by Omo Aisagbonhi
Vanderbilt University School of Medicine
We had orthopedic clinic today. Many of our adult patients presented with fractures secondary to road traffic accidents. The pediatric cases were more varied. Some of the patients seen included:
- 2yo with hereditary neurofibromatosis; over here, neurofibromatosis is diagnosed clinically. The diagnostic criteria are the presence of two or more of the following: 1. six or more café-au-lait spots 5mm or greater in diameter; 2. freckling in the armpit or groin; 3. two or more neurofibromas of any type or one plexiform neurofibroma; 4. two or more Lisch nodules; 5. optic glioma; 6. distinctive bone lesions, particularly an abnormally formed sphenoid bone or tibial pseudoarthrosis; and 7. a first-degree relative diagnosed with neurofibromatosis using the above criteria. This two year old has several café-au-lait spots along with left leg hypertrophy, with left long being longer and wider than the right, due to both bone (femur, tibia and fibular) and soft tissue hypertrophy. She also has a lower limb deformity, genu valgus, which we plan to correct at a later date. Her mom has several café-au-lait spots (but no limb abnormalities) and her 5yo older sister has no café-au-lait spots, but hypertrophic left hand digits and left toes. Her mom and sister did not carry official neurofibromatosis diagnoses, but do now since she clearly has neurofibromatosis based on clinical criteria. Genetic tests are not readily available, but this is a classic example of variable expression routinely observed in autosomal dominant neurofibromatosis.
- 4yo with club foot secondary to polio; we did her corrective surgery today. The surgery was interesting. First, we did an ETA (elongation of the tendo-achilles), in which the calcaneal (Achilles) tendon was identified and bisected in a Z configuration, then re-attached. Then, the bursa deep to the calceneal tendon was ruptured. Finally, a wedge resection of the cuboid was performed. Pins were placed to close the resected segment; subcutaneous tissue and skin were closed, and the foot was placed in a cast to help set it in the proper configuration.
- 2yo M with bowed legs (bilateral genu varus). The big thing was to rule out Ricketts. This was assessed by checking for costochondrial rosary beading, wrist flaring and forehead bossing. The plan is to have him follow up in 6months. Follow up involves regular measurement of the intercondylar distance and varus angle; expect correction in physiological bowing. The orthopedic surgeons here have observed that an intercondylar distance <9cm is likely to correct itself with time; >9cm often needs to be surgically corrected.
- 14yo with Blount’s disease (pathological bowing). She had very bowed legs that had been surgically corrected two other times in childhood, but became bowed as she grew. Plan is for one more correction in a year or so; she’s likely stopped growing and would no longer need corrections after this one.
- 6yo with physiologic genu valgus expected to correct on its own with time; for valgus, the follow up is of the inter malleolar distance and valgus angle.
- 2yo with foot drop and high stepping gait secondary to IM injection that caused damaged to the common peroneal branch of her sciatic nerve. Plan is for long-term physiotherapy and toe-raising splint.
- 3mo with osteogenesis imperfecta which was clinically diagnosed on basis of neonatal femoral and tibial fractures as well as the presence of bluish sclera (she was compared to her healthy twin). This was quite interesting to me as I did not expect to see a child with OI in Nigeria. Anyway, the plan is to treat her fractures as they occur; babies with her type of OI (OI type I, not type II, which is so severe the babies die following the trauma of birth) usually do well as they learn to avoid doing things that will cause them injury.
- 14yo with congenital absence of the R. proximal femur that walks by bending her left leg to compensate for the very short right leg; she appears to be kneeling/crawling as she walks, but she’s able to stand upright on the L. leg. The correction would be to amputate the R. lower limb and place a prosthetic; she does not want this.
- Neonate with bilateral club foot due to congenital bilateral absent fibulas. We placed casts to begin serially correcting the clubbing.
- 28yo with remote history of ectopic pregnancy, presented with bilateral hip pain. X-ray showed bilateral cystic lesions at the femoral head and narrowed interarticular space. We did a TB skin test (which is non-specific in this population exposed to BCG vaccine, but the more specific IFN-gamma release assay is also considerably more expensive), also sent her for HIV testing and her blood group was checked because people with sickle cell disease are prone to avascular necrosis of the hip (she’s AA). I think, given her h/o ectopic pregnancy, there is a likelihood of gonorrhea/chlamydia exposure and possibility of septic arthritis from gonococcus; but this would be an atypical presentation.
Later in the day, I attended morbidity and mortalilty conference where I learned that the most common admissions on the medicine service are due to cardiovascular/ cerebrovascular diseases and CHF; not infectious diseases though, in the month of July, one patient died of malaria and another of AIDS. Another thing that was interesting to note is that for some patients, cause of death ends up being undetermined. E.g. the case presented of a man who presumptively died of hypoglycemia secondary to metastatic liver cancer; assumed to have liver cancer because his liver enzymes were elevated and he had a bone tumor. However, he was initially diagnosed with bone TB in the ortho clinic, but did not receive TB treatment (again, here, TB diagnosis is non-specific due to BCG vaccination). He never had any scans to show or disprove liver tumor and did not have an autopsy at death. All we know is that he came to the hospital with hypoglycemia and his labs showed elevated liver enzymes. There is a need for better access to pathology.