Laryngeal atresia is a very rare congenital anomaly requiring immediate tracheotomy. We present a case of laryngeal atresia with tracheoesophageal fistula who showed immediate respiratory difficulty after ligation of umbilical cord and died of aspiration pneumonia at 8 days of age. The atretic portion of larynx is composed of irregulary arranged cartilaginous tissue, bundles of intrinsic muscle and soft tissue without epithelium-lined lumen. The lungs show normal development and evidences of aspiration pneumonia.
Infant, Newborn ; Humans

The umbilical cord length may be a reliable indicator of fetal activity during gestation because it grows in response to tensile forces related to fetal movement. But there has been little attention to normal range of the umbilical cord length. We analyzed the umbilical cord length of 2342 cases de1ivered in Seoul National University Hospital to determine mean values according to the gestational age. The umbilical cord length increased linearly from 15.5cm of 13 week to 50.6 cm of 44 week. After 42 week, the growth was nearly stopped. There was no difference by fetal sex. The length of umbilical cord was variable even within same gestational age.

Amputation through the epiphyseal plate is very rare. Authors experienced only one case of ankle amputation through the epiphyseal plate in one huadred seventeen replantation cases during last seven years. It can make severe growth disturbance after replantation especially if the bone shortening is carried out at epiphyseal plate. Authors have developed new idea to make boae shortening during replantation with preservation of the epiphyseal plate in amputation through the epiphyseal plate in three years old female. It is, so called, double osteotomy shortening. Bone shortening is performed at 5cm above amputated epipyaeal plate to avoid further damage in epiphyseal plate, and a bone segment 3cm in length is resected,by double osteotomy shortening technique. After then, bone reduction and internal fixation is followed. With above technique, authors succeeded in replantation without further damage in epiphysis. In three years and ten months follow-up, bony growth in replanted tibia shows normal pattern, and epiphyseal plate in replanted site is working and remained open. The patient can walk without pain, and motor-sensory function have almost completely recoverered.
Amputation ; Ankle ; Epiphyses ; Female ; Follow-Up Studies ; Growth Plate ; Humans ; Osteotomy ; Replantation ; Tibia

No abstract available.
Arthritis, Infectious* ; Hip Joint* ; Hip*

Infantile myofibromatosis with systemic involvement is a very rare disease and is characterized by numerous nodules composed of spindle cells of a myofibroblastic nature. There are often disseminated throughout the subcutis, muscle, skeleton and viscera. We report an autopsy case of infantile myofibromatosis in a stillborn female fetus of 32 weeks of gestation. The nodules, Imm to 2 cm, were found over the whole body and viscera. The involved viscera were the heart, tongue, esophagus, gastrointestinal tract, portal areas of the liver, spleen anc pancreas. There were also associated malformations, viz., frontal meningoencephalocele, flexion defer-mities, syndactyly, cleft palate, agenesis of corpus callosum, pachygyria, diaphragmatic hemia, renal hypoplasia, etc. Multiple basaloid follicular hamartomas of the skin were noted on the face and extremeties. There are no previous reports in the literature of infantile myofibromatosis in conjunction with the above skin lesion and congenital malformations.
Infant ; Male ; Female ; Humans ; Hamartoma

Two cases of urachal anomaly (1 urachal cyst and 1 patent urachus) are reported in a neonate and an eleven-year old boy, respectively. In case 1, the patient was born after an uncomplicated pregnancy to a mother who had taken progesterone during the first trimester. Because of breech presentation, cesarian section was elected to deliver a male baby weighing 2.3 kg who showed abdominal distension. The patient died of respiratory difficulty several minutes after birth. At autopsy, there was a large cyst in the midpoint of the abdominal and pelvic cavity. This round cyst was composed of two components, urachus and urinary bladder. No area of umbilicocystic fistula was present. The lining epithelium was chiefly of transitional type. Assocaited anomalies were segmental stenosis of posterior urethra, absence of abdominal musculature, bilateral polycystic kidney of Potter type IV, hydroureter, and hypoplasia of lungs. Low set ears, micrognathia and club foot were also present. In case 2 the patient was 11-year old boy. He had suffered from intermittent urinary dribbling from umbilicus since early infancy, whenever the abdominal pressure was increased. The patency of urachus was confirmed by fistulography. And the urachal anomaly was surgically removed. Histopathologically the resected patent urachus consisted of pseudostratified columnar to transitional epithelium resting on fibrous stroma mixed with well formed smooth muscle bundles.
Pregnancy ; Female ; Male ; Infant, Newborn ; Humans ; Cysts

The 26 year old male with the hypertrophic cardiomyopathy confirmed by M-mode and 2-D echocardiography is reported with the review of the literatures.
Adult ; Cardiomyopathy, Hypertrophic* ; Echocardiography ; Humans ; Male

No abstract available.
Animals ; Brain* ; Mice* ; RNA, Messenger*

No abstract available.
Adult ; Hidradenitis ; Humans

No abstract available.
Lung* ; Tracheoesophageal Fistula*