Pelvic lipomatosis is a rare disease of unknown cause, characterised by overgrowth of benign mature fat cells commonly located in the perivesical and perirectal spaces. The disease is rare amongst Asian population and has a wide range of presenting symptoms varying from lower urinary tract symptoms to bowel symptoms. However, this disease can be easily diagnosed based on certain characteristic radiological imaging techniques. We report a case of pelvic lipomatosis in an asymptomatic Chinese gentleman, who presented with classical radiological features of pelvic lipomatosis but provided us with challenging therapeutic scenario. (JUMMEC 2010; 13(2): 111-114)

Three autopsy cases of arthrogryposis multiplex congenita are studied. They were two deadborns and one neonatal death. All of them had characteristic abnormalities involving multiple joints. Neither primary myopathy nor abnormalities of anterior horn cells of the spinal cord were detected in our cases. However, two cases had minor central nervous system anomalies. All four cases showed pulmonary hypoplasia of varying degree. Two of three cases showed facial dysmorphism such as micrognathia and low set ears, and one showed cleft lip and palate. Ventricular septal defect, umbilical hernia and ureteral anomalies were also associated.
Infant, Newborn ; Humans

Body stalk anomaly represents an extreme maldevelopment of embryonic body folding and is characterized by absence of the umbilicus and umbilical cord. The failure of complete obliteration of the extraembryonic coelom is responsible for the absence of the umbilical cord formation and the wide-based insertio of the amnioperitoneal membrane onto the placental chorionic plate. We have analyzed 10 autopsy cases of various midline anomalies of the body that could best be classified into body stalk anomaly. All cases were either stillborns or dead immediately after birth. The pregnancy was interrupted due to this anomaly in 6 cases, and their gestational ages varied from 17 weeks to 37 weeks. The affected fetuses were characterized bt absent or vestigial umbilical cord, and ruptured amnion with direct amnioperitoneal connection without the mediation of the umbilical cord. Exomphalos with abdominal wall defect and serve scoliosis were characteristic components of this anomaly, that provided important clues in differentiating other similar anomalies. Other associated anomalies included neural tube defect, intestinal atresia, genitourinary and skeletal defects, pulmonary hypoplasia, single umbilical artery and narrow-spaced chest and abdomen, etc. These findings strongly suggest that anomaly of body stalk represents mechanical teratogenesis due to early amnion repture and subsequent effect, and should be categorized into amniotic band disruption syndrome.
Pregnancy ; Female ; Humans ; Teratogens

Foreign body reaction of tissues is frequently encountered in routine surgical pathology, whether they contain foreign body or not. Though their presence or identification may be the important clue for the legal problems as in malpractice, and the identification of foreign body will give us the chance for reconstruction of patients' medical history, pathologists often overlook or neglect what they are. Besides curiosity, the description of individual foreign body and common site for its occurence will be of great help. Therefore, we classify and describe the features of 182 foreign bodies collected in practice of surgical pathology during 1979~82.

Surgical Technique: The technique involves creating a central crater, flipping the nucleus out into the supracapsular space, and creating another crater through the posterior surface that will eventually connect with the anterior crater to produce a central hole. The donut-shaped nucleus is then chopped and the fragments are aspirated with greater ease. Results: The technique was performed successfully in seven eyes of six patients with only minor problems. Phacoemulsification times were longer than usual because of the additional sculpting, but clinical results were not adversely affected. Conclusion: For dense or hard cataracts for which the surgeon anticipates a tough posterior plate, this technique is a safe alternative to the usual chopping techniques.
Human ; Male ; Female ; Aged ; Middle Aged ; CATARACT ; PHACOEMULSIFICATION ; SCLEROSIS

BACKGROUND: Hydrops fetalis is defined as abnormal accumulation of serous fluid in two or more fetal compartments, and this malady is known to be associated with various pathologic conditions. METHODS: We collected 149 cases of hydrops fetalis out of 2,312 autopsies, and we tried to elaborate the underlying causes of hydrops fetalis. The diagnosis was based on the material from either antenatal termination or intrauterine death. RESULTS: The relative incidence of hydrops fetalis was 6.44% of all the pediatric autopsies we performed. The gestational age was evenly distributed from 18 to 33 weeks, except for 30 to 31 weeks. There was no sex difference in the incidence of hydrops fetalis. The main causes were cardiovascular diseases (30.9%), cystic hygroma (13.4%), chromosomal anomaly (8.05%), thoracic conditions (7.38%), followed by urinary tract malformation (4.03%), infection (4.03%) and anemia (3.36%). The most common chromosomal anomaly was Turner syndrome and the second one was Down syndrome. CONCLUSION: Since various conditions can be the cause of hydrops fetalis, pathologists should pay attention to elaborate the underlying causes in every single autopsy.
Anemia ; Autopsy* ; Cardiovascular Diseases ; Diagnosis ; Down Syndrome ; Edema* ; Fetus ; Gestational Age ; Hydrops Fetalis* ; Incidence ; Lymphangioma, Cystic ; Sex Characteristics ; Turner Syndrome ; Urinary Tract

The patient was a 7 months old Korean girl, who was admitted to Department of Ophthalmology of the Seoul National University Hospital, with 6 months history of the left eyelid swelling and exophthalmos. Visual acuity and ocular movement were unaffected. Orbit CT revealed 1.5x.5 cm sized well circumscribed intraconal mass in the left retrobulbar space with pressure erosion of adjacent bone. At surgery, the mass was pinkish gray and firm, and was adherent to adjacent tissue. The whole mass could not be removed, and a local excision was done.

VATER association represents vertebral defects, anal atresia, tracheo-esophageal fistula with without esophageal atresia, renal defects and radial limb dysplasia. The probability of the simultaneous occurrence of any three of these defects is so unlikely that it suggests a sporadic non-random association. This non-random association appears to be related to some chromosomal anomalies, the caudal regression syndrome, mesodermal defects in early developmental period or the matemal use of sex hormones during embryogenesis. We report three autopsy cases of the VATER association that showed most of the known major and minor defects as well as an unusual concurrence of other defects, i.e., scoliosis, talipes varus, absent penis, urethral agenesis and stenosis, rectourethral fistula, rib anomaly, single umbilical artery, Meckel's diverticulum, diaphragmatic hemia, absent rectum, short neck, simian crease, low set ear, and hypoplastic lung.

Five fetal maxillas were obtained from the autopsy file of fetal postmortem examination, and were examined by serial micro-sections of frontal plane and horizontal plane. Especially the area around the incisive canal of the maxilla was carefully observed. The results are as follows. 1) In 5 fetal maxillas extra-dental laminas and supernumerary tooth germs which are severely malformed in shape are found in the dilated incisive canal, where prominent vessels and nerves are distributed. 2) The supernumerary tooth germs disclose almost normal histo-differentiation of odontoblast and ameloblast, and there shows relatively abundant perifollicular fibrosis in the place of perifollicular bone. 3) It is observed that the over-growth of the extradental lamina from the dental ridge of deciduous central incisor frequently tends to direct toward the incisive canal that includes prominent vessels and nerves.

A three year old girl with a left renal teratoid Wilms tumor is reported. The tumor was located both inside and outside the kidney parenchyme, to form a well encapsulated mass containing two lobulated solid and cystic masses. The tumor consists predominantly of otherwise typical Wilms tumor irregularly mixed with teratoid tissue elements such as intestinal tract, mucous glands with argentaffin cells, goblet cells and transitional epithelium. These heterologous elements were regarded as diverse epithelial differentiation of totipotent cells in certain nephrogenetic period, and this tumor was considered to be hest called "teratoid Wilms tumor".