Incontinentia pigmenti (IP) is a rare multisystemic ectodermal disorder, which is characterized by vesicular, verrucous, and pigmented cutaneous lesions, and is frequently associated with various developmental defects of the eye, CNS, teeth, hair, and nail. It is regarded as an X-linked dominant genetic disorder. We recently experienced a case with IP, who presented with irregular, reticular, and slate-gray to brown colored pigmentation on the whole body at birth. Skin lesions were much improved by 6 month of age. The mother of this infant had the history of same cutaneous lesions in her neonatal period, suggesting that these lesions had familial tendency.
Ectoderm ; Hair ; Humans ; Incontinentia Pigmenti* ; Infant ; Mothers* ; Nuclear Family* ; Parturition ; Pigmentation ; Skin ; Tooth

Traditionally postpartum hemorrhage is a bleeding more than 500 ml that occurs immediately after the placenta is delivered. It remains one of the most common causes of maternal mortality. Morbid adhesion of the placenta is emerging as a major cause of massive postpartum hemorrhage unresponsive to medical therapy. We experienced three cases of major postpartum hemorrhage over 5,000 ml, developed from anterior placenta previa totalis with adhesion of placenta. The hemorrhage had been controlled successfully by compressing abdominal aorta intermittently with the operator's hand during Cesarean hysterectomy. In immediate life- threatening postpartum hemorrhage this simple and safe technique can be used in slowing the bleeding while stabilizing the patient and preparing for definitive treatment.
Aorta, Abdominal ; Hand ; Hemorrhage ; Humans ; Hysterectomy ; Maternal Mortality ; Placenta ; Placenta Previa ; Postpartum Hemorrhage* ; Postpartum Period*

Ultrasonograhy of pancreas has been already established to be a effective method in the evaluation ofpancreatic mass lesion. In addition to ultrasonography, ultrasonoguied percutaneous fine needle aspiration biopsyof pancreatic mass has been known to be a safe method of obtaining tissue diagnosis in patients with pancreaticcancer without operation. From March, 1984 to June, 1986 ultrasonography and ultrasonoguided percutaneous fineneedle aspiration biopsy were peformed in 40 patients who had been finally diagnosed as pancreatic cancer at theDepartment of Radiology, Yonsei University College of Medicine. The results were summarized as follows: Inultrasonographic findings of pancreatic cancer, (1) The location of pancreatic mass in 19 cases in head, 3 in headand body, 7 in body, 4 in body and tail, 5 in tail and 2 diffuse type. (2) The size of tumor ranged under 2cm in 2cases, between 2.1–4cm in 21, between 4.1–6cm in 13 and over 6cm in 4 cases. (3) The other ultrasonographicfindings were 6 cases common bile duct dilatation, 4 pancreatic duct dilatation, 10 peripancreatic nodeenlargements, 16 liver metastatsis and 3 stomach involvement. In ultrsonoguided percutaneous fine needleaspiration biopsy of total 40 cases of pancreatic cancer. (4) The cytologic diagnosis were postive malignant cellin 31 cases( 77.5%), highly suspicious malignant cell, but inconclusive in 3(7.5%), negative malignant cell in 2(5.0%) and material insufficiency in 4 cases(10%) (5) No significant complication durinng or after procedure wasfound.
Biopsy ; Biopsy, Fine-Needle ; Biopsy, Needle ; Common Bile Duct ; Diagnosis ; Dilatation ; Head ; Humans ; Liver ; Methods ; Pancreas ; Pancreatic Ducts ; Pancreatic Neoplasms ; Stomach ; Tail ; Ultrasonography

In hydranencephaly, the cerebral hemispheres are absent or represented by membranous sacs with remnants of frontal, temporal or occipital cortex dispersed over the membrane. The brain stem is relatively intact. The cause of hydranencephaly is unknown, but bilateral occlusion of the internal carotid arteries during early fetal development can explain most of the pathologic abnormalities. We evaluated a case of hydranencephaly by magnetic resonance (MR) angiography. MR angiography shows both common, external carotid and vertebrobasilar arteries with no delineation of both internal carotid arteries from their origins. A brief review of the related literature was given on this subject.
Angiography ; Arteries ; Brain Stem ; Carotid Artery, Internal ; Cerebrum ; Fetal Development ; Hydranencephaly ; Magnetic Resonance Angiography* ; Membranes

