Aesthetic Consideration of Management in Encephalocutaneous Lipomatosis: How we do it

Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital neurocutaneous syndrome that was characterized by unilateral, smooth, hairless fatty tissue nevi of the scalp, termed nevus psiloliparus, facial lesions, multiple anomalies involving the eye, and ipsilateral porencephalic cysts with cortical atrophy, cranial asymmetry, marked developmental delays, and mental retardation. A 12-month-old boy was referred to our clinic for evaluation of non-scarring alopecia on the left side with an underlying fatty mass in the left parietal scalp and left-sided multiple periocular masses. It showed a large lipomatous mass on the scalp with overlying alopecia. Multiple skin tags and defects in the left periocular area were also observed. Additional ocular anomalies included epibulbar lipodermoid, iris coloboma, and localized peripapillary hypopigmentation lesions. After complete excision, the wound was covered with a local flap. The histologic examination revealed a mass surrounded by a well-developed capsule within the dermal layer, adipose tissue and connective tissue septa extending into the reticular dermis. No recurrence was observed at follow-up. It is essential to differentiate suspected ECCL from other syndromes which present with epibulbar chorisotomas. Neuroimaging, and pathological studies may be helpful for correct diagnosis. We will correct multiple periocular lesions in preschool age and follow up developmental problems like developmental delay and mental retardation constantly.

Chronic Ulcerating Lesions due to Limited Form of Wegener's Granulomatosis on the Face: Cosmetic Consideration

Wegener's granulomatosis (WG) is a systemic disease characterized by necrotizing granulomas and vasculitis involving the upper and lower respiratory tract as well as the kidneys. Limited form of WG usually involves the head and neck, lacks renal involvement, and may not progress to generalized disease. We report the case of limited form of WG who presented not systemic symptom but several times relapsed multiple ulcerating lesions on the face, uveitis and keratoconjunctivitis. A 23 year-old female initially presented with ulcerative skin lesions on the left cheek and nose. The skin lesion had commenced as an ulcerative and nodulopapular lesion on her right cheek initially, 8 months ago. Subsequently, there was progression of the disease to her left cheek and nose. The patient was treated with oral prednisolone and oral cyclophosphamide. Two weeks later, skin lesion had started to heal, oral prednisolone tapered to 15 mg. Eight weeks later, all of skin lesions were healed well. With silicone gel sheets and Laser therapies, we gained excellent cosmetic results. In the aesthetic aspect, early recognition of rare variants of limited form of WG, facial chronic ulcerative wounds that are nonresponsive to conservative treatment, is very important as appropriate therapy can prevent facial mutilation.

Four cases of toxic liver injury associated with Dictamnus dasycarpus / 대한간학회지

We report four cases of toxic hepatitis that occurred after taking a decoction made by boiling down the root of Dictamnus dasycarpus. The four patients had a median age of 60 years, common symptoms of jaundice and general weakness, and stated that they had not consumed alcohol for at least 5 years. The markers of hepatitis A, B, and C were all negative in the patients. Abdominal ultrasonography revealed common bile ducts with normal diameters. Two of the cases were a mother and daughter, and the other two were sporadic. All of them had consumed a decoction made by boiling down the root of Dictamnus dasycarpus five or six times daily until several days before admission. Dictamnus dasycarpus induced liver injury presenting with a benign course lasting less than 1 month after cessation of the causative agent. We suggest that this natural product can cause acute hepatitis in rural areas.

Cholestatic Jaundice Associated with Juxtapapillary Diverticula without Common Bile Duct Stones

BACKGROUND/AIMS: Cholestatic jaundice caused by a juxtapapillary diverticulum can be treated by excision of the diverticula or endoscopic sphincterotomy(EST). The aim of this study is to evaluate the effectiveness of EST for cholestatic jaundice in patient with juxtapapillary diverticulum with absence of common bile duct stones. METHODS: We recruited patients who underwent Endoscopic Retrograde Cholangio Pancreatography(ERCP) between September 2000 and May 2005. The presence of Juxtapapillary diverticula was seen in 92(29.3%)) out of 315 patients underwent ERCP. Cholestatic jaundice associated with juxtapapillary diverticula was considered when patients have jaundice, elevated serum bilirubin and alkaline phosphatase more than normal, and there are no other obstructive lesion on abdominal ultrasonography or computed tomography(CT) scan. The number of the patients were 13 who had cholestatic jaundice associated with juxtapapillary diverticula without common bile duct stones. All 13 patients underwent EST and had no complications. The patients were followed up to visit outpatient department or interviewed on the telephone. RESULTS: Of the 13 patients, 10 were male and 3 were female. The mean age was 70.4 years and mean duration of follow-up were 28.9 months. All 13 patients who had gallbladder presented RUQ discomfort and jaundice. Seven patients presented gallstone pancreatitis with jaundice. Nine patients had GB stone, 1 patients had GB sludge. In the follow-up period, from 1month to 46 months after EST, 1 patient died of unknown cause within 1 year. One patient had recurred gall stone pancreatitis with jaundice 12 months later. CONCLUSIONS: EST can be considered as a useful therapeutic modality especially in eldery patients with cholestatic jaundice and gallstone pancreatitis associated with juxtapapillary diverticulum.

