Propranolol as an alternative treatment option for pediatric lymphatic malformation.

2013

Ozeki M, Kanda K, Kawamoto N, Ohnishi H, Fujino A, Hirayama M, Kato Z, Azuma E, Fukao T, Kondo N.

Source

Department of Pediatrics, Gifu University Graduate School of Medicine, Gifu University.

Abstract

Lymphatic malformation (LM), which was previously termed lymphangioma, is a rare congenital malformation of the lymphatic system and its treatment is still challenging. Propranolol (beta blocker) has been recently developed as a first-line treatment of infantile hemangioma. Our study aimed to assess the effect of propranolol on pediatric LM and the relationship between its effectiveness and vascular endothelial growth factor (VEGF) family members (VEGF-A, C and D). Six Japanese patients with LM (age range: 10 months-19 years old; 2 macrocystic, 2 microcystic and 2 combined type) were enrolled. Oral propranolol was administered at 2 mg/kg/day. The efficacy of propranolol for LM was evaluated by the rate of volume change as calculated from MRI imaging and by symptomatic improvement. In all patients, there were no significant side effects. Patients 3 and 5 were classified as objective responders with tumor volume reduction of 30.6% and 22.9%, respectively, at 24 weeks. Patient 1 showed 8% tumor volume reduction and patient 6 showed symptomatic improvement, hence, both were classified as minimal responders. The other two patients were classified as non-responders. Plasma VEGF-A, C, and D levels were significantly higher in the LM group than in the controls (all P < 0.01 by Mann-Whitney test). VEGF-A and D levels at 24 weeks were significantly lower than those at pre-treatment (P = 0.031, 0.047 by Wilcoxon matched pairs test). Though further trials with this treatment must be carried out, we propose that propranolol may be an alternative therapy option for intractable LM.

PubMed

Case report of fetal axillo-thoraco-abdominal cystic hygroma.

Case report of fetal axillo-thoraco-abdominal cystic hygroma.

Arch Gynecol Obstet. 2009 Apr 10

Masood SN, Masood MF.
Dow University of Health Sciences, Karachi, Pakistan, sh_naz@yahoo.com.

Cystic hygroma (moist tumor) was first described in 1828 by Redenbacher. The cyst usually results owing to an absence or an inefficient connection between the lymphatic and venous systems. Of this type of malformation 75% cases are localized in the nuchal region; however, only 20% are found in the axilla while 5% of these hygromas are in other locations. Prognosis depends on associated fetal co-morbidities. There are many case reports on cystic hygroma but only a few on the axillo-thoraco-abdominal variant. This is a case report of a huge late-onset fetal axillo-thoraco-abdominal cystic hygroma, which was diagnosed at term followed by a difficult vaginal delivery in a 38-year-old woman. The baby did not have any congenital anomaly other than cystic hygroma with no evidence of intrathoracic or intra-abdominal extension of mass and a pelvic kidney reported on neonatal ultrasound and CT scan. The surgical excision of the cyst was done on the fourth day following birth and the histopathology report confirmed the diagnosis. Management of fetal cystic hygroma with the use of a sclerosing agent is a new modality being explored. Risk of recurrence in subsequent pregnancies for aneuploidy is not increased. The baby has been followed up to 5 months of birth and is thriving well. Karyotype shows an XX pattern.

SpringerLink

Abdominal lymphangioma in children: Report of three cases.

Abdominal lymphangioma in children: Report of three cases.
Surg Today. 2009
Muramori K, Zaizen Y, Noguchi S.
Department of Pediatric Surgery, Matsuyama Red Cross Hospital, Matsuyama, Japan.

Most cases of abdominal lymphangioma are asymptomatic. However, patients may occasionally present with acute abdomen because of an intestinal obstruction or peritonitis caused by infected cysts, hemorrhaging, and/or torsion. These conditions may differ based on the location of the lymphangioma and do not always require emergency surgery. This report presents two cases of abdominal lymphangioma derived from the omentum, which required emergency surgery because of severe abdominal distension and peritonitis caused by torsion, respectively. In contrast, another case of retroperitoneal lymphangioma presented with acute abdomen and underwent elective surgery after conservative therapy for peritonitis. Elective surgery is acceptable if the symptoms are controllable under conservative therapy. However, it is important not to overlook the possible lethal complications such as intestinal and/or urological obstruction, aggressive peritonitis, and torsion of the cyst, which require emergency surgery.

SpringerLink