picibanil and Edema

Lymphatic malformation in the orbital cavity and surrounding region often causes disfigurement and visual problems. To better clarify the evolution and treatment of this condition, the authors studied a retrospective cohort of 42 consecutive patients seen between 1971 and 2003 and analyzed anatomic features, complications, and management. The ratio of female to male patients was 1:1. Most periorbital lymphatic malformations were noted at birth (59 percent), presenting as either unilateral swelling (60 percent) or a periorbital mass (24 percent). Sixty-two percent of lesions were on the left side. The ipsilateral cheek, temple, and forehead also were involved in 57 percent of patients. Twenty-two percent of lesions were intraconal, 30 percent were extraconal, and 48 percent were in both spaces. Forty-five percent of children had an associated cerebral developmental venous anomaly. Periorbital lymphatic malformation caused major morbidity; 52 percent of patients had intralesional bleeding and 26 percent of patients had a history of infection. Other common complications included intermittent swelling (76 percent), blepharoptosis (52 percent), proptosis (45 percent), pain (21 percent), amblyopia (33 percent), chemosis (19 percent), astigmatism (17 percent), and strabismus (7 percent). Ultimately, 40 percent of children had diminished vision and 7 percent became blind in the affected eye. Management of periorbital lymphatic malformation involved an interdisciplinary team that included an interventional radiologist, a craniofacial surgeon, and an ophthalmologist. The two therapeutic strategies were sclerotherapy (40 percent) and resection (57 percent); most patients required several interventions. A coronal approach was used for subtotal excision of fronto-temporal-orbital lymphatic malformation in 13 patients, whereas a tarsal incision was used for lesions isolated to the eyelid (n = 14). Ocular proptosis was temporarily managed by tarsorrhaphy (n = 9), but expansion of the bony orbit was needed to correct persistent proptosis (n = 8). Orbital exenteration was necessary in two patients.

Topics: Abnormalities, Multiple; Adolescent; Adult; Blepharoptosis; Blindness; Cerebral Veins; Child; Child, Preschool; Cohort Studies; Combined Modality Therapy; Edema; Exophthalmos; Eye Infections; Face; Female; Hemorrhage; Humans; Infant; Infant, Newborn; Lymphatic Abnormalities; Male; Orbit; Picibanil; Plastic Surgery Procedures; Retrospective Studies; Sclerotherapy; Vision Disorders

Chylothorax results from various causes, such as malignancy, trauma, or infection. POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a multisystemic syndrome that is associated with plasma cell disorder. Pleural effusion is a common manifestation of POEMS syndrome, but the association of POEMS syndrome with chylothorax has not been reported. We report on a 61-year-old female patient who initially presented with dyspnea and bilateral leg edema. Importantly, the patient had normal renal function. Her chest X-ray and computed tomographic imaging showed bilateral pleural effusion, and her chest drainage revealed chylothorax. Detailed examination failed to reveal the definitive cause of the chylothorax. She received several treatments for chylothorax, namely, a low-fat diet or fasting, total parenteral nutrition, a somatostatin analog (octreotide), thoracic duct ligation by video-assisted thoracic surgery, and pleurodesis. However, further examination revealed endocrinopathy, monoclonal plasma cell disorder, peripheral neuropathy, and elevation of the serum level of vascular endothelial growth factor. The patient's condition was consequently diagnosed as POEMS syndrome. Eventually, her chylothorax was controlled by pleurodesis, and she was transferred to another hospital for stem cell transplantation. Herein, we report on the apparent first case of POEMS syndrome with chylothorax. In some cases of idiopathic chylothorax, the underlying primary disease may be latent, such as in the present patient. POEMS syndrome is rare, but this syndrome should be included in the differential diagnosis of chylothorax with unexplained etiology.

Topics: Chylothorax; Diagnosis, Differential; Dyspnea; Edema; Female; Humans; L-Lactate Dehydrogenase; Leg; Middle Aged; Picibanil; Pleural Effusion; POEMS Syndrome; Thoracic Surgery, Video-Assisted; Treatment Outcome; Triglycerides

The case of recurrent intraabdominal neurenteric cyst after surgical excision in an infant is described. After several operative resections, we changed therapeutic strategy and performed local injection of OK-432. Indeed, there is experience with the application of OK-432 for other entities, especially lymphangioma, but to our knowledge, there is no report published so far on the treatment of neurenteric cyst with OK-432. We describe for the first time an effective and simple treatment of recurrent neurenteric cyst.

Topics: Abdomen; Combined Modality Therapy; Edema; Fistula; Follow-Up Studies; Humans; Infant, Newborn; Injections, Intralesional; Instillation, Drug; Irritants; Leg; Male; Neural Tube Defects; Pelvis; Picibanil; Recurrence; Reoperation; Sacrococcygeal Region; Urinary Bladder, Neurogenic