OBJECTIVETo improve the diagnostic accuracy of fetal pulmonary venous abnormalities through the analysis of the fetal pulmonary vein anatomy.

METHODS234 cases of congenital cardiac abnormalities were detected by echocardiography during pregnancy in An Zhen Hospital, Capital Medical University from May 2010 to August 2015. Autopsy was then performed. The type of fetal pulmonary venous malformation, cardiac abnormalities, systemic venous malformations, and other internal organs deformities were documented.

RESULTSThere were ninteen cases of pulmonary venous malformations among the 234 cases of fetal congenital heart disease. These included two cases of congenital pulmonary venous hypoplasia (CPVH) or atresia, four cases of partial anomalous pulmonary venous drainage (PAPVD), seven cases of total anomalous pulmonary venous drainage (TAPVD), five cases of atresia of common pulmonary vein (CPV), one case of congenital pulmonary venous hypoplasia with total anomalous pulmonary venous drainage. There were eleven cases with single ventricle, eight cases with right aortic arch, seven cases with single atrium and six cases with pulmonary valve stenosis. Eleven cases had pulmonary hypoplasia and nine cases had abnormal spleen.

CONCLUSIONSThere are many variations in pulmonary venous abnormalities associated with severe and complex cardiac abnormalities and internal organs malformation. Care should be exercised during autopsy examination to look for all branches of the pulmonary vein.

Autopsy ; Female ; Fetal Diseases ; Heart Defects, Congenital ; diagnosis ; Humans ; Pregnancy ; Pulmonary Veins ; abnormalities ; Spleen ; pathology

Splenogonadal fusion is a rare developmental anomaly in which an abnormal connection between the splenic tissue and gonads or mesonephric derivatives is present. Less than 200 cases have been reported since it was first described in 1883. Preoperative misdiagnosis is common and may lead to unnecessary orchidectomy if testicular neoplasm is suspected. To avoid such outcomes, it is important to be aware of the features of splenogonadal fusion. We report the case of a five-month-old male infant with continuous-type, left-sided splenogonadal fusion, which was discovered during groin exploration for a scrotal mass. Although the lesion was first noted during an episode of nonspecific viral fever, such an association is uncommon. Preoperative ultrasonographic evaluation favoured a diagnosis of a large haemangioma. This is the first reported case of splenogonadal fusion from Sri Lanka.
Diagnosis, Differential ; Humans ; Infant ; Male ; Spleen ; abnormalities ; surgery ; Splenic Diseases ; diagnosis ; surgery ; Testicular Diseases ; diagnosis ; surgery ; Testis ; abnormalities ; surgery

Funnel Chest ; complications ; Humans ; Infant, Newborn ; Kidney ; abnormalities ; Male ; Prune Belly Syndrome ; complications ; Situs Inversus ; complications ; Spleen ; abnormalities

Situs inversus totalis is a rare congenital disorder, which is total transposition of thoracic and abdominal organs. Its incidence is 1 in 10,000~50,000 live births. This might be associated with multiple abnormalities such as accessory spleen, asplenia, intestinal malrotation and so on. For this reason, in cases of operation in patients with situs inversus totalis, we need to scrutinize the presence of accompanied anomalies. Moreover, if Dieulafoy gastric bleeding has occurred, vascular anomalies can be accompanied. This 31-year-old male patient with situs inverses totalis was admitted to our hospital for management of UGI (upper gastrointestinal) bleeding. Gastroendoscopy revealed Dieulafoy disease in the upper body of the stomach as the cause of UGI bleeding. Several attempts with endovascular embolization and hemoclips were applied but failed. We performed a suture & ligation of the Dieulafoy lesion as well as total resection of accessory spleen with devascularization of prominently developed vessels around the upper stomach. We report this case with a review of the literature.
Abnormalities, Multiple ; Adult ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Hemorrhage ; Humans ; Incidence ; Ligation ; Live Birth ; Male ; Situs Inversus ; Spleen ; Stomach ; Sutures

