Journal of Pathology and Translational Medicine

Case Report

Congenital Pulmonary Lymphangiectasia, Associated with Total Anomalous Pulmonary Venous Return.: Seong Wook Hwang, Mee Seon Kim, Po Eun Park, Tae In Park; Korean J Pathol. 2011;45(6):650-653.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.6.650

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Abstract PDF: Congenital pulmonary lymphangiectasia (CPL) is very rare. It shows diffuse pulmonary lymphatic dilatation without lymphatic proliferation. CPL can occur as a primary disorder or arise secondarily from other diseases such as the obstruction of pulmonary veins or lymphatics. The prognosis of CPL is very poor. Approximately 50% of infants are stillborn and most others usually die within the first day of life. The present case showed diffuse lymphangiectasia in the subpleural, interlobular, and peribronchovascular areas. The flat lining cells were immunohistochemically positive for D2-40 and CD31. CPL is usually diagnosed by clinicoradiological or postmortem examinations. However, our case was diagnosed by an antemortem lung biopsy. We report a case of CPL with total anomalous pulmonary venous return.

Original Articles

Congenital Anomalies Observed by Autopsies at the Seoul National University Children's Hospital.: Jin Haeng Chung, Jeong Wook Seo, Chong Jai Kim, Chul Woo Kim, Je G Chi; Korean J Pathol. 1997;31(2):93-99.

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Abstract PDF: A retrospective analysis was performed on the 968 cases of fetal or pediatric autopsies over five year period (1990-1994), at the Seoul National University Children's Hospital. Age/mode distribution of cases were artificial abortus(30.6%), spontaneous abortus(12.0%), stillbirth(21.9%), neonates(29%), infants(2.8%) and children(0.9%). Male/female ratio was 1.21. Overall incidence of congenital anomalies was 60.8% and 34.0% of all cases had anomalies involving multiple organ systems. Percentage of cases with any anomaly was 71.6% in artificial abortus, 35.3% in spontaneous abortus, 59% in still births, 65.5% in neonates and 38.9% in infant and children. Common organ systems involved were the cardiovascular system (39.0%), musculoskeletal system (23.6%), nervous system (22.6%), gastrointestinal system (19.9%), and urinary system (14.6%). From these results, we found that the congenital anomalies were most significant diseases of the perinatal period and the cardiovascular anomalies were the most common anomalies of them.

Morphohistometric Investigation and bcl-2 Expression in the Placenta of Chromosomally Abnormal Pregnancy.: Joung ho Han, Kyu Rae Kim, Yeon Lim Suh, Mi Kyung Kim, Young Hyeh Ko, Dae Shick Kim, Howe Jung Ree; Korean J Pathol. 1999;33(5):353-360.

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Abstract PDF: To evaluate the significance of placental histology, a collaborative histological and cytogenetic study was performed on the products of 88 spontaneous abortions, and subsequently bcl-2 immunostaining was performed on 62 cases. The morphometric parameters included were DCIRCLE, FORMSHAPE, CPRATIO, and the expression of bcl-2 immunostainig was graded in four categories (I to IV). The results were as follows: 1) 40% (n=35) were chromosomally abnormal: trisomies predominated (57%, n=20) and was followed by triploidy (14%, n=5), double trisomy (6%, n=2), monosomy X (6%, n=2), inversion (9) (6%, n=2). 2) mean of DCIRCLE in chromosomally abnormal pregnancy was 40 micrometer larger than that in chromosomally normal pregnancy (p=0.012, one side t-test), while no difference was found in FORMSHAPE and CPRATIO between chromosomally abnormal and normal pregnancy. 3) bcl-2 expression was found in syncytiotrophoblast and cytotrophoblast. bcl-2 expression was weaker in chromosomally abnormal pregnancy with intensity I and II of 59% than chromosomally normal pregnancy with intensity I and II of 24%. 4) In comparison bcl-2 expression with DCIRCLE, in chromosomally normal abortion one (10%) in I & II and one (3%) in III & IV showed large DCIRCLE (above 360 micrometer), while 11 (85%) in I & II and 3 (33%) in III & IV in chromosomally abnormal pregnancy. It would mean that bcl-2 protein is necessary in preservation of pregnancy and placental morphology. Abnormal villous diameter and weak bcl-2 expression may be suggestive of chromosomal anomaly. Besides other histologic parameters, application of bcl-2 immunostaining and morphometric analysis probably give more sensitive and specific results in identifying chromosomally abnormal abortion.

Case Reports

Aqueductal Atresia with Forking Anomaly: Report of 3 cases.: Na Hye Myong, Mi Kyung Kim, Je G Chi; Korean J Pathol. 1994;28(5):514-521.

