Pediatric Surgery (99-107)
Biologically active benign vascular tumor are characterized initially by cellular proliferation, and followed in most cases by involution.
Appear first few weeks after birth, commonly located on the head and neck, may be superficial or deep and may involve the viscera.
Superficial lesions, aka capillary hemangiomas are firm, bright red and raised. Deep or cavernous, lesions are softer and may have blue discoloration, less likely to resolve.
Most should be left alone. Indications for treatment include significant facial distortion, interference with fxn, thrombocytopenia from platelet trapping, and CHF.
Management includes steroids, cyclophosphamides, a-interferon, embolization, and surgical excision.
Biologically inert errors of morphogenesis of vessels. They are not proliferative and only grow with the child. Remain stable over time.
Port wine stain, is seen at birth as a red or purple non-raised lesion, usually on the face. Arteriovenous fistula is another example of a malformation.
Common on the extremities and the CNS. In the extremities they are usually multiple and may cause heart failure and hypertrophy of the affected limb.
Port wine stain never regresses and is best treated with laser photocoagulation.
Tumors in Children
Most common extracranial solid tumor of childhood. Has the ability to undergo maturation to a benign form, ganglioneuroma. It spontaneously disappears more than any other human malignancy.
Derived from embryonic neural crest tissue. Arises anywhere in SNS. Three-quarters are intraabdominal. Of these, most arise from adrenal medulla. Most children will secrete catecholamines and their breakdown products in the urine.
Abdominal mass is the present feature in most patients. Tumors in the mediastinum may produce respiratory distress, or may cause Horner’s.
Systemic features include fever, weight loss, failure to thrive, anemia, and HTN. Most have metastasis at dx.
Surgical resection remains the mainstay of treatment and is the only method of a cure. Survival rate is 40-50%. Survival rate of infants is 75% and children older than two is only 20%.
Embryonal neoplasm of kidney. Associated with other anomalies such as hypospadias, hemihypertrophy, and aniridia (congenital absence of iris).