Although renal calcium crystal deposits (nephrocalcinosis) may occur in acute phosphate poisoning as well as type 1 renal tubular acidosis (RTA), hyperphosphatemic hypocalcemia is common in the former while normocalcemic hypokalemia is typical in the latter. Here, as a unique coexistence of these two seperated clinical entities, we report a 30-yr-old woman presenting with carpal spasm related to hypocalcemia (ionized calcium of 1.90 mM/L) due to acute phosphate poisoning after oral sodium phosphate bowel preparation, which resolved rapidly after calcium gluconate intravenously. Subsequently, type 1 RTA due to Sjögren's syndrome was unveiled by sustained hypokalemia (3.3 to 3.4 mEq/L), persistent alkaline urine pH (> 6.0) despite metabolic acidosis, and medullary nephrocalcinosis. Through this case report, the differential points of nephrocalcinosis and electrolyte imbalances between them are discussed, and focused more on diagnostic tests and managements of type 1 RTA.
Hypocalcemia; Nephrocalcinosis; Sodium Phosphate; Distal RTA; Sjögren's Syndrome
We report a rare case of the concurrent manifestation of central diabetes insipidus (CDI) and type 2 diabetes mellitus (DM). A 56 year-old man was diagnosed as a type 2 DM on the basis of hyperglycemia with polyuria and polydipsia at a local clinic two months ago and started an oral hypoglycemic medication, but resulted in no symptomatic improvement at all. Upon admission to the university hospital, the patient's initial fasting blood sugar level was 140 mg/dL, and he showed polydipsic and polyuric conditions more than 8 L urine/day. Despite the hyperglycemia controlled with metformin and diet, his symptoms persisted. Further investigations including water deprivation test confirmed the coexisting CDI of unknown origin, and the patient's symptoms including an intense thirst were markedly improved by desmopressin nasal spray (10 µg/day). The possibility of a common origin of CDI and type 2 DM is raised in a review of the few relevant adult cases in the literature.
Polyuria; Central diabetes insipidus; Type 2 diabetes mellitus; Water deprivation test
A 67-year-old woman presented with memory impairment and behavioral changes. Brain MRI indicated hepatic encephalopathy. Abdominal CT scans revealed an intrahepatic portosystemic venous shunt that consisted of two shunt tracts to the aneurysmal sac that communicated directly with the right hepatic vein. The large tract was successfully occluded by embolization using the newly available AMPLATZERTM Vascular Plug II and the small tract was occluded by using coils. The patient's symptoms disappeared after shunt closure and she remained free of recurrence at the 3-month follow-up evaluation.
Hepatic encephalopathy; Portosystemic shunt; Surgical; Embolization; Therapeutic
A pancreatic hamartoma is a rare benign lesion that may be mistaken for malignancy. A pancreatic hamartoma can present with vague, non-specific symptoms, which can be difficult to diagnose despite modern diagnostic tools. We report here a pancreatic hamartoma diagnosed after surgical resection. A 52-year-old female presented with postprandial abdominal discomfort. Abdominal computed tomography and pancreatic magnetic resonance imaging revealed a 2.2 × 2.5-cm cystic mass in the pancreatic head. The patient underwent a pylorus-preserving pancreaticoduodenectomy. The histopathological and immunohistochemical studies helped make the diagnosis of pancreatic hamartoma. Here, we report a case of pancreatic hamartoma and review the relevant medical literature.
Hamartoma; Pancreas; Pancreaticoduodenectomy
Broncholiths are defined as calcified materials that occur in a tracheobronchial tree or in a cavity communicating with that. Broncholith has variable clinical features. The therapeutic options to remove broncholiths are so variable that clinicians need to select the most safe and effective methods by mass size, mobility, and location. As yet, there is no consistent guideline removing a broncholith. We report 2 successful cases of removing a fixed broncholith by flexible bronchoscopy guided cryoadhesion. With repeated technique of thawing and freezing with ryoprobe, we could extract the fixed broncholith safely. This method is promising as a way to remove broncholith in the future.
