It is uncommon for pediatric patients with rhabdomyosarcoma to present with clinical and/or laboratory features of disseminated intravascular coagulation (DIC). We report a case of metastatic alveolar rhabdomyosarcoma with severe bleeding because of DIC in a 13-year-old boy. He experienced persistent oozing at the site of a previous operation, gross hematuria, and massive epistaxis. Two weeks after initiating combination chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide, the patients' laboratory indications of DIC began to resolve. During this period, the patient received massive blood transfusion of a total of 311 units (26 units of red blood cells, 26 units of fresh frozen plasma, 74 units of platelet concentrates, 17 units of single donor platelets, and 168 units of cryoprecipitate), antithrombin-III and a synthetic protease inhibitor. Despite chemotherapy and radiation therapy, he died 1 year later because of disease progression. In children with metastatic rhabdomyosarcoma and massive DIC, prompt chemotherapy and aggressive supportive care is important to decrease malignancy-triggered procoagulant activities.
Adolescent ; Blood Platelets ; Blood Transfusion ; Child ; Cyclophosphamide ; Dacarbazine ; Disease Progression ; Disseminated Intravascular Coagulation ; Doxorubicin ; Drug Therapy* ; Drug Therapy, Combination ; Epistaxis ; Erythrocytes ; Hematuria ; Hemorrhage ; Humans ; Male ; Plasma ; Protease Inhibitors ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar* ; Tissue Donors ; Vincristine

The deletion of the distal long arm of chromosome 1 is associated with a characteristic facial appearance and a pattern of associated malformations. Characteristic manifestations include a round face with prominent 'cupid's bow' and downturned corners of the mouth, thin vermilion borders of lips, a long upper lip with a smooth philtrum, a short and broad nose, epicanthal folds, apparently low-set ears, micrognathia, microcephaly, abnormal hands and feet, variable cardiac or genital anomalies, moderate to severe mental retardation, and growth retardation. Using fluorescent in situ hybridization (FISH) analysis to map precisely the deletion, we present a case of chromosome 1q44 deletion with craniofacial characteristics, multiple congenital anomalies, and growth and psychomotor retardation. In comparison with other reported cases of 1q43-44 deletion, the subject does not show hydrocephalus, seizure, syn- or polydactyly of hands, and a urogenital anomaly. However, an arachnoid cyst, pinpoint dimple on the midline of the forehead, a right-sided supernumerary nipple and auricular pit, polydactyly of the right foot, adducted thumb, and flexion restriction of the proximal interphalangeal joint with a simian line in both hands were observed additionally.
Arachnoid ; Arm ; Chromosomes, Human, Pair 1 ; Ear ; Female ; Foot ; Forehead ; Hand ; Humans ; Hydrocephalus ; In Situ Hybridization, Fluorescence ; Infant* ; Intellectual Disability ; Joints ; Korea ; Lip ; Microcephaly ; Mouth ; Nipples ; Nose ; Polydactyly ; Seizures ; Thumb

PURPOSE: Cephalohematoma is a common birth injury that is absorbed spontaneously at most of the time. However, it sometimes can be accompanied with severe intracranial lesion which may ultimately lead to death. The aim of this study is to know when the CT and/or MRI study is useful by analyzing the risk factors for intracranial lesion associated with cephalohematoma in newborn. METHODS: From January 2006 to December 2010, 162 infants diagnosed with cephalohematoma were studied retrospectively. We examined the demographic and clinical data, and also examined the reasons for neuroimaging studies with CT/MRI findings. The risk factors for intracranial lesion associated with cephalohematoma were analyzed by uni- and multivariate analysis as well. RESULTS: Among 162 patients, many were groups of normal birth weight, first-born, singlet or vaginally delivered newborns. Of these patients, 13.6% had neurologic symptoms, 4.1% had other birth injuries, and 1.2% died with associated intracranial lesion. Parietal region was the commonest site and most were greater than 3 cm in diameter. Simple skull X-ray was performed in 56.8% and CT/MRI in 28.4%. Giant hematoma and neurologic symptom were the two common reasons for requesting CT/MRI. Intracranial lesions such as hemorrhage, hypoxic-ischemic encephalopathy, and infarction were shown in almost two thirds of patients who performed CT/MRI. Multivariate analysis showed that significant risk factors were being first-born, having large size hematoma (> or = 5 cm) or having seizure. CONCLUSION: CT and/or MRI is recommended in cephalohematoma of newborn, especially for first-born baby with large size hematoma (> or = 5 cm) or seizure.
Birth Injuries ; Birth Weight ; Hematoma ; Hemorrhage ; Humans ; Hypoxia-Ischemia, Brain ; Infant ; Infant, Newborn ; Infarction ; Multivariate Analysis ; Neuroimaging ; Neurologic Manifestations ; Retrospective Studies ; Risk Factors ; Seizures ; Skull