The Holt-Oram syndrome or cardiomelic syndrome is characterized by the association of upper limb and heart malformations. Most frequently, abnormalities of the thumb and secundum atrial septal defects are associated with the disease. The mode of inheritance is autosomal dominant. The etiology of this disease is unknown but is most likely of multifactorial origin. Here we report a case Holt-Oram syndrome with affected mother which was diagnosed at 18th gestational weeks by prenatal ultrasonograpy. Fetal ultrasonography revealed abnormalities of upper limbs, and heart. The upper limbs were shorter than normal, syndactyly of both hands were seen and both wrists were markedly angulated. Ventricular septal defect was suspicious. After genetic counselling her pregnancy was terminated at 22nd gestational week.
Hand ; Heart ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Mothers* ; Pregnancy ; Syndactyly ; Thumb ; Ultrasonography* ; Ultrasonography, Prenatal ; Upper Extremity ; Wills ; Wrist

BACKGROUND: The skin temperature is often decreased during anesthesia because of a cool ambient temperature in the operating room. Contractility of the muscles may be affected by lowering the muscle temperature. This study was designed to verify that efforts to maintain normothermia on the monitored arm can make recovery from deep neuromuscular blockade more reliable. METHODS: A total of 60 patients were enrolled in this study. Each patient was randomly assigned to group 1 (the monitored arm was shielded with a passive warming protector) or group 2 (the monitored arm was was exposed to the ambient operating room temperature). Conventional inhalation anesthesia was induced with propofol and alfentanil, and this was maintained with O2, N2O and isoflurane. The twitch response of the adductor pollicis muscle was recorded. After calibration of the TOF watch(R), 0.6 mg/kg of rocuronium was injected. During anesthesia, the post tetanic count (PTC) was checked every 5 to 6 minutes until the first response to a train-of-four (TOF) stimulations appeared. The ambient operating room temperature, the core temperature and the skin temperature were checked simultaneously. RESULTS: The skin temperature of group 2 was lower than that of group 1 (33.89 +/- 0.81 degreesC and 35.41 +/- 0.45 degreesC, respectively, P < 0.05). When the data was plotted with the equation y=be(-ax), this equation well represented the data of group 1 (R2 = 0.82), but it did not well represent the data of group 2 (R2 = 0.54). CONCLUSIONS: During recovery from deep neuromuscular blockade with using rocuronium, it may be desirable to maintain normothermia at the thenar area for a better recovery time from a given PTC.
Alfentanil ; Androstanols ; Anesthesia ; Anesthesia, Inhalation ; Arm ; Calibration ; Factor IX ; Humans ; Hypothermia ; Isoflurane ; Monitoring, Intraoperative ; Muscles ; Neuromuscular Blockade ; Operating Rooms ; Propofol ; Skin Temperature

The common carotid artery is an artery which supplies the head and neck with oxygenated blood. Although unilateral common carotid artery occlusion or bilateral internal carotid artery occlusion have been reported, the incidence of both common carotid artery occlusion is very rare. As previous report which reviewed 5400 carotid duplex ultrasonograms, 2.5% of internal carotid artery occlusion, 0.24% of unilateral common carotid artery occlusion and none of bilateral common carotid artery occlusion were reported. Common carotid and subclavian arteries are important in the blood supply to the vasculatures of head and upper extremities. Bilateral common carotid artery occlusion might be a cause of stroke, transient ischemic attack or other neurologic sequalae. Cerebral oximetry is a simple method of measuring regional cerebral oxygen saturation (rSO2), which appears to reflect changes in cerebral perfusion and it has been increasingly applicated in many clinical situations such as vascular surgeries involving head/ neck and operations adopting cardiopulmonary bypass. This case describes a successful anesthetic management in a patient with occlusion of bilateral common carotid and subclavian arteries using continuous cerebral oxygenation monitoring during laparoscopic cholecystectomy.
Anesthesia ; Arteries ; Cardiopulmonary Bypass ; Carotid Artery, Common ; Carotid Artery, Internal ; Cholecystectomy, Laparoscopic ; Equipment and Supplies ; Head ; Humans ; Incidence ; Ischemic Attack, Transient ; Neck ; Oximetry ; Oxygen ; Perfusion ; Stroke ; Subclavian Artery ; Upper Extremity