Bronchial Diseases; Calculi; Cryotherapy; Bronchoscopy
Ultrasound-guided cannulation of a large-bore catheter into the internal jugular vein was performed to provide temporary hemodialysis vascular access for uremia in a 65-yr-old woman with acute renal failure and sepsis superimposed on chronic renal failure. Despite the absence of any clinical evidence such as bleeding or hematoma during the procedure, a chest x-ray and computed tomographic angiogram of the neck showed that the catheter had inadvertently been inserted into the subclavian artery. Without immediately removing the catheter and applying manual external compression, the arterial misplacement of the hemodialysis catheter was successfully managed by open surgical repair. The present case suggests that attention needs to be paid to preventing iatrogenic arterial cannulation during central vein catheterization with a large-bore catheter and to the management of its potentially devastating complications, since central vein catheterization is frequently performed by nephrologists as a common clinical procedure to provide temporary hemodialysis vascular access.
Hemodialysis; Complication; Central Venous Catheterization
Terson syndrome was originally used to describe a vitreous hemorrhage arising from aneurysmal subrarachnoid hemorrhage. Terson syndrome can be caused by intracranial hemorrhage, subdural or epidural hematoma and severe brain injury but is extremely rare in intraventricular hemorrhage associated with moyamoya disease. A 41-year-old man presented with left visual disturbance. He had a history of intraventicular hemorrhage associated with moyamoya disease three months prior to admission. At that time he was in comatose mentality. Ophthalmologic examination at our hospital detected a vitreous hemorrhage in his left eye, with right eye remaining normal. Vitrectomy with epiretinal membrane removal was performed. After operation his left visual acuity was recovered. Careful ophthalmologic examination is mandatory in patients with hemorrhagic moyamoya disease.
Moyamoya disease; Terson syndrome; Intraventricular hemorrhage
In this report, we describe a Korean patient with May-Hegglin anomaly from a mutation of the MYH9 gene. The proband was a 21-year-old man with thrombocytopenia. He did not have a bleeding tendency. His neutrophil count was normal at 7490/mm3; however, the neutrophils contained abnormal basophilic inclusions in their cytoplasm. The platelet count was decreased at 15000/mm3 with giant platelets. Coagulation test results were not remarkable. Direct sequencing of MYH9 revealed that he was heterozygous for a mutation in exon 1, which was a 97T>A substitution mutation affecting codon 33, substituting tryptophan with arginine (Trp33Arg). Family study showed that both of his parents had normal phenotype and genotypes, indicating a de novo occurrence of the mutation in the proband.
May-Hegglin anomaly; MYH9; thrombocytopenia; Korean
Here we report a case of central retinal artery occlusion after chiropractic manipulation on the neck. A 49-year old man presented at the hospital because of sudden visual loss in his right eye after chiropractic neck manipulation. He had received chiropractic manipulation of the neck by a chiropractor eight days prior. When he first visited us, his best corrected visual acuity in his right eye was hand motion. A full ophthalmic examination was performed. There was cherry-red spot in the macula in his right eye. We performed a fluorescein angiogram and cervical color Doppler. The arterio-venous transit time in the fluorescein angiogram was delayed, and we detected stenosis of the right internal carotid artery with diffuse atherosclerotic plaques in the right common carotid artery. We prescribed ginko biloba extract (Tanamin). Three years after his first visit, the best corrected visual acuity of his right eye was 20 / 200.
Chiropractic manipulation; Retinal artery occlusion
A 66-year-old female presented with a 1-month history of dyspepsia. An initial upper gastrointestinal endoscopy with biopsy revealed a low-grade mucosa-associated lymphoid tissue (MALT) lymphoma. A rapid urease test was positive for Helicobacter pylori. Endoscopic ultrasound (EUS) and computed tomography (CT) revealed a 30×15-mm lymph node (LN) in the subcarinal area. Histopathologic and phenotypic analyses of the biopsy specimens obtained by EUS-guided fine-needle aspiration revealed a MALT lymphoma, and the patient was diagnosed with a stage 4E gastric MALT lymphoma. One year after H. pylori eradication, the lesion had disappeared, as demonstrated by endoscopy with biopsy, CT, fusion whole-body positron emission tomography, and EUS. Here, we describe a patient with gastric MALT lymphoma that metastasized to the mediastinal LN and regressed following H. pylori eradication.
