Colon Perforation during Air Enema Reduction of Intussusception

PURPOSE: Although air enema reduction has been known as a good method of diagnosis and treatment of intussusception, it could develop colon perforation. However, there have been few studies about this complication. So we analyzed the risk factors of colon perforation during air enema reduction in patients with intussusception. METHODS: We reviewed the charts of 12 colon perforation patients during air enema reduction of intussusception, who were admitted to Gil Medical Center from Jan. 1990 to Dec. 2001. Their age, sex, major symptoms, length of time till hospital visit, types of intussusception, operative findings and pathologic reports were reviewed. RESULTS: Among 657 cases, 596 patients(90.7%) were successfully treated, but 12 patients(1.83%) failed in air enema reduction and had colon perforation. In patients with colon perforation the male to female ratio was 11 : 1, and average age was 5.3 months. The most common symptom at the time of hospital visit was vomiting(91.7%). Cyclic irritability(75.0%), bloody stool(75.0%) and abdominal mass(41.7%) were also noted. The average length of time between symptom onset and hospital visit was 44.7 hours. Types of intussusception were predominantly ileocolic, ileocecal, and ileoileocolic. The site of perforation was most commonly found at the proximal part of intussusception including ascending colon(50%) and transverse colon(50%). Most cases were uncomplicated, and had a single perforation. Pathologic reports showed hemorrhagic necrosis and mesenteric laceration at the site of colon perforation. Complications of colon perforation were tension pneumoperitonium(58.3%), requiring immediate decompression. CONCLUSION: The chance of colon perforation during air enema reduction increases in cases with small bowel obstruction on simple abdominal x-ray of a patient younger than 6 months, delay in time till hospital visit and higher air pressure during reduction. Therefore more careful investigation is needed in these cases.

Influence of Climate Factors and PM10 on Rotaviral Infection: A Seasonal Variation Study / 대한소아소화기영양학회지

PURPOSE: Recently, while the authors were experiencing that the epidemic period of rotaviral infection happened more in the early spring, we tried to find out how the outbreaks of rotaviral infection are changing in detail depending on the weather condition since it has something to do with the climate factors and PM10. METHODS: Fourteen hundreds seventy nine patients who were proved to be positive to rotavirus were chosen among children less than 5 years old from January 1995 to June 2003. Among various climate factors, monthly average temperature, humidity, rainfall and PM10 were selected. RESULTS: Rotaviral infection was most active in 2002 as 309 (20.9%) patients. It has been the spring that is the most active period of rotaviral infection since 2000. The temperature (RR=0.9423, CI= 0.933424~0.951163), rainfall (RR=1.0024, CI=1.001523~1.003228) and PM10 (RR=1.0123, CI= 1.009385~1.015248) were significantly associated with the monthly distribution of rotaviral infection. CONCLUSION: Through this study we determined that the epidemic period of rotaviral infection is changed to spring, which is different from the usual seasonal periods such as late fall or winter as reported in previous articles. As increased PM10 which could give serious influence to the human body, and changing pattern of climate factors such as monthly average temperature and rainfall have something to do with the rotaviral infection, we suppose that further study concerning this result is required in the aspects of epidemiology, biology and atmospheric science.

Two Cases of Infantile Hemangioendothelioma of the Liver in Neonates

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.

Incontinentia Pigmenti in a Mother and her Daughter

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.

Comparison of Brainstem Auditory Evoked Response in Normal Infants, Male and Female, Right and Left Ear

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.

A Case of Saldino-Noonan Type Short Rib-Polydactyly Syndrome

Short rib-polydactyly syndrome (SRPS) is a rare type of skeletal dysplasia characterized by short limb dysplasia, thoracic hypoplasia, polydactyly and multiple visceral anomalies. It is transmitted as a autosomal recessive trait. There have been 4 classic types of SRPS, of which Saldino-Noonan type is the most common, and is characterized by very narrow thorax, short limb, postaxial polydactyly, striking metaphyseal dysplasia of tubular bones, pelvic abnormalities, hypoplasia of iliac bones, flat acetabulae, and abnormalities of urogenital, anorectal and cardiovascular system. We report a newborn infant who had typical features of Saldino-Noonan type SRPS, clinically and radiologically and had compatible autopsy findings.