PURPOSE: This study assesses the usefulness of alkaline phosphatase (ALP) in predicting the radiologic osteopenia and rickets for very low birth weight infants (VLBWI) below 1,500 g of birth weight. METHODS: Medical records of 258 VLBWI who were admitted at NICU between January 2009 and December 2011 were reviewed retrospectively. ALP max was defined by maximum level of ALP during admission. Subjects were divided into two groups: the study (n=112, ALP max > or =450 IU/L) and the control group (n=146, ALP max <450 IU/L). The two groups were compared based on the demographic and perinatal risk factors, clinical parameters, and morbidity. Also compared between the groups were: the level of ALP max, total and ionized calcium, inorganic phosphorus, and radiologic finding. To assess the usefulness of ALP in predicting the radiologic osteopenia and rickets, the sensitivity, specificity, and the positive (PPV) and negative predictive values (NPV) according to ALP max level were examined. A receiver operator characteristic (ROC) curve was constructed to determine the optimal cut-off value of ALP for the radiologic diagnosis. RESULTS: The study group was more immature and smaller, and had more perinatal risk factors with higher morbidity. The sensitivity and specificity for the radiologic finding on the basis of ALP max 450 IU/L were 68.8% and 64.9%, PPV 39.3%, and NPV 86.3% with a significance (P<0.001). In ROC curve, we observed the appropriate cut-off value to be 459.5 IU/L (P<0.001). CONCLUSION: The baseline of ALP max 450 IU/L was useful in predicting the radiologic osteopenia and rickets in VLBWI.
Alkaline Phosphatase ; Bone Diseases, Metabolic ; Calcium ; Humans ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Very Low Birth Weight ; Medical Records ; Parturition ; Phosphorus ; Retrospective Studies ; Rickets ; Risk Factors ; ROC Curve ; Sensitivity and Specificity

Carcinoid tumors of the thymus are vanishingly rare, and the characteristic cytologic findings of this condition have never before been reported in Korea. Recently, we encountered a 58-year-old woman who had been suffering from general weakness and weight loss for several months. Radiological imaging revealed a large anterior mediastinal mass. A fine needle aspiration biopsy (FNAB) of the mass showed predominantly scattered single cells, as well as some loose clusters of small cells with scanty cytoplasm. Some of these small cells exhibited plasmacytoid features, with moderately granular cytoplasm. We also discuss the cytological differential diagnosis between thymic carcinoid and other mediastinal tumors.
Biopsy ; Biopsy, Fine-Needle* ; Carcinoid Tumor* ; Cytoplasm ; Diagnosis, Differential ; Female ; Humans ; Korea ; Middle Aged ; Thymus Gland ; Weight Loss