A Case of Multiple Splenic Lymphangiomas Diagnosed by Percutaneous Needle Biopsy

Lymphangioma of the spleen is an extremely rare disease in adults, and performing splenectomy has been considered necessary for its diagnosis and treatment, but the diagnosis of an isolated splenic mass lesion without performing splenectomy is a challenging problem. Herein, we report on a case of multiple splenic lymphangiomas that were found incidentally in a 56-year-old female; these lesions were diagnosed by percutaneous splenic biopsy without splenectomy. We suggest that this approach is a reasonable option for benign looking-appearing splenic tumors because splenectomy and its postsplenectomy complications can be avoided.

The Morphometric Changes of the Enteric Nervous System in the Intestinal Transection-reanastomosis Model / 체질인류학회지

This study was performed to investigate the morphometric changes of myenteric plexus and type 1 interstitial cells of Cajal (ICC-I) in regeneration process of small intestine transection. Sprague Dawley rats (200~250 g) were anesthetized with ether; then the full thickness of ileal wall were semitransected; and subsequent end-to-end anastomosis were performed by using 6-0 silk suture thread. Sham-operated rats, which only underwent the laparotomy, were used for control group. Experimental animals were sacrificed at 3 days, 7 days, 15 days, and 30 days after the operation. In each group myenteric plexus and ICC-Is were prepared by histochemical method (NADH-TR stain for myenteric plexus, ZIO stain for ICC-Is) and cell numbers were counted by image analyzer (Image plus pro-5.0, Media Cybermedics, USA). Degeneration of myenteric neurons and ICC-Is occurred simultaneously and it was similar in oral and anal to the site of transection. Degeneration effects were most prominent at 3 days and 7 days after intestinal transection. In myenteric plexus, many neurons had degenerated appearances and about 40% of them were lost. Most of ICC-Is had cytoplasmic vacuoles and 20~37% of the cells were lost. At 15 days after transection, there were no more degeneration in myenteric neurons (20~25% cell loss) and ICC-Is (20~13% cell loss). At 30 days after transection, numbers of myenteric neurons were not recovered as that of the control group. However numbers of ICC-Is were as similar as that of the control group. In conclusion, we confirmed that degeneration effects of intestinal transection are more severe in myenteric plexus than in ICC-Is, and recovery of cell loss occurs more slowly in myenteric plexus.

Colonoscopic Removal of an Inverted Appendix / 대한소화기내시경학회지

An inverted appendix is a rare condition that occurs congenitally or is the result of surgery or intussusception associated with diseases such as appendix tumors, mucocele, fecalith, or worms. An inverted appendix produces similar abdominal symptoms to acute appendicitis, that vary from non-specific to acute or chronic pain in the right lower abdomen. There are no reports of this condition in Korea that the authors are aware of. We report a case 31-year-old male who visited our hospital complaining of pain in the right lower abdomen. A barium enema showed a moving tubular filling defect. An inverted appendix that appeared as a polyp was dissected by colonoscopy.

Colonoscopic Removal of an Inverted Appendix / 대한소화기내시경학회지

An inverted appendix is a rare condition that occurs congenitally or is the result of surgery or intussusception associated with diseases such as appendix tumors, mucocele, fecalith, or worms. An inverted appendix produces similar abdominal symptoms to acute appendicitis, that vary from non-specific to acute or chronic pain in the right lower abdomen. There are no reports of this condition in Korea that the authors are aware of. We report a case 31-year-old male who visited our hospital complaining of pain in the right lower abdomen. A barium enema showed a moving tubular filling defect. An inverted appendix that appeared as a polyp was dissected by colonoscopy.

