is made a review of this entity in medical literature, examination about the: etiology, prenatal diagnosis, prenatal and postnatal following and complications. In conclusion, the congenital splenic cyst has a good prognosis with an spontaneous postnatal resolution in the majority of the cases. No association was observed between cromosopathy or fetal pathology with the examined cases.

Re-evaluation of histological findings of nonparasitic splenic cysts.

OBJECTIVE: The pathogenesis of nonparasitic splenic cysts (NPSCs) has not been clarified completely. The aim of this multinational and multicentre retrospective study was to further elucidate the origin of NPSCs. METHODS: From 1980 to 2006, 50 children and adolescents were surgically treated for NPSC at six paediatric surgical centres in four European countries. The initial histology report of 35 NPSCs, 22 epidermoid cysts, 11 pseudocysts or post-traumatic cysts and two mesothelial cysts was available. Additional re-evaluation, including immunohistochemistry, to detect cytokeratin, carcino-embrionic antigen and mesothelioma antibody in the inner surface of the cysts was carried out. Special attention was given to the possibility of preceding trauma to the splenic area and whether it played a role in the genesis of NPSC. RESULTS: The pathological re-evaluation showed 30 epidermoid cysts, four mesothelial cysts and one pseudocyst. Immunohistology revealed eight epidermoid and two mesothelial linings of the cysts in those 11 patients in whom pseudocyst was diagnosed originally. No pseudocyst was documented in those patients who had a history of previous blunt abdominal trauma but was not proved by ultrasound and computed tomography scan. CONCLUSION: In contrast with the prevailing belief, it has been demonstrated that NPSCs are congenital in origin, and there is no clinically proven evidence that trauma does play a role in their genesis.

Interrupted inferior vena cava syndrome.

We report a case of interrupted inferior vena cava (IVC) as a rare developmental defect. Inferior vena cava interruption is usually accompanied with azygos and hemiazygos continuation, and is asymptomatic. Consequently, venous blood from the caudal part of the body reaches the heart via the azygous vein and superior vena cava. A 50 year old female who came for routine health check-up was found to have pulmonary hypertension on two dimensional echocardiography. On further investigations she also had restriction on pulmonary function test. When computed tomography pulmonary angiography was done, showed dilated azygous vein without pulmonary embolism. Computed tomography of the abdomen demonstrated interrupted inferior vena cava. Such patients are at increased risk of deep vein thrombosis and pulmonary embolism.

[Management of gastrointestinal malformations in children with asplenia syndrome].

Asplenia syndrome (AS) is a complex disorder that includes a variety of cardiovascular and gastrointestinal malformations (GMs), and its prognosis remains poor. We treated 40 AS patients between 2000 and 2010, 10 of whom underwent surgery to correct GMs. These comprised 7 hiatus hernias (HH), 1 intestinal malrotation (IM), 1 stomach volvulus, and 1 ompalocele. In this review, we consider the optimal surgical timing and methods of management, especially for HH and IM. Surgery was performed in 6 of 7 HH patients. Four of those underwent laparotomy, 1 thoracotomy, and 1 laparoscopy. The Toupet method or fixation of the His angle or hiatus closure was selected in each case. Because the stomach intruding into the mediastinum may cause pulmonary venous obstruction and respiratory impairment, surgical repair is preferably performed prior to Glenn surgery. Among the 9 IMs, 8 were nonrotational and required no treatment, and only 1 required surgical treatment. GMs in AS are difficult to manage because of the unique anatomy, meaning there are no fixed surgical techniques. Each patient must be carefully examined preoperatively, and the optimum surgical method selected. Close collaboration with cardiovascular surgeons is vital when determining the timing and method of surgery.

Complicated congenital splenic cyst: saved by a splenunculus.

A 12-year-old girl presented with a large congenital splenic cyst complicated by Salmonella organisms. After failure of conservative management and percutaneous drainage, a splenectomy was performed. An incidental splenunculus was preserved. On follow up the splenunculus had increased to normal splenic size and there was no evidence of Howell-Jolly bodies, suggesting normal splenic function.

Anesthesia for pheochromocytoma resection in a child with Fontan circulation.

PURPOSE: To report the anesthetic management of a successful resection of a pheochromocytoma in a child with a completed Fontan circulation. CLINICAL FEATURES: The patient was an 11-yr-old boy with Ivemark syndrome who had undergone Fontan palliation at three years of age. Six weeks earlier, he had been diagnosed with a norepinephrine-producing pheochromocytoma, and he had been pretreated with oral propranolol and phenoxybenzamine. During surgery, intravenous administration of magnesium sulphate, esmolol, and phentolamine provided good hemodynamic control. Postoperatively, the patient tended to be hypotensive, and treatment with fluid administration resulted in prolonged intensive care. CONCLUSION: Although intraoperative management was not problematic, postoperative care of this 11-yr old child with pheochromocytoma was complicated by residual sympathetic blockade.

Congenital splenic cyst - case study.

BACKGROUND: Splenic cysts represent a pathology seldom encountered in practice, the most often etiology being, parasitic, congenital and pseudo-cysts, which usually appear post-traumatically or after a splenic infarction.Splenic cysts indicate a surgical treatment when they are large (and thus present a high risk for complications such as rupture, hemorrhaging, compression of the neighboring organs), when they are symptomatic or present complications. CASE REPORT: We present the case of a patient diagnosed with splenic cyst in our Clinic, its discovery being incidental, while conducting investigations for an abdominal pain syndrome. DISCUSSIONS: The clinical case presented completely abides to the literary description-the diagnostic has been incidental, the large size of the cyst has determined the need for surgery (splenectomy), its etiology has been established by means of anatomo-pathological report (the presence of the epithelial inner wall that indicates congenital cysts).