Marginal zone B-cell lymphoma; Stomach
Hemophagocytic lymphohistiocytosis (HLH) is an unusual syndrome characterized by fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogenemia, and pathologic findings of hemophagocytosis in the bone marrow and other tissues. HLH may be familial or associated with different types of infections, autoimmune disorders, or malignancies. Infection-associated HLH has been reported in various viral, bacterial, fungal, and parasitic infections, and case reports of parasitic infections implicated in HLH include rare cases from Plasmodium vivax infection, which occasionally affects both military personnel and civilians in Korea. We describe an unusual case of HLH resulting from Plasmodium vivax infection and review the literature. This case suggests that clinical suspicion of HLH is important when P. vivax infection is accompanied by cytopenias. Administration of antimalarial drugs may prevent irreversible end organ damage resulting from P. vivax-associated HLH.
Hemophagocytic lymphohistiocytosis; Plasmodium vivax
Malignant gastrointestinal stromal tumors (GISTs) are rare non-epithelial, mesenchymal neoplasms of the gastrointestinal tract that metastasize or recur in 30% of patients who undergo surgical resection with curative intent. A 59-year-old man visited our hospital for an examination of a palpable mass in the left abdomen. Fourteen months prior to his visit, the patient underwent gastric wedge resection to remove a GIST of the gastric cardia. At the time of surgery, no evidence of metastatic disease was observed and the pathological interpretation was a high-risk GIST. A follow-up computed tomography scan of the abdomen revealed a partially necrotic solid mass (9.8 × 7.6 cm) and enhancing mass in the spleen (2.3 cm). On exploration, multiple masses were found in the liver, greater omentum, and mesentery. Here, we report a case of recurring GIST of the stomach that metastasized to the spleen. To the best of our knowledge, few reports of metastasis to the spleen exist.
Gastrointestinal stromal tumors; Neoplasm metastasis; Spleen
Primary sarcomas of the pancreas are extremely rare, accounting for 0.1% of malignant pancreatic (non-islet) neoplasms. Pancreatic leiomyosarcoma is a highly aggressive malignancy that spreads in a similar manner to gastric leiomyosarcoma, i.e., by adjacent organ invasion, hematogenous spread, and lymph node metastasis. These tumors are large at the time of diagnosis and are usually found at an advanced stage. We report a case of a 70-year-old female with intermittent right upper quadrant abdominal discomfort. Radiological, histopathological, and immunohistochemical studies revealed the tumor to be a primary leiomyosarcoma of the pancreas. Herein, we describe a patient with a primary leiomyosarcoma of the pancreas who presented with clinical and radiological findings indicative of a mass in the pancreatic head.
Leiomyosarcoma; Pancreas; Primary
A 67 year old male at a regular checkup underwent esophagogastroduodenoscopy. On performing esophagogastroduodenoscopy, a lesion about 1.2 cm depressed was noted at the gastric angle. The pathology of the biopsy specimen revealed a well-differentiated adenocarcinoma. On performing an abdominal computed tomography (CT) scan & positron emission tomography-computed tomography (PET-CT) scan, no definite evidence of gastric wall thickening or mass lesion was found. However, lymph node enlargement was found in the left gastric and prepancreatic spaces. This patient underwent laparoscopic assisted distal gastrectomy and D2 lymph node dissection. On final examination, it was found out that the tumor had invaded the mucosal layer. The lymph node was a metastasized large cell neuroendocrine carcinoma with an unknown primary site. The patient refused chemotherapy. He opted to undergo a close follow-up. At the postoperative month 27, he had a focal hypermetabolic lesion in the left lobe of the liver that suggested metastasis on PET-CT scan. He refused to undergo an operation. He underwent a radiofrequency ablation.