A Case of Emphysematous Pyelonephritis with Emphysematous Cystitis

A 68 year old male with diabetes mellitus visited our hospital due to general weakness and abdominal pain. Patient was in severe septic condition, abdominal X-ray and CT revealed gas shadow on left kidney and bladder. E-coli grew on urine culture. Thus we were able to diagnose the patient as the emphysematous pyelonephritis with emphysematous cystitis. The patient was treated with antibiotics and bilateral bladder catheter drainage. However the patient expired due to severe sepsis. Although the emphysematous pyelonephritis and emphysematous cystitis are rare disease, they result in life threatening complication in diabetic patients. Because of rarity of these diseases, the clinical and radiological classifications, the treatment and the progress are not established. These two diseases have the same causes such as diabetes mellitus or urinary tract obstruction and can be expanded due to anatomical relationships resulting in increased mortality. The authors report 68 year old male diabetic patient with emphysematous pyelonephritis with emphysematous cystitis with literature review.

Blood glucose/insulin index and carbohydrate-to-insulin ratio in Korean type 2 diabetic patients treated by continuous subcutaneous insulin infusion / 대한내과학회지

BACKGROUND: According to the results of Diabetes Control and Complication Trial, the best strategy to prevent and/or delay diabetic complications is to maintain the normal blood glucose levels. This led to emphasize the necessity of intensive management of diabetes. The purpose of the study was to determine blood glucose/insulin index and carbohydrate (CHO)-to-insulin ratios after normalization of blood glucose levels, and to select the factors to influence the blood glucose/insulin index and CHO-to-insulin ratios in Korean type 2 diabetic patients with continuous subcutaneous insulin infusion (CSII) treatment using insulin pump and CHO counting as a meal management. METHODS: Fifty-five type 2 diabetic patients who started CSII therapy checked their blood glucose levels before and after three meals, and recorded the amounts of insulin injected in fasting and each meal and the amounts of carbohydrates consumed in each meal. Actual blood glucose/insulin index and CHO-to-insulin ratio were determined using the records, and also they were calculated using Functional Insulin Treatment Training Methods (FITTM) proposed by Howorka. Calculated values were compared to actual values. The meaningful factors to influence the actual values were selected by backward stepwise regression analysis. RESULTS: The average age of the subjects was 49.9+/-12.5 years, and the duration of diabetes was 8.7+/-4.5 years. Their body mass index was 22.5+/-3.6 kg/m2. Daily insulin requirements to normalize the blood glucose levels reached to maximum levels at l0 day CSII treatment, and they were reduced and stabilized after 20 days of the treatment. The quotient K, representing insulin sensitivity, was also decreased after CSII treatment. The basal insulin index was 0.21 IU/kg at 10 days and 0.16 IU/kg at 20 days in our study, and these values were lower than the value from FITTM, 0.35 IU/kg The blood/glucose index was -2.5 mmol/L at 10 day CSII treatment, and it was decreased to -4.4 mmol/L at 20 day treatment to the stable levels. CHO-to-insulin ratio provided by FITTM was 2.59 IU/CHO exchange unit and the ratio was 3.12 IU/CHO exchange unit at 10 days, which was decreased to 1.84 IU/CHO exchange unit at 20 days in our study. CHO-to-insulin ratios at breakfast from carbohydrate counting were higher than those of lunch and dinner, and the ratios were 1.5 to 2.5 IU/CHO exchange unit. According to the stepwise regression analysis, the blood glucose/insulin index was affected by gender, age, body mass index (BMI), fasting blood glucose levels and fasting c-peptide levels, and CHO-to-insulin ratios were influenced by gender, age, BMI, post-prandial blood glucose levels and post-prandial c-peptide levels. CONCLUSIONS: CSII treatment can make blood glucose levels maintain in normal ranges in Korean type 2 diabetic patients, and can improve insulin sensitivity. Basal insulin requirements were lower and prandial insulin requirements were higher than those calculated from FITTM. This difference between Korean and the Western can be related to difference of insulin secretion from pancreas and nutrient intake.

Inappropriate Secretion of Antidiuretic Hormone in a Patient with Squamous Cell Carcinoma of the Lung mimicking Pulmonary Tuberculosis

A 71-year-old man with squamous cell carcinoma of lung mimicking pulmonary tuberculosis associated with the syndrome of inappropriate secretion of ADH(SIADH). Laboratory examination showed serum sodium concentration of 108 mEq/l. serum osmolality 247 mEg/l urine sodium concentration of 141 mEq/l urine osmolality 547 mEq/l. normal levels (<0.56 pg/ml) of serum antidiuretic hormone (ADH). ADH release was regulated normally with changes in serum osmolality. SIADH was diagnosed on the basis of hyponatremia with corresponding serum hypoosmolality and a high urine osmolality due to continuous sodium excretion. In this case, SIADH was probably induced by increased renal sensitivity to ADH and suspected high serum ANP level of unknown origin.