PURPOSE: Brainstem auditory evoked response(BAER) is used as screening test for hearing disorders, damages of the central nervous system and congenital anomalies. We studied the difference values according to gender and stimulation sites in normal full-term infants. METHODS: We performed BAER in 38 male and 28 female normal full-term infants, delivered in the Gil Medical Center, Gachen Medical School, from March to July 1996, aged one to seven days. Amplitude I, V, V/I and latency I, III, V and interpeak latency(IPL) I-III, III-V, I-V were measured at 90, 60, 45, 30 dB. Data were analyzed between both sex and between both ears with Student t-test. RESULTS: There were no significant difference in male and female group with the same side's stimulation. At 90 dB, amplitude I of left ear stimulation was significantly higher than right in male and female. Amplitude V/I of right ear stimulation was significantly higher than left ear stimulation in total only. At 90 dB, latency I of right was significantly longer than left in male and female. Latency III of right was longer significantly in total only. IPL I-III, I-V was significantly longer in left than right in male and total. At 60 dB intensity, all data except latency I in total, showed no significant difference. CONCLUSIONS: Interpreting BAER, stimulation site and intensity should be considered. and further studies will be needed for the evaluation of the difference between left and right ear.
Brain Stem* ; Central Nervous System ; Ear* ; Evoked Potentials, Auditory* ; Female* ; Hearing Disorders ; Humans ; Infant* ; Male* ; Mass Screening ; Schools, Medical

Alagille syndrome is characterized by chronic cholestasis, posterior embryotoxon, skeletal abnormalities, cardiovascular abnormalities, and a typical face with prominent forehead and pointed chin. Its histological feature includes paucity of interlobular bile ducts. We experienced a 49-day-old female infant presenting with frequent upper respiratory tract infection and persistent jaundice. She had a typical face and chronic cholestasis. Echocardiograms revealed peripheral pulmonary stenosis. The histological examination of liver revealed paucity of interlobular bile ducts.
Alagille Syndrome* ; Cardiovascular Abnormalities ; Chin ; Cholestasis ; Female ; Forehead ; Humans ; Infant ; Jaundice ; Liver ; Pulmonary Valve Stenosis ; Respiratory Tract Infections

Infantile hemangioendothelioma(IHE) of the liver is a rare benign vascular tumor that presents most commonly in infants before the age of 6 months. IHE presents as abdominal mass, cutaneous hemangiomas, unexplained jaundice, bleeding disorders, or congestive heart failure. Death often results from congestive heart failure despite appropriate treatment with digoxin and diuretics. IHE also is associated with Kasabach-Merritt syndrome, anemia, intraperitoneal hemorrhage secondary to rupture, consumptive coagulopathy and vascular malformation involving brain, skin, gut, and other organs. Although children with asymptomatic lesions may experience spontaneous regression within a year, symptomatic lesions shoud be treated aggressively because this disease can progress rapidly and may be fatal. Treatment options are divided into medical treatment, interventional therapy including embolization, and surgical resection. Corticosteroid may hasten involution by inhibiting proliferation of endothelial and smooth muscle cells, and this trial is warranted in most cases before invasive procedures are used. If steroid therapy is unsuccessful, early definitive treatment using embolization or ligation of the hepatic artery, resectional surgery, and orthotopic liver transplantation shoud be considered. We experienced symptomatic IHE in two neonates. In the first case, she showed respiratory failure and consumptive coagulopathy, and symptoms were aggravated despite steroid therapy, so a left lobectomy was performed. In the second case, he presented high output cardiac failure, and was successfully treated by the coil embolization of left hepatic artery. This coil embolization of hepatic artery for treating IHE was the first case in Korea we know of.
Anemia ; Brain ; Child ; Digoxin ; Diuretics ; Embolization, Therapeutic ; Heart Failure ; Hemangioendothelioma* ; Hemangioma ; Hemorrhage ; Hepatic Artery ; Humans ; Infant ; Infant, Newborn* ; Jaundice ; Kasabach-Merritt Syndrome ; Korea ; Ligation ; Liver Transplantation ; Liver* ; Myocytes, Smooth Muscle ; Respiratory Insufficiency ; Rupture ; Skin ; Vascular Malformations