Hypoxic - ischemic Encephalopathy in Term Infants: Correlation of Neurosonographic Findings in Basal Ganglia and Thalamus with Prognosis

PURPOSE: To evaluate abnormal neurosonographic (NSG) findings of thalami and basal ganglia in full term babies with hypoxic-ischemic encephalopathy and to correlate the findings with follow-up studies and prognosis. METHODS: We evaluated 13 full term babies with abnormal NSG findings of thalarni and basal ganglia. NSG was performed within 7 days after clinical abnormalities. Follow-up NSG was done in 11 cases; CT scan in 4 and MRI in 7. We classified NSG findings as diffuse, unilateral, and focal types according to increased echogenicity and evaluated prognosis based on follow-up studies and neurological sequelae. RESULTS: Nine cases of diffuse type had diffuse echogenic changes of bilateral thalami and basal ganglia, slit-like lateral ventricles suggesting cerebral edema, and increased parenchymal echogenicity. In diffuse type, follow-up studies showed more prominent echogencities and ventricular dilatations and cerebromalacia. One case of unilateral type caused by thromboembolism had unilateral echogenicity of right thalamus and basal ganglia with increased echogenicity of the ipsilateral cerebral hemisphere and compression of the lateral ventricle, suggesting cerebral infarction. Follow-up study showed unilateral cystic cerebromalacia. Three cases of focal type had a localized echogenic area in thalamus with lacunar infarction, which decreased in size during follow-up. Among nine cases of diffuse type, one died within 2 days, two were discharged against medical advice, and six had severe neurologic sequelae. One case of unilateral type had a moderate degree of neurologic sequelae. All 3 cases of focal type had normal development. CONCLUSION: Pattems of abnormal echogenicity in thalami and basal ganglia in fullterm infants with hypoxic-ischemic encephalopathy are correlated with the outcome and may be helpful for treatment planning.

A Case of Alagille Syndrome

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.

Magnetic Resonance Angiography in One Case of Hydraencephaly

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.

The Effect of Congenital Gut Obstruction on Fetal Growth

PURPOSE: The human fetus is primarily dependent on the placenta for its nutrition. However, as the fetus matures, it swallows increasing amounts of amniotic fluid, which contributes to the growth of fetus. Accordingly fetuses with congenital obstruction of the gut at high level have a reduced capacity for intestinal absorption of amniotic fluid. We undertook a study to investigate the effect of congenital gut obstruction on fetal growth. METHOD: A retrospective review of the records of all patients presenting congenital gut obstruction over 6-year period (from 1992 to 1997) in Chung-ang Gil hospital was performed. Patients with a complete proximal obstruction were included in group A; patients with incomplete or lower obstruction were included in group B. RESULT: 1) The ratio of male to female was 1.5:1. The mean birth weight and gestational age were 2.89+/-0.60kg and 38.7+/-0.20weeks. The mean birth weight and gestational age in group A were 2.68+/-0.69kg and 37.8+/-0.25weeks. The mean birth weight and gesnal age in group B were 2.980.54kg and 39.1+0.17weeks. There was significant difference between group A and B (P0.05). 3) In group A, 3 (42.8%) of 7 patients with associated anomalies had IUGR, whereas 8 (40.0%) of 20 patients without associated anomalies had IUGR (P>0.05). The corresponding figures for group B were 23.0% and 14.8%, respectively (P>0.05). CONCLUSION: Congenital gut obstruction causes IUGR by reducing intestinal absorption of amniotic fluid and the effect of IUGR is more pronounced as the obstruction is proximal to jejunum rather than distal to it.

