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Brazilian Journal of Otorhinolaryngology 2020
Topics: Airway Obstruction; Head and Neck Neoplasms; Humans; Infant; Lymphangioma; Male; Picibanil; Sclerosing Solutions; Sclerotherapy; Tracheostomy; Treatment Outcome
PubMed: 27297957
DOI: 10.1016/j.bjorl.2016.04.016 -
Journal of the Chinese Medical... Dec 2014Chylothorax, a relatively rare complication of thoracic surgery, mostly occurs on the right side. We present a 16-year-old male who received thoracoscopic surgery for...
Chylothorax, a relatively rare complication of thoracic surgery, mostly occurs on the right side. We present a 16-year-old male who received thoracoscopic surgery for left spontaneous pneumothorax. Chylothorax developed on the postoperative 2(nd) day and resolved after diet control on the 4(th) day. Unfortunately, chylothorax recurred 2 weeks later. Chest drainage and nil per os with total parental nutrition were given but in vain. Thereafter, chemical pleurodesis with OK-432 was performed. Chylothorax resolved on the next day. The relevant literature is reviewed and possible pathogenesis clarified.
Topics: Adolescent; Animals; Chylothorax; Humans; Male; Picibanil; Pleurodesis; Pneumothorax; Postoperative Complications
PubMed: 25088907
DOI: 10.1016/j.jcma.2013.09.010 -
Medicine Nov 2017Linear nevus sebaceous syndrome (LNSS) is a rare neurocutaneous syndrome, characterized by nevus sebaceous,central nervous system (CNS), ocular and skeletal...
RATIONALE
Linear nevus sebaceous syndrome (LNSS) is a rare neurocutaneous syndrome, characterized by nevus sebaceous,central nervous system (CNS), ocular and skeletal abnormalities. The present study describes KRAS somatic mosaic mutation in a case of LNSS with lymphatic malformations (LMs).
PATIENT CONCERNS
A 4-month-old female with a clinical diagnosis of LNSS presented with infantile spasms, mental retardation, skull dysplasia, ocular abnormalities, congenital atrial septal defect, and LMs.
DIAGNOSIS
Cervical ultrasonography revealed a 4.6 × 4.6 × 2.2cm no echo packet with clear boundary in the subcutaneous tissues of the right neck. The neck MRI indicated a cyst in the subcutaneous tissues of the right neck. Whole-exome sequencing revealed a low-level heterozygous mutation of the KRAS gene (c.35C > T; p.G12D, 19%) in the skin lesion sample. This mutation was not present in the blood samples of the patient and her parents.
INTERVENTIONS
The patient received sclerotherapy with paicibanil (OK-432) injection for the cyst.
OUTCOMES
Following 1 year of treatment, the patient exhibited fewer seizures. The mental and motor development was significantly improved. The patient can currently walk with assistance and speak simple words.
LESSONS
LNSS is a rare, congenital neurocutaneous syndrome consisting of a spectrum of abnormalities involving the skin, central nervous system, eyes, LMs and other systems. LNSS can be caused by postzygotic somatic mutation in the RAS family of genes. Multidisciplinary evaluation and treatment is needed.
Topics: Antineoplastic Agents; Female; Humans; Infant; Nevus, Sebaceous of Jadassohn; Picibanil; Sclerotherapy
PubMed: 29381910
DOI: 10.1097/MD.0000000000008016 -
Pediatrics and Neonatology Jun 2016Retroperitoneal lymphangioma is extremely rare. Although these neoplasms are benign, they can grow progressively with subsequent compression and infiltration of the...
Retroperitoneal lymphangioma is extremely rare. Although these neoplasms are benign, they can grow progressively with subsequent compression and infiltration of the adjacent structures. Surgical excision is demanding when the lesion surrounds vital structures and it is generally fraught with a high recurrence and morbidity rate. We report the case of a huge retroperitoneal lymphangioma in a newborn treated successfully with intracystic injection of OK-432.
Topics: Antineoplastic Agents; Humans; Infant, Newborn; Injections; Lymphangioma; Male; Picibanil; Retroperitoneal Neoplasms; Sclerotherapy
PubMed: 24140312
DOI: 10.1016/j.pedneo.2013.06.011 -
BMC Medical Imaging Jul 2017Congenital cystic lymphangiomas are benign malformations due to a developmental disorder of lymphatic vessels. Besides surgical excision, sclerosant therapy of these...
BACKGROUND
Congenital cystic lymphangiomas are benign malformations due to a developmental disorder of lymphatic vessels. Besides surgical excision, sclerosant therapy of these lesions by intracavitary injection of OK-432 (Picibanil®), a lyophilized mixture of group A Streptococcus pyogenes, is a common therapeutical option. For an appropriate application of OK-432, a detailed knowledge about the structure and composition of the congenital cystic lymphangioma is essential. SonoVue® is a commercially available contrast agent commonly used in sonography by intravenous and intracavitary application.
CASE PRESENTATION
Here we report the case of 2 month old male patient with a large thoracic congenital cystic lymphangioma. Preinterventional imaging of the malformation was performed by contrast-enhanced ultrasound after intracavitary application of SonoVue® immediately followed by a successful sclerotherapy with OK-432.
CONCLUSIONS
Contrast agent-enhanced ultrasound imaging offers a valuable option to preinterventionally clarify the anatomic specifications of a congenital cystic lymphangioma in more detail than by single conventional sonography. By the exact knowledge about the composition and especially about the intercystic communications of the lymphangioma sclerosant therapy becomes safer and more efficient.
Topics: Contrast Media; Humans; Infant; Lymphangioma; Male; Microbubbles; Picibanil; Sclerosing Solutions; Sclerotherapy; Treatment Outcome; Ultrasonography
PubMed: 28683719
DOI: 10.1186/s12880-017-0213-9 -
Sultan Qaboos University Medical Journal Feb 2018Head and neck masses can present in different pathologies that usually vary according to the age of the patient. We report five cases of benign head or neck masses...
Head and neck masses can present in different pathologies that usually vary according to the age of the patient. We report five cases of benign head or neck masses occurring among patients of different ages who were managed at the Bahrain Defence Force Royal Medical Services Hospital, Ar-Rifaa, Bahrain, between 2005-2014. All of the patients were treated using the sclerotherapeutic agent OK-432. Although surgical removal is usually considered optimal treatment in the management of such cases, OK-432 appears to be a promising alternative.
Topics: Adult; Bahrain; Child, Preschool; Cysts; Female; Head; Head and Neck Neoplasms; Humans; Infant; Male; Middle Aged; Neck; Picibanil; Sclerotherapy
PubMed: 29666687
DOI: 10.18295/squmj.2018.18.01.014