Surgical reconstruction of malignancies of the head and neck often leave large defects that demands reconstruction. A maxillectomy defect creates a communication from oral cavity to nasal cavity that may extend to the orbit. This can leave a large anatomical defect that invades surrounding anatomical boundaries including the oral cavity, nasal cavity, orbital cavity, soft tissues of the face, and anterior skull base. Surgical repair of maxillary defects has been widely reported. Skin graft, local and regional flaps such as local mucosal flaps, buccal fat pad, temporalis muscle and pectoralis major muscle pedicled flaps, and free tissue transfer can be used depending largely on the size of the defect. We performed facial reconstruction using a latissimus dorsi musculocutaneous free flap for covering large defects that involved exposed orbit, nasal, and oral cavities in seven patients after total maxillectomy for maxillary cancer. One case was immediate reconstruction and the others were secondary reconstruction during the follow up period after primary cancer surgery. The skin of the latissimus dorsi musculocutaneous flap was pliable and its texture was similar to that of the face. The muscle bulkiness was sufficient to reconstruct the soft tissue of the intraoral and nasal lining and external skin deficits. All flaps had survived and serious complications were not developed. None of the patients need secondary defatting procedures later for the excessive bulkiness, but oronasal fistulas developed in two patients and one patient had cicatrical ectropion of lower eyelid. All donor defects were closed primarily and there has been no noticeable residual functional problems or discomfort in the shoulder area.
Adipose Tissue ; Ectropion ; Eyelids ; Fistula ; Follow-Up Studies ; Free Tissue Flaps* ; Head ; Humans ; Mouth ; Myocutaneous Flap ; Nasal Cavity ; Neck ; Orbit ; Shoulder ; Skin ; Skull Base ; Superficial Back Muscles* ; Surgical Flaps ; Tissue Donors ; Transplants

A carotid body tumor, also known as a paraganglioma in neck, originates from cells of neural crest tissue and arises at the carotid bifurcation. Carotid body tumors may present at any age and do not occur more frequently in either sex. A 39-year-old woman visited our hospital with a mass on the left side of her neck. She had first noticed the mass 20 years ago, and it has rapidly grown recently, especially in last two years. Magnetic resonance imaging showed a large solid mass with marked posterior and superior extensions splaying at the carotid bifurcation. In addition, carotid arteriography revealed a solid vascular mass in the carotid bifurcation and many feeding vessels from large adjacent arteries. Under the fluoroscopy control, PVA particles and coils were used for embolization. On completion, angiogram demonstrated as approximately 80% reduction in tumor vascularity with obliteration of most of the afferent tumor vessels. Thereafter, the patient underwent surgery under general anesthesia. An incision was made along the anterior border of the sternocleidomastoid muscle and the carotid sheath was opened. The tumor was carefully dissected from the common carotid, external carotid, and then the internal carotid arteries which has close adherence of the sheath to the tumor. The internal jugular, facial veins, spinal accessory nerves and branches of vagus nerve were sacrificed. On gross finding, the tumor was a well encapsulated soft tumor mass measuring 12 x 9 x 6 cm in size and completely surrounded by a fibrous capsule. The patient was discharged 21days after the operation with satisfactory result and no specific neurologic deficit.
Accessory Nerve ; Adult ; Anesthesia, General ; Angiography ; Arteries ; Carotid Artery, Internal ; Carotid Body Tumor* ; Carotid Body* ; Female ; Fluoroscopy ; Humans ; Magnetic Resonance Imaging ; Neck* ; Neural Crest ; Neurologic Manifestations ; Paraganglioma ; Vagus Nerve ; Veins

A large gap in peripheral nerve will not allow effective regenaration unless a grafting conduit is used to bridge the defect. Conventionally, nerve tissue has been used as a conduit in nerve reconstruction; however, results from techniques using these grafts are often unsatisfactory. A number of recent investigations have indicated that nerve fibers will regenerate through a nonneural tube. A 45-years-old female patient with a protruding mass on her lateral neck area visited our hospital. She complained of tingling sensation on the lateral aspect of palm, extending to 4, 5th finger. Mild degree of hypothenar muscular atrophy was also observed. Magnetic resonance image showed well-circumscribed mass with homogenous density located in brachial plexus area. We performed tumor excision with conglumerated C7 resection. The histologic finding was schwannoma with mixed type of Antoni A and B. A piece of sternocleidomastoid muscle was used as a conduit to bridge the gap and entubulization, the implantation of silicone guide tubes, was performed as an alternative method to the repair of transected nerves in the silicone tubes. The proximal and distal nerve stumps are introduced into each end of the tube and are held in place by epineural sutures. During three months of follow-up period, neuropathic pain and tingling sensation slowly subsided. Nerve conduction test and electromyography performed at the time of 86th day after the surgery showed much improved pattern compared to those performed on the 14th day after the operation. These results show that basement membrane of muscle fiber and silicone tube are able to orientate and promote peripheral nerve regeneration in a manner analogous to the endoneurial tubes of peripheral nerves.
Basement Membrane ; Brachial Plexus* ; Electromyography ; Female ; Fingers ; Follow-Up Studies ; Humans ; Muscle, Skeletal* ; Muscular Atrophy ; Neck ; Nerve Fibers ; Nerve Regeneration ; Nerve Tissue ; Neural Conduction ; Neuralgia ; Neurilemmoma* ; Peripheral Nerves ; Regeneration ; Sensation ; Silicones* ; Sutures ; Transplants