Clinical Study of Neonatal Adrenal Lemorrhage

PURPOSE: Neonatal adrenal hemorrhage is not rare disease which can be caused by such risk factors as sepsis, large baby, birth trauma and asphyxia. The clinical manifestations include jaundice, anemia, abdominal mass and differentiation from neuroblastoma, renal vein thrombosis and adrenal abscess is needed. Through the clinical assessment of presenting features, we hope that this study be of any help to early detection and proper management of neonatal adrenal hemorrhage. METHODS: The 16 subjects out of neonates admitted to our hospital from July 1991 to June 1997 were diagnosed as neonatal adrenal hemorrhage. The risk factors, clinical manifestations, diagnostic methods and prognosis of neonatal adrenal hemorrhage were evaluated in these 16 cases. RESULTS: 1) Among 16 cases, males were 10 (62.5%) and females were 6 (37.5%). Mean birth weight was 3.601.08kg and mean gestational age was 39.82+1.08 week, and all were fullterm babies. In modes of delivery, vaginal deliveries were 12 cases (75.0%) and cesarean sections were 4 cases (25.0%). 2) 13 cases (81.2%) were involved in right side, 2 cases (12.5%) in left side and 1 case (6.3%) bilaterally. 3) The risk factors include sepsis in 6 cases (37.5%), large baby in 5 cases (31.3%), birth trauma in 5 cases<31.3%) and asphyxia in 3 cases (18.8%). 4) The clinical manifestations include jaundice in 7 cases (43.8%), anemia in 7 cases (43.8%), fever in 6 cases (37.5%) and abdominal mass in 3 cases (18.8%). 5) In the time of diagnosis, until 7 days of birth were 9 cases (56.2%), 8-14 days were 3 cases (18.8%), 15-21 days were 2 cases (12.5%) and 22-28 days were 2 cases (12.5%). Follow-up studies were done in 13 cases (81.2%), and tha lesions all decreased without any specific complications or sequelae. CONCLUSION: Neonatal adrenal hemorrhage can be diagnosed by abdominal ultrasono- gram in the presence of suggestive manifestations of jaundice, anemia, fever and abdominal mass, with relatively good prognosis. Differentiation from other conditions as well as avoidance of unnecessary explorations can be achieved by serial follow-up examinations of abdominal ultrasonogram.

A Case of Aplasia Cutis Congenita Group 5

Aplasia cutis congenita is a rare disorder characterized by localized or generalized absence of skin at birth. The various hypothesis and classification are proposed. Type V in Frieden's classification, which is associated with fetus papyraceus or placental infarcts, occurs as a large cutaneous defect on the trunk and extremities. We experienced the patient had skin defect affecting the lower abdomen symmetrically, with no family history of the any disorders or chromosomal anomalies and associated anomalies. A twin fetus was died in utero. We report a case of aplasia cutis congenita associated with fetus papyraceus with the review of the associated literatures.

Clinical study of intussusception in infancy and childhood: comparing the result of barium enema with that of air enema

Intussusception is the most common cause of acquired intestinal obstruction during infancy and early childhood and requires early diagnosis and treatment. We observed 431 cases of intussusception who admitted at Chungang Gil Hospital from January 1987 to June 1991, and compared the result of treatment with Barium Enema in 302 cases with that of Air Enema in 129 cases. The results were as follows; 1) In sex distribution, males were more affected than females as a ratio of 2:1. 2) In age incidence, 78.2% of the cases were between 3~10 months. 3) Slight seasonal prevalence was noted in spring and autumn. 4) The common symptoms and signs were cyclic irritability (89.1%), vomiting (78.9%), bloody stool (84.9%) and abdominal mass(43.1%). 5) The common diseases accompanied were URI (39.7%), AGE (13.0%) and UTI(1.2%). 6) 5.8% of cases were visited after the 48 hours of symptom onset, in these cases the operation rate was 39.1%. So the longer duration of symptoms persists, the lower reduction rate follows. 7) The most common type of intussusception was ileocolic type, and the lowest reduction rate was noted in ileoileocolic type. 8) In the reduction rate, 87.4% with Barium Enema and 89.1% with Air Enema. 9) After 24 hours of symptom onset, the reduction rate, 74.4% with Barium Enema and 78.6% with Air Enema. 10) Recurrence rate was 9.3% with Barium Enema and 7.8% with Air Enema. 11) In Barium Enema, 1 case of bowel perforation was observed, and he expired with barium peritonitis, in Air Enema, 2 cases of bowel perforation were observed, and cured after operation. 12) We observed some advantages in Air Enema such as simplicity, high reduction rate and absence of fatal complications. And we think that Air Enema is a useful diagnstic and treatment method of intussusception.