Lymphatic malformations originate during fetal development as a result of lymphatic obstruction, hypoplasia, dysplasia, and _____?

1.   ?    Aplasia
2.   ?    Tumor
3.   ?    Infection
4.   ?    In utero kinking

When do the lymphatic sacs begin to develop?

1.   ?    Neck develops at 10th week of gestation
2.   ?    Iliac sacs develop at 10th week of gestation
3.   ?    Neck develops at 6th week of gestation

Which genetic syndrome is NOT associated with an increased incidence of lymphedema?

1.   ?    TurnerÂ’s syndrome
2.   ?    DiGeorgeÂ’s syndrome
3.   ?    NoonanÂ’s syndrome

Which is NOT one of the 3 types of lymphangioma?

1.   ?    Lymphangioma simplex (small channels)
2.   ?    Cavernous lymphangioma
3.   ?    Cystic hygroma
4.   ?    Lymphangioma multiforme

What percentage of lymphangiomas are present at birth?

1.   ?    10%
2.   ?    33%
3.   ?    66%
4.   ?    80%

What is the most common complication of lymphangioma?

1.   ?    Bleeding
2.   ?    Enlargement
3.   ?    Infection - local
4.   ?    Sepsis

What is the most common site of cystic hygroma?

1.   ?    Axilla
2.   ?    Neck
3.   ?    Mediastinum
4.   ?    Inguinal

True or false: Recurrence rates after resection of lymphangioma are ____?

1.   ?    1 – 2%
2.   ?    Less than 5%
3.   ?    5 – 15%
4.   ?    20%

True or false: Intralesional Bleomycin A-5 has been used as primary treatment for cystic hygroma, with resolution reported in up to 55% of patients.

1.   ?    True
2.   ?    False

What is OKT-3?

1.   ?    Steptococcal derivative
2.   ?    Fungal derivative
3.   ?    Synthetic analog of interferon gamma

At what age is lymphangioma of the intestine most commonly seen?

1.   ?    Newborn period
2.   ?    Less than 3 years of age
3.   ?    Preschool
4.   ?    Adolescence

True or false: pulmonary lymphangiectasia is often fatal.

1.   ?    True
2.   ?    False

True or false: Lymphangiectasia of bone does not occur.

1.   ?    True
2.   ?    False

Which of the 3 categories of lymphedema accounts for 80% of primary lymphedema?

1.   ?    Congenital lymphedema (MilroyÂ’s disease)
2.   ?    Lymphedema praecox (MeigeÂ’s disease)
3.   ?    Lymphedema tarda

Primary lymphedema occurs principally in girls or boys?

1.   ?    25% Female
2.   ?    50% Female
3.   ?    70% Female
4.   ?    90% Female

T or F: Milroy's disease is inherited in an autosomal recessive manner.

1.   ?    True
2.   ?    False

What is yellow nail syndrome?

1.   ?    Fungal infection of the nail
2.   ?    Nail pulp lymphangioma
3.   ?    Nicotine staining in chronic smokers
4.   ?    Blockage of nail bed lymphatics

T or F: MRI is useful in differentiating lymphangioma from lymphedema?

1.   ?    True
2.   ?    False

What is the pathology in 90% of children with lower extremity lymphedema?

1.   ?    Microscopic webs in central or peripheral lymphatics
2.   ?    Hypoplasia of the distal lymphatics
3.   ?    Central aplasia of lymphatics

How often can patients with lower extremity lymphedema be managed without operation?

1.   ?    20%
2.   ?    50%
3.   ?    75%
4.   ?    More than 85%

Which is NOT a complication of lymphedema?

1.   ?    Right heart failure
2.   ?    Brawny edema
3.   ?    Skin thickening or discoloration
4.   ?    Cellulitis and lymphangitis

T or F: Pneumatic sequential pumping systems are very effective in treating chronic lymphedema.

1.   ?    True
2.   ?    False

What is the most effective surgical approach to lymphedema unresponsive to conservative therapy?

1.   ?    Microvascular anastomosis
2.   ?    Subcutaneous OKT3
3.   ?    Subcutaneous resection, sequentially