Agnogenic myeloid metaplasia (AMM) is a myeloproliferative disorder characterized by leukoerythroblastosis, tear-drop erythrocytes, extramedullary hematopoiesis with hepatosplenomegaly, and varying degrees of myelofibrosis. The mean age at presentation is about 60 years, and pediatric cases are rare. We experienced a case of AMM in a 9 months old female who was presented with pallor, huge splenomegaly and intermittent fever. Peripheral blood showed leukoerythroblastosis poikilocytosis, and tear drop cells. Bone marrow was difficult to aspirate, and biopsy specimen showed increased reticulin with decreased cellularity, which was compatible with myelofibrosis. We presented a case of AMM with brief review of the literatures.
Biopsy ; Bone Marrow ; Erythrocytes ; Female ; Fever ; Hematopoiesis, Extramedullary ; Humans ; Infant ; Myeloproliferative Disorders ; Pallor ; Primary Myelofibrosis* ; Reticulin ; Splenomegaly

A 20-month-old boy presented with a intraparenchymal mass in the right frontoparietal area manifesting as complex partial seizure, secondary generalization and left hemiparesis. Magnetic resonance images (MRI) of the brain showed inhomogeneously enhancing mass in the right frontoparietal area which has irregular margin and perilesional edema. Based on the radiological findings, a preoperative diagnosis was an intraaxial tumor, such as pilocytic astrocytoma or dysembryoplastic neuroepithelial tumor. The patient underwent a surgery including frontal craniotomy. The tumor had a partially extreme adherence to the surrounding brain tissue but it showed no dural attachment. Gross-total resection of the tumor was achieved. Postoperative follow-up computed tomography scans showed no residual tumor. The pathological findings confirmed the tumor as a WHO grade I meningioma, transitional type. Nine months after the surgery, follow-up brain MRI showed no recurrence of the tumor, porencephaly in site where the tumor was resected; the patient's symptoms had fully recovered. We report the case of a meningioma in a 20-month-old boy.
Astrocytoma ; Brain ; Child ; Craniotomy ; Edema ; Follow-Up Studies ; Generalization (Psychology) ; Humans ; Infant ; Magnetic Resonance Spectroscopy ; Meningioma ; Neoplasm, Residual ; Neoplasms, Neuroepithelial ; Paresis ; Recurrence ; Seizures

A 20-month-old boy presented with a intraparenchymal mass in the right frontoparietal area manifesting as complex partial seizure, secondary generalization and left hemiparesis. Magnetic resonance images (MRI) of the brain showed inhomogeneously enhancing mass in the right frontoparietal area which has irregular margin and perilesional edema. Based on the radiological findings, a preoperative diagnosis was an intraaxial tumor, such as pilocytic astrocytoma or dysembryoplastic neuroepithelial tumor. The patient underwent a surgery including frontal craniotomy. The tumor had a partially extreme adherence to the surrounding brain tissue but it showed no dural attachment. Gross-total resection of the tumor was achieved. Postoperative follow-up computed tomography scans showed no residual tumor. The pathological findings confirmed the tumor as a WHO grade I meningioma, transitional type. Nine months after the surgery, follow-up brain MRI showed no recurrence of the tumor, porencephaly in site where the tumor was resected; the patient's symptoms had fully recovered. We report the case of a meningioma in a 20-month-old boy.
Astrocytoma ; Brain ; Child ; Craniotomy ; Edema ; Follow-Up Studies ; Generalization (Psychology) ; Humans ; Infant ; Magnetic Resonance Spectroscopy ; Meningioma ; Neoplasm, Residual ; Neoplasms, Neuroepithelial ; Paresis ; Recurrence ; Seizures

Infantile fibrosarcoma is a rare malignant soft tissue tumor occurring especially in newborn and young children under 2 years. We experienced three cases of infantile fibrosarcoma presenting in the neonatal period. Case 1 presented with a multiseptated cystic mass on his left thigh at birth that was diagnosed as lymphangioma. After picibanil injection, we noted the size of the mass doubled and a solid lesion was prominent in the magnetic resonance image. Case 2 was found to have a reddish mass on his lower back mimicking hemangioma. Over 2 weeks, the mass grew rapidly with internal hemorrhaging. Case 3 was noted to have an encircling mass around the splenic flexure, which developed into congenital bowel obstruction. All of the tumors were resected completely, but microscopic resection margin was not clear in two patients. The two patients received adjuvant chemotherapy and all patients are well without evidence of recurrence.
Chemotherapy, Adjuvant ; Child ; Colon, Transverse ; Fibrosarcoma ; Hemangioma ; Humans ; Infant, Newborn ; Lymphangioma ; Magnetic Resonance Spectroscopy ; Parturition ; Picibanil ; Recurrence ; Thigh

Progressive muscular dystrophy is a genetically determined myopathy of unknown etiology and is a primary degenerative disease of skeletal muscle fibers. The authors reviewed 32 cases of progressive muscular dystrophy and made following observations. 1. The types among the 32 cases were as follows. 1) Duchenne type ; 16 2) Limb-Girdle type ;12 3) Facioscapulohumeral type ;3 4) Distal myopathy ;1 5) Ocular myopathy ;0 2. 13 of the 32 patients had relevant family history. 3. In general, creatinine excretion was decreased in 24-hour urine and creatine was increased. 4. 6 of the 10 patients tested had increased serum C.P.K. level.
Creatine ; Creatinine ; Distal Myopathies ; Humans ; Muscle Fibers, Skeletal ; Muscular Diseases ; Muscular Dystrophies

PURPOSE: This study aimed to assess the long-term clinical outcome of laparoscopic Nissen fundoplication (LNF) in children according to their neurologic status. METHODS: The study retrospectively analyzed the data of 82 children (62 neurologically impaired and 20 neurologically normal children with primary gastroesophageal reflux disease) who had undergone LNF between 2003 and 2012. The main outcome measures were the occurrence of recurrence that required reoperation and post-procedure complications such as infections, pneumonia, and gastrointestinal complications including ileus, dysphagia, and delayed gastric emptying. RESULTS: The median age at the time of the LNF was 25 months (range, 1-192 months), and the median of body weight was 10.0 kg (range, 2.8-37.0 kg). The average weight gain was 1.55±1.68 kg at 6 months, 3.32±2.30 kg at 1 year, and 5.63±4.22 kg at 2 years after surgery. Six (9.7%) of the 62 neurologically impaired patients and two (10.0%) of neurologically normal lost their body weight or had no weight changes. Eight (12.9%) of the 62 neurologically impaired children had required redo surgery because of gastroesophageal reflux disease recurrences, while 2 (10.0%) of the 20 neurologically normal children had experienced recurrences. In the neurologically impaired children, the postoperative complications included pneumonia (n=1), wound infection (n=1), urinary tract infection (n=1), dysphagia (n=1), delayed gastric emptying (n=1), and ileus (n=2). All of these complications were not found in the neurologically normal group, except for only one case of infectious colitis. However, there was no statistically significant difference between the two groups in postoperative complications. CONCLUSION: The outcomes of laparoscopic fundoplication were similar in the neurologically impaired children and in the neurologically normal children.
Body Weight ; Child* ; Colitis ; Deglutition Disorders ; Fundoplication* ; Gastric Emptying ; Gastroesophageal Reflux ; Humans ; Ileus ; Laparoscopy ; Neurologic Manifestations ; Outcome Assessment (Health Care) ; Pneumonia ; Postoperative Complications ; Recurrence ; Reoperation ; Retrospective Studies ; Urinary Tract Infections ; Weight Gain ; Wound Infection