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)

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

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

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

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.

This study is aimed to observe the expressions of different genes, including the extracellular matrix proteins, growth factors, and transcription factors during different developmental stages of mouse submandibular gland. Reverse transcription-polymerase chain reaction (RT-PCR) and the antisense inhibition in organ culture system were performed using mouse embryos and newborns. Total 140 mouse embryos (E14(80), E15(20), E16(20), E18(20)) and 30 newborn mice (D2(10), D3(10), D6(10)) obtained from 60 pregnant mice and 3 adult mice (3 weeks old) were used for the cDNA production and the salivary gland organ culture. Syndecan, perlecan, laminin alpha1 chain, TGF beta1, beta 3, and sonic hedgehog mRNAs were expressed in the early stage (E14~E16) of the submandibular gland development, whereas transglutaminase C (TGase C), E-cadherin, epimorphin, laminin beta2 and gamma1 chains, and HGF mRNAs were expressed in the middle and late stages (E16~E18, D2~D6). Antisense inhibition of different genes in the organ culture of E14 mouse embryos of submandibular gland showed specific growth retardation in the development of ductal and acinar cells. Especially, the antisense inhibition of perlecan, E-cadherin, laminin alpha1 chain, laminin beta2 chain, and syndecan mRNA arrested the growth of ductal and acinar cells. While the antisense inhibition of integrin beta5 greatly affected the acinar cell differentiation and also produced cystic dilatation of salivary ducts, the antisense inhibition of fibronectin showed aberrant growth of ectomesenchymal tissues of the mouse submandibular gland.
Acinar Cells ; Adult ; Animals ; Cadherins ; Dilatation ; DNA, Complementary ; Embryonic Structures ; Extracellular Matrix Proteins ; Fibronectins ; Gene Expression* ; Hedgehogs ; Humans ; Infant, Newborn ; Intercellular Signaling Peptides and Proteins ; Laminin ; Mice* ; Organ Culture Techniques* ; RNA, Messenger ; Salivary Ducts ; Salivary Glands ; Submandibular Gland* ; Syndecans ; Transcription Factors

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

Experimental allergic encephalomyelitis (EAE) has been a well established animal model of postvaccinatal demyelinating diseases occurring in humans. Therefore elucidation of its pathogenesis would be very critical for the understanding of various human demyelinating diseases including multiple sclerosis. This study was performed to characterize the infiltrating cells in inflammatory sites and analyze the nature of the damage of blood brain barrier in experimental allergic encephalomyelitis. Experimental allergic encephalomyelitis was produced by administering homologous spinal cord homogenate together with complete Freund's adjuvant in guinea pigs. Immunostainings on guinea pig IgG, IgM, IgA and muramidase were performed by peroxidase-antiperoxidase or indirect immunofluorescent methods. The blood-brain barrier change was assessed by administering fluorescent Evans blue. Following results were made. In juvenile animals, both clinical findings and histopathologic changes were first noted by 3 weeks after injection and progressed during the whole experimental period. However, these findings were delayed in onset and low in incidence in adult animals. The clinical and pathologic changes started from the caudal portions and extended rostrally. The blood-brain barrier (BBB) was damaged and progressed starting also from the caudal portion of the spinal cord. The BBB changes were more severe in young animal than adult animals. Those changes preceded th histologic alterations. It is suggested that the BBB susceptibility is responsible for the caudal onset of histologic changes. Although the lesion has been thought to be induced by T-cell mediated hypersensitivity, infiltrating cells consisted mainly of muramidase positive histiocytes. A few immunoglobulin positive B cells or plasma cells could also be demonstrated in the lesion. The former usually infiltrated the parenchyme and the latter remained around the small or medium-sized vessels.
Adult ; Male ; Female ; Humans ; Animals ; Incidence

We examined sixty-three human embryos ranged from three weeks to eight weeks of fertilization age and 117 human fetuses from eleven weeks to fourty weeks of gestational age. Anatomical structure of developing tongue could be classified into eight developmental stages. The first is the sgage of mesial swelling of tongue primordium in the fertilization age of 28~40 days (Streeter stage 13~16), the second is the stage of lateral swelling of tongue primordium in the fertilization age of 41~46 days (Streeter stage 17~18), the third is the sgage of vertical positioning of tongue in the fertilization age of 47~53 days (Streeter stage 19~21), the fourth is the transitional stage of tongue from vertical position to horizontal position in the fertilization age of 54~56 days (Streeter stage 22~23), the fifth is the stage horizontal positioning of tongue in the gestational age of 11 weeks, the sixth is the stage of protrusion of tongue in the gestational age of 12 weeks, the seventh is the stage of maturation of tongue muscle in the gestational age of 7-10 months. The development of tongue papilla characteristically progresses into three stages. The first stage is the epithelial ingrowth for the crypt formation, the second stage is the anatomical formation of vallate, fungiform and filiform papillae, and the third stage is the differentiation of taste buds in the vallate and fungiform papillae or the formation of thick spike-like keratinization at the tip of filiform papilla. We observed that the tongue primordium mainly derived from occipital myotome developed more repidly than other oro-facial structures, so it transitionally occuied the spaces of the pharynx and the posterior nasal cavity, and directly affected the formation of palate and the growth of maxilla and mandible. Whereas the tongue papilla development showed continuous developmental sequences during the fetal period.
Humans