Abnormalities, Multiple ; surgery ; Adolescent ; Humans ; Male ; Spleen ; abnormalities ; Testis ; abnormalities

Cerebral Infarction ; etiology ; Dextrocardia ; etiology ; Female ; Humans ; Infant ; Spleen ; abnormalities ; Syndrome

Abnormalities, Multiple ; diagnosis ; Adolescent ; Humans ; Male ; Spleen ; abnormalities ; Testis ; abnormalities

A 33-year-old man was admitted to our hospital with chest pain and exertional dyspnea. Two-dimensional echocardiography showed prominent trabeculations and deep intertrabecular recesses, findings consistent with noncompaction of the ventricular myocardium. Thoracoabdominal CT and cardiac magnetic resonance imaging (CMR) revealed situs ambiguous with polysplenia and noncompaction of the left ventricular myocardium. CMR also demonstrated delayed enhancement of the trabeculations located at the apical portion of the left ventricle. The coronary angiogram was normal. This is the first case of noncompaction of the ventricular myocardium associated with situs ambiguous with polysplenia.
Abnormalities, Multiple/*pathology ; Adult ; Echocardiography ; Heart Ventricles/abnormalities ; Humans ; Magnetic Resonance Imaging ; Male ; Myocardium/*pathology ; Spleen/*abnormalities ; Syndrome ; Tomography, X-Ray Computed

Autoimmune thrombocytopenic purpura (AITP) is an autoimmune disorder that results from antiplatelet autoantibodies; these autoantibodies cause platelet destruction in the reticluoendothelial system. Oral corticosteroid therapy is the first line treatment. Splenectomy is the major treatment modality after the failure of more conservative medical therapy. Approximately 15% of the patients will relapse either soon after splenectomy or, as is less common, many years later. The presence of an accessory spleen should be sought. We experienced a patient with a known diagnosis of autoimmune thrombocytopenic purpura who had a worsening thrombocytopenia 11 years after splenectomy. This patient was diagnosed with an accessory spleen. Accessory splenectomy was performed with only a transient elevation of the platelets. We report here on this case with a review of the literature.
Adult ; Female ; Humans ; Purpura, Thrombocytopenic, Idiopathic/*surgery ; Recurrence ; Spleen/*abnormalities/radionuclide imaging/surgery ; *Splenectomy

VACTERL association is occuring in conjuction with vertebral anomalies, anal atresia, cardiac abnormalities, tracheoesophageal fistula, renal agenesis and limb defects. Additional abnormalities may include microphthalmia, abnormal lung lobulation and spleen defects. We describe a infant born to consanguineous healthy parents with multiple congenital anomalies of the skeleton and internal organs. A woman at 25 weeks gestaion was transferred to our hospital due to abnormal antenatal sonographic finding(molded calvarium, huge mass in abdomen, hyperechoic intestine and femur bowing). She admitted to our hospital due to decreased fetal movement 3 weeks later. Fetal death in uterus was diagnosed by sonogram. Its phenotype displays imperforated anus, absence of genital organ, absence of utrethral opeining, disunion of maxilla and mandible, varus deformity of bilateral knee joints, widening of interphalangeal space in right foot. The diagnosis was confirmed by clinical features, radiological findings, and autopsy. We reported this case of VACTERL association with review of literatures.
Abdomen ; Anal Canal ; Anus, Imperforate ; Autopsy* ; Congenital Abnormalities ; Diagnosis ; Extremities ; Female ; Femur ; Fetal Death ; Fetal Movement ; Foot ; Genitalia ; Humans ; Infant ; Intestines ; Knee Joint ; Lung ; Mandible ; Maxilla ; Microphthalmos ; Parents ; Phenotype ; Skeleton ; Skull ; Spleen ; Tracheoesophageal Fistula ; Ultrasonography ; Uterus