Hamartoma ; congenital ; pathology ; Humans ; Mesoderm ; Mouth ; Mouth Diseases ; congenital ; pathology

OBJECTIVETo analyze the clinical, imaging and pathological findings of congenital intrapulmonary lymphangioma and hemangioma in 5 infants and young children.

METHODData of 3 cases with congenital intrapulmonary lymphangioma and 2 cases with haemangioma were analyzed.

RESULTAll the 5 cases had cough, difficulty in breathing, cyanosis of lips, and shortness of breath. Imaging study indicated cystic and space occupying changes of partial lung. In the two cases of hemangioma, the blood vessels passing through the hemangioma were observed on CT films. Histochemical studies showed that the cystic pockets from removed tissues were different in size and there was a line of flat endothelial tissue around these pockets. Immunochemical studies indicated D2-40 positive, factor VIII weak positive for lymphangioma cases; while in hemangioma cases, factor VIII was positive, D2-40 was negative or weakly positive, and both of cytokeratin was negative.

CONCLUSIONIt is very important to consider and identify congenital intrapulmonary lymphangioma or hemangioma when a patient has cystic pockets and space occupying change in their lung by imaging and pathological studies.

Female ; Hemangioma ; congenital ; pathology ; Humans ; Infant ; Lung Neoplasms ; congenital ; pathology ; Lymphangioma ; congenital ; pathology ; Male

Child, Preschool ; Facial Asymmetry ; congenital ; pathology ; Growth Disorders ; congenital ; pathology ; Humans ; Hypertrophy ; congenital ; Infant ; Male

Congenital pulmonary lymphangiectasis (CPL) is a rare, poorly documented disease, characterized by abnormal dilatation of pulmonary lymphatics without lymphatic proliferation. This disease is seen almost exclusively in infancy and early childhood. It can usually be divided into primary (congenital) and secondary forms. The primary form presents in neonates, and the patients mostly die due to the respiratory distress, shortly after birth. The authors experienced two cases of primary CPL in a 13-day-old male neonate and a one-day-old male neonate, showing prominent lymphatic dilatation in the septal, subpleural, and peri-bronchial tissue throughout both lungs. The latter case was associated with congenital cardiac anomaly including single ventricle. These are unique cases of CPL in Korea of which the diagnosis was established through post-mortem examination. Therefore, the authors report these two cases with primary CPL with a review of the literature.
Humans ; Infant, Newborn ; Lung/pathology ; Lung Diseases/congenital/*pathology ; Lymphangiectasis/congenital/*pathology ; Lymphatic System/pathology ; Male

Congenital sternal foramen is an anomaly whose occurrence is rare in human but is especially unusual in animals. This defect was formed when fusion of multiple ossification centers was incomplete. It may be associated with other lesions in body organs especially cardiac anomalies. In the present study, we report a very rare case of congenital sternal foramen in a Holstein calf. The oval defect was like a gunshot wound and located at the lower third of the sternum. Apparently, the rest of skeleton system seems normal. The awareness of the anomaly is important for better diagnosis and treatment of diseases.
Animals ; Bone Diseases ; congenital ; pathology ; veterinary ; Cattle ; Cattle Diseases ; congenital ; pathology ; Congenital Abnormalities ; pathology ; veterinary ; Sternum ; pathology

The author has experienced a case of microphthalmos with large orbital cyst in a 4 months old female, that was found at the time of birth. To facilitate fitting a cosmetic prosthesis, the microphthalmos with cyst was removed surgically. On serial section I could find an area of discontinuation of the sclera that was suspected to be the defective closure of the embryonic cleft. Some aberrant retinal tissue was found in the wall of the cyst, and markedly disorganized ocular tissue forming a tumor-like mass filled the microphthalmic eyeball. In view of these histopathologic findings I could draw the conclusion that developmental failure of the embryonic eyeball and consequential proliferation of the embryonic neuroepithelial cells occurred at an early developmental stage causing the formation of microphthalmos with cyst.
Cysts/complications/congenital/*pathology ; Female ; Humans ; Infant ; Microphthalmos/complications/*pathology ; Orbital Diseases/complications/congenital/*pathology

Adult ; Female ; Humans ; Nevus, Pigmented ; congenital ; pathology ; Scalp ; pathology ; Skin Neoplasms ; congenital ; pathology

For hand surgeons, the treatment of children with congenital differences of the upper extremity is challenging because of the diverse spectrum of conditions encountered, but the task is also rewarding because it provides surgeons with the opportunity to impact a child's growth and development. An ideal classification of congenital differences of the upper extremity would reflect the full spectrum of morphologic abnormalities and encompass etiology, a guide to treatment, and provide prognoses. In this report, I review current classification systems and discuss their contradictions and limitations. In addition, I present a modified classification system and provide treatment principles. As our understanding of the etiology of congenital differences of the upper extremity increases and as experience of treating difficult cases accumulates, even an ideal classification system and optimal treatment strategies will undoubtedly continue to evolve.
Hand Deformities, Congenital/*classification/pathology/*surgery ; Humans

Congenital median dermoid is an uncommon disease. Surgery is the main curative treatment. To review a clinical case and to summarise the characteristics and treatment experience of this disease, referring to the related literature, it is expected that we can provide more clinical thought and therapeutic method for congenital median dermoid fistula of nasal dorsum.
Dermoid Cyst ; congenital ; therapy ; Fistula ; congenital ; therapy ; Humans ; Nose ; pathology ; Nose Neoplasms ; congenital ; therapy

Background: Open heart surgery with cardiopulmonary bypass (CPB) causes haemostatic abnormalities which result in postoperative excessive bleeding. Objectives: To investigate haemostatic disorders before - after CPB and postoperative bleeding. Subjects and methods: Sixty congenital cardiac patients with and without cyanosis were recruited consecutively. Hematology and coagulation tests were done 1 day before operation, 15 minutes after protamine administration, 2 and 6 hours after the operation in the intensive care unit (lCU). Mediastinal chest tube drainage (MCTO) was measured for the first 6h in the ICU. Results: Significant differences between 2 groups could be found for red blood cells, hemoglobin, hematocrit, fibrinogen, D-dimer, fibrinogen degradation products (FOP), von Kaulla and platelet aggregation to epinephrine before operation (p < 0.05). There were not significant differences in platelets, PT, APTT, platelet aggregation to adenosine diphosphate (AOP) in the acyanotic and cyanotic patients (p > 0.05). Most hematology parameters were decreased significantly and hemostasis measurements were prolonged after operation in both groups (p < 0.05). There was also the significant difference in MCTO between 2 groups (p < 0.05). Conclusion: Coagulation, hemostasis and fibrinolysis disorders; thrombocytopenia and acquired transient platelet dysfunction may be responsible for bleeding complications after CBP \r\n', u'\r\n', u'
Heart Defects ; Congenital/ pathology ; epidemiology ; Hemostatic Disorders/ pathology ; diagnosis