Gastric cancer; Neuroendocrine tumors; Neoplasms, unknown primary
Basal cell carcinoma (BCC), which frequently occurs in sun-exposed areas of the head and neck region, is the most common cutaneous malignancy. The nipple-areola complex (NAC) is an uncommon site for BCC to develop. BCCs in this region display more aggressive behavior and a greater potential to spread than when found in other anatomical sites. This paper outlines the case of 67-year-old female with a solitary asymptomatic black plaque on the right areola. The lesion was initially recognized as Paget's disease of the nipple by a general surgeon. However, the histopathological features showed a tumor mass of basaloid cells, a peripheral palisading arrangement and scattered pigment granules. Finally, the patient was diagnosed with pigmented BCC of the NAC and was referred to the department of dermatology. Positron emission tomography-computed tomography revealed the absence of distant metastasis. A wide excision was done. The lesion resolved without recurrence or metastasis during 14 months of follow-up.
Areola; Basal cell carcinoma; Nipple
The natural course of untreated patients with signet ring cell carcinoma of the stomach remains poorly understood while assumptions have been made to distinguish it from other types of gastric cancer. A 74-year-old Korean woman was diagnosed with early gastric cancer with signet ring cell histology and refused surgery. A satellite lesion was identified 46 months after the initial diagnosis. The patient finally agreed to undergo distal subtotal gastrectomy 53 months following the initial diagnosis. Postoperative histological examination of both lesions confirmed signet ring cell carcinoma associated with submucosal invasion. There was no evidence of lymph node metastasis.
Disease progression; Gastrectomy; Carcinoma, signet ring cell; Stomach neoplasms
Parry-Romberg syndrome (PRS) is a relatively rare degenerative disorder that is poorly understood. PRS is characterized by slowly progressing atrophy affecting one side of the face, and is frequently associated with localized scleroderma, especially linear scleroderma, which is known as en coup de sabre. This is a report of the author's experiences with PRS accompanying en coup de sabre, and a review of the ongoing considerable debate associated with these two entities. Case 1 was a 37-year-old woman who had right hemifacial atrophy with unilateral en coup de sabre for seven years. Fat grafting to her atrophic lip had been conducted, and steroid injection had been performed on the indurated plaque of the forehead. Case 2 was a 29-year-old woman who had suffered from right hemifacial atrophy and bilateral en coup de sabre for 18 years. Surgical corrections such as scapular osteocutaneous flap and mandible/maxilla distraction showed unsatisfying results.
Encoup de sabre; Hemifacial atrophy; Parry-Romberg syndrome
Nevus sebaceous (NS) is a benign neoplasm occurring mainly on the face and scalp. It commonly occurs as a solitary, well-demarcated lesion. This paper presents a case of multiple nevus sebaceous, which presented as multiple lesions occurring on the temporal scalp and on the contralateral side of the chin. Multiple NS have only rarely been reported.
Multiple; Nevus sebaceus
Undifferentiated carcinoma with osteoclast-like giant cells is a rare neoplasm of the exocrine pancreas. Some similar cases have been reported, but the histogenesis of these tumors varies and is controversial. We report here on a case of undifferentiated carcinoma of the pancreas with osteoclast-like giant cells. A 77-year old woman presented with abdominal pain and anorexia. Abdominal computed tomography and magnetic resonance imaging showed an approximately 10 × 5 cm highly attenuated mass arising from the tail of the pancreas and invading the spleen and adjacent bowel loop. The initial impression was a malignant endocrine tumor or solid-pseudopapillary tumor of the pancreas. The patient underwent a distal pancreatectomy with splenectomy and left hemicolectomy. The histopathology and immunohistochemistry helped make the diagnosis that of an undifferentiated carcinoma with osteoclast-like giant cells of the pancreas.
Osteoclast-like giant cells; Undifferentiated carcinoma; Pancreas
This is a case of a sudden cardio-pulmonary arrest in a 29 year-old female, which occurred immediately after a large bolus infusion of propofol (100 mg) intravenously during dilatation and curettage. The arrest suddenly occurred, and the patient was eventually transferred to our emergency room (ER) on cardiopulmonary resuscitation. At that time, severe hyperkalemia up to 9.1 mEq/L and ventricular fibrillation were noted. Resuscitation in ER worked successfully with conversion of electrocardiograph to sinus rhythm, but this patient expired unfortunately. On view of this acute event immediately after the bolus injection of propofol accompanied without other identified causes, severe hyperkalemia induced by propofol was strongly assumed to be the cause of death. To our understanding with the literature survey, propofol as a cause of hyperkalemia has not been well described yet. Through this case, the relationship as a cause and an effect between propofol and hyperkalemia is suggested.