For the reconstructive procedures in the head and neck region, the methods range from local, mostly myocutaneous, flaps and skin grafts to free flaps. To ensure a satisfactory functional and aesthetic result, good texture and color of the flap are essential. Especially in the face, surgical reconstruction has to take account of the aesthetic units and a thin flap to enable mimic function. Moreover, the donor defect needs to be reduced without functional or aesthetic impairment. As a basic concept, first formulated by Gillies in 1920, the more adjacent the donor site is, the better the skin will match the recipient site. To achieve these goals, a thin reliable flap, harvested close to the face and neck region with good skin texture match, and a hairless sensated skin surface are needed. We have found that the shoulder is a region providing an optimum skin texture match to the neck. In this article, fasciocutaneous island flap, nourished by the supraclavicular artery, was used through the subcutaneous tunnel for the defect of neck. We used this flap in 2 patients who have supraglottic squamous cell carcinoma and lateral neck soft tissue defect after cellulitis. The functional result was satisfactory and the tunneling maneuver significantly improves the donor site aesthetically by reducing the scar. So we report these present clinical two cases of the flap in reconstructive procedures of neck.
Arteries ; Carcinoma, Squamous Cell ; Cellulitis ; Cicatrix ; Free Tissue Flaps ; Head ; Humans ; Neck* ; Shoulder ; Skin ; Tissue Donors ; Transplants

The traditional focus of surgeon of all disciplines has been on curing patients. Unfortunately, this focus on cure has sometimes resulted in neglect of the functional outcomes and QOL that are experienced by patients. The treatment of oral cavity and oropharyngeal cancer can result in marked alteration in the functional status and general health. However, few studies have addressed themselves to measuring the speech, swallowing results, and the quality of life after definitive therapy. By use of sophisticated techniques, we can now document, quantify, and critically analyze the surgeon's reconstructive efforts. Using these methods, we have evaluated the radial forearm free flap in 26 patients receiving oral reconstruction between 1994 and 1999. The radial forearm fasciocutaneous free flap has proved extremely versatile in reconstructing defects of the orofacial region and has become a workhorse flap in head and neck reconstruction. It has advantages including small bulk, easy dissection, vascularity, and malleability. This study evaluated long term effects on performance statusin a cohort of patients successfully treated with a surgical approach. The results were compared by site and treatment modality. These patients age ranged from 33 to 75 years with a mean age of 52.6 years. The 10 patients in this series did not have preoperative radiotherapy, while sixteen other patients underwent full curative dosages of 6000-6300 Gy within six weeks of surgery. Sites of the lesions included the anterior two thirds of thw tongue, base of tongue, floor of mouth, and tonsil. The functional status were generally excellent. The speech, swallowing, aspiration, oral incompetence, VPI were examined in all pateints. The total score1 12.9 in mobile tongue cancer patients(hemiglossectomy), 7 in total glossectomy patients and 11.0 in tongue base, 12.5 in mouth floor, 9.5 in tonsilar cancer patients. Postopeative radiation therapy didn't affect the functional disability and the sensate flap can give more benefits for postoperative early rehabilitaition than non-sensate flap. Despite the devastating effects of ablative surgery, the application of avaliable reconstructive techniques can help to improve the quality of life of these patients. Our series serves to identify site-specific speech and swallowing problems to facilitate posttreatment rehabilitation of patients with oral cavity and oropharyngeal cancer.
Cohort Studies ; Deglutition ; Forearm* ; Free Tissue Flaps* ; Glossectomy ; Head ; Humans ; Mouth Floor ; Mouth* ; Neck ; Oropharyngeal Neoplasms ; Palatine Tonsil ; Quality of Life ; Radiotherapy ; Rehabilitation ; Tongue ; Tongue Neoplasms