Question 1#

In obstetrical brachial plexus paralysis (OBPP):

A. Primary suture without tension is frequently possible

Babies who do not recover biceps function by the age of 3 months should be considered for immediate operation. 

Also note:

• Primary suture without tension is rarely possible. Nerve grafting is usually necessary for root or trunk ruptures.
• In the presence of root avulsions an internal neurotization should be attempted between different roots, particularly as children seem to have a far greater capacity to accommodate to differential neurotizations.
• When it is not possible to perform an internal neurotization, an external neurotization can be performed using one or more of the following donor nerves in the following order of preference: the pectoral nerves, the intercostal nerves and the accessory nerve.
• The reconstruction should be protected from excessive motion for the first 3 weeks.
• Physiotherapy should be continued up to 2 years of age but then continued by the parents in the form of play and activities of daily living.
• Secondary surgery can be considered when it is clear that recovery following reconstruction is no longer progressing.

References:
1. Gilbert A. Primary repair of the obstetrical plexus. Indian Journal of Plastic Surgery 2005; 38(1): 34-42.

Question 2#

Which statement concerning haemangioma is incorrect?

A. Present in 10% of newborns with white skin

Present in 10% of newborns with white skin. This statement is incorrect; haemangiomas are present in approximately 10% of white skinned children by the age of 1 year, but only 1-2% of newborns. Female infants are three times more likely to have haemangiomas, and the incidence is increased in premature neonates. They occur in all races but less commonly in African or Asian skin. Although traditionally considered to be sporadic, autosomal dominant segregation within families has been described but rare. Haemangiomas present with a variety of precursor lesions in approximately 50% of cases and these can be present at birth.

Question 3#

The common starting dosage for the treatment of infantile haemangioma with oral prednisolone:

A. 0.002-0.004mg/kg/day

2-4mg/kg/day. Corticosteroids are the mainstay of therapy for haemangiomas that require treatment, most commonly administered orally, but intra-lesional and topical preparations can be useful. They are most useful in the proliferative phase. 2-4mg/kg/day is the standard starting regime but some advocate up to 5mg/kg/day and the therapy is usually continued for several months and gradually tapered as tolerated.

Question 4#

Which is not a feature of Klippel-Trenaunay syndrome (KTS)?

A. Limb overgrowth

Cerebriform palmar hyperplasia. This is not a feature of Klippel-Trenaunay syndrome (KTS). KTS is a sporadic disorder characterised by a triad of vascular malformation, venous varicosity and growth disturbance of soft tissue or bone. Capillary malformation of the port wine type is the most common vascular malformation but can be present with underlying lymphatic malformation. Overgrowth (hyperplasia) is most common but undergrowth can also occur in the affected limb. Cerebriform palmar hyperplasia is a feature of Proteus syndrome, another overgrowth syndrome.

Question 5#

A 7-year-old boy has hypernasality and velopharyngeal incompetence. He underwent repair of a ventricular septal defect at birth and repair of a cleft palate at age 9 months. His mother says that he has had difficulties with language learning. Physical examination shows upward slanting of the palpebral fissures, a broad nasal root, a small mouth, and a thin upper lip. Which of the following studies is most likely to lead to a diagnosis in this patient? 

A. Measurement of serum creatine kinase level

Fluorescent in situ hybridisation (FISH) analysis. This is the classic test to diagnose velocardiofacial syndrome, otherwise known as Di George syndrome, Shprintzen’s syndrome and catch 22 syndrome (chromosome locus 22q11.2; C - cardia, A - abnormal facies, T - thymic aplasia, C - cleft palate, H - hypocalcaemia). Inheritence is usually sporadic.

References:

1. Shprintzen RJ. Velocardiofacial syndrome. Otolaryngeal Chin North Am 2000; 33: 1217-40.
2. Raymond GV. Craniofacial genetics and dysmorphology. In: Plastic surgery: indications, operations, and outcomes. Achauer BM, Eriksson E, Guyuron B, Coleman JJ, Russell RC, Vander Kolk CA, Eds. St. Louis, USA: Mosby, 2000.