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Abstract PDF: Aqueductal forking was first described by Russell (l949) as a cause of aqueductal obstruction and a form of congenital malformation with simple stenosis, it is a relatively common cause of congenital hydrocephalus not associated with spina bifida or meningomyelocele. Pathologically it is characterized by two distinct channels separated by non-gliotic brain tissue. We describe variable clinicopathologic findings of 3 autopsy cases showing hydrocephaly due to aqueductal atresia with forking case 1 was a 35-week-old female showing Potter's syndrome, dextrocardia, and skeletal anomaly. case 2 was a 29-week-old male abortus with micrognathia, simian crease, club feet, and minor defects of visceral organs. Case 3 was a 32-week-old female abortus with associated anomalies such as a low-set ear, ectopic thymus and thyroid, and Meckel's diverticulum. On serial sections of brain stems of all 3 cases, were seen variably shaped and atretic lumina of aqueducts with distinct two channe1s and intervening brain tissues of normal cellularity.

Sirenomelia: An autopsy case report.: Yeong Ju Woo, Hye Kyoung Yoon, In Sook Lim; Korean J Pathol. 1994;28(1):96-98.

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Abstract PDF: Sirenomelia is a severe form of caudal regression syndrome that results in a fusion of the lower extremities, which is not compatible with life. A various spectrum of anomalies affecting primarily the musculoskeletal, genitourinary and gastrointestinal systems also can occur. This rare malformation has a reported incidence rate of approximately 1 in 60,000 births, with a range of 1 to 1 percent of all malformed infants. We experienced a sirenomelic case with combined anomalies of genitourinary, cardiovascular and gastrointestinal systems. Maternal obstetric history revealed oligohydramnios and intrauterine fetal growth retardation, and the baby was spontaneously delivered at 37 weeks of gestational age, but died I hour after birth.

Original Articles

Scanning Electron Microscopic Findings of Hair Anomalies.: Chul Jong Yoon, Je G Chi, Hai Won Chung; Korean J Pathol. 1993;27(5):491-500.

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Abstract PDF: Scanning electron microscopic(SEM) examination of hair is an efficient and definite method for the diagnosis of hair anomaly. The Hair specimen is placed on a stub, gold coated, and directly viewed without prior fixation or treatment for identification. We have analyzed 25 cases of scalp hair and 2 cases of eyebrow that were sampled with the suspicion of hair anomalies at Seoul National University Hospital during the last 7 year period from January 1988 to June 1992. All 27 cases had certain abnormalities of the hair. They consisted of monilethrix(1 case), hair casts(4 cases), pili torti(1 case), uncombable hair syndrome(1 case), trichorrhexis nodosa(5 cases), woolly hair(5 cases) and other anomaly(1 case). Some cases of them were mixed with two or three anomalies in hairs. Their combinations were; hair casts and woolly hair(1 case), hair casts and pili torti and uncombabla hair syndrome(1 case), hair casts and uncombable hair syndrome and trichorrhexis nodosa(1 case), trichorrhexis nodosa and uncombable hair syndrome(3 cases). Most of these defects included brittleness of scales and distortion of the hair shaft.

Body Stalk Anomaly: Analysis of 10 Autopsy Cases.: Seung Sook Lee, Je G Chi; Korean J Pathol. 1993;27(3):235-242.

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Abstract PDF: 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.

Case Reports

Agenesis of the Dorsal Pancreas: An autopsy case.: Won Sang Park, Ki Hwa Yang, Seok Jin Kang, Byoung Kee Kim, Sun Moo Kim; Korean J Pathol. 1992;26(1):71-75.

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Abstract PDF: Agenesis of the dorsal pancreas is one of the rare congenital anomalies of the pancreas. Six cases of them have been reported. We have experienced an autopsy case of agenesis of the dorsal pancreas associated with fetal death in the uterus. Grossly, the body and tail of the pancreas and uncinate process were not found and those were partially replaced by adipose tissue. No abnormality was noted in the other organs. Microscopically, pancreatic tissue with autolytic change was identified only in the head portion of the pancreas.

Amniotic Band Syndrome: An autopsy case report.: Hye Seon Ahn, Gil Ro Han, Jin Hee Sohn, Jung Il Suh; Korean J Pathol. 1989;23(4):482-486.

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Abstract PDF: We report an autopsy case of amniotic band syndrome exhibiting microcephaly, asymmetric encephalocele, microphthalmia, nasal deformity, cleft lip and palate accompanied by left maxillary and zygomatic bone deformities. The amniotic membrane of the placenta was also attached to the herniated brain. The twenty-year-old primigravid mother had no history of taking drug, irradiation, infection or trauma before or during pregnancy.

A Case of Ocular Neurofibromatosis.: Je G Chi, In Ae Park; Korean J Pathol. 1987;21(1):62-65.

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Abstract PDF: Neurofibromatosis is one of the neurocristopathies that involve many system or tissues forming various types of lesion. Almost every tissue or organ can be involved by this disease. However, the eyeball itself is very rarely affected by this process. The findings seen in our case indicate the diversity of lesions in neurofibromatosis, and also suggest hamartomatous nature. Heterotopic ganglion cells and glial cells in uveal tract are not easily understood. We reported here a case of intra-occular neurofibromatosis with its characteristic involvement of the uveal tract, in a 21 year old female. Her ocular symptoms began at her age of 11 as poor vision and were slowly progressive together with multiple facial neurofibromas. The involved left eyeball showed many ganglioneuroglial cell nests in iris, ciliary body and retina. Minute plexiform neurofibromas were also seen in small nerve twigs around the eyeball.

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