propofol; hyperkalemia; heart arrest; propofol infusion syndrome
Lymphangiomas are rare congenital benign tumors arising from the lymphatic system, and are mostly encountered in the neck and axillary regions of pediatric patients (95%). Lymphangioma of the pancreas is extremely rare accounting for less than 1% of these tumors. We report here on a case of pancreatic cystic lymphangioma. A 54-year-old woman presented with intermittent postprandial abdominal discomfort and radiating back pain. Abdominal computed tomography scan revealed 8 × 6.5 cm hypodense cystic mass arising from the tail of the pancreas without septa or solid component. The initial impression was a pancreatic pseudocyst. The patient underwent distal pancreatectomy with splenectomy. The histopathologic and immunohistochemical study helped make the diagnosis of a pancreatic cystic lymphangioma. Herein, we report a case of pancreatic cystic lymphangioma mimicking pancreatic pseudocyst and review the relevant medical literature.
Pancreas; Cystic lymphangioma; Surgical excision; Pseudocyst
Epidural sacral nerve compression as an initial feature of leukaemia is a rare complication. The findings in a 16-year-old boy who presented to an emergency department with symptoms of faecal incontinence are reported herein. Radiological imaging demonstrated soft tissue masses in the sacral epidural space. The diagnosis of acute myeloid leukaemia was confirmed on bone marrow aspirate. The characteristics and management of extramedullary leukaemia are discussed.
We report here on a case of a 23-year-old male who received en block spondylectomy for a vertebral Ewing's sarcoma at our hospital. Nine days after surgery, he presented with severe back pain and motor weakness of the lower extremities. Based on the physical examination and the computed tomography scan, he was diagnosed with acute cauda equina syndrome that was caused by compression from an epidural hematoma. His neurological functions recovered after emergency evacuation of the hematoma. This case showed that extensive surgery for a malignant vertebral tumor has a potential risk of delayed epidural hematoma and acute cauda equina syndrome and this should be treated with emergency evacuation.
Epidural hematoma; Ewing's sarcoma; Spine; En block spondylectomy
A 63-year-old female diagnosed with relapsed multiple myeloma visited our hospital complaining of a persistent cough. Since July 2006, she had been taking 100 mg thalidomide daily and gradually developed shortness of breath and a persistent dry cough. A chest X-ray and computed tomography showed ground glass opacities in both lungs. An open lung biopsy of the right middle lobe under general anesthesia revealed chronic peribronchial inflammation, mild interstitial fibrosis, and intra-alveolar macrophage infiltration, with some hemosiderin features, compatible with non-specific interstitial pneumonia (NSIP). After discontinuing the thalidomide, the patient's symptoms did not deteriorate, although the radiographs did not improve. The patient is alive and well with regular outpatient follow-up without progression of the NSIP.
Lung diseases, interstitial; Thalidomide; Multiple myeloma
Ehlers-Danlos syndrome (EDS) type IV is characterized by its clinical manifestations, which are easy bruising, thin skin with visible veins, and rupture of arteries, uterus, or intestines. Arterial complications are the leading cause of death in vascular EDS because they are unpredictable and surgical repair is difficult due to tissue fragility. The authors report a case presented with cervical radiculopathy due to a segmental fusiform aneurysm of the cervical vertebral artery. Transfemoral cerebral angiography (TFCA) was done to verify the aneurysmal dilatation. However, during TFCA, bleeding at the puncture site was not controlled, skin and underlying muscle was disrupted and profound bleeding occurred during manual compression after femoral catheter removal. Accordingly, surgical repair of the injured femoral artery was performed. At this time it was possible to diagnose it as an EDS with fusiform aneurysm on cervical vertebral artery. Particularly, cervical fusiform aneurysm is rare condition, and therefore, connective tissue disorder must be considered in such cases. If connective tissue disorder is suspected, the authors suggest that a noninvasive imaging modality, such as, high quality computed tomography angiography, be used to evaluate the vascular lesion to avoid potential arterial complications.
Ehlers-Danlos syndrome; Cervical radiculopathy; Fusiform aneurysm; Vascular reconstruction