Megadactyly with syndactyly of the right toes, a rare case report.

INTRODUCTION AND IMPORTANCE: Megadactyly of the foot is uncommon non hereditary congenital anomalies of the extremities and poses a dilemma on treatment however multiple treatment modalities were developed but is not uniform to all patients with megadactyly. The goal of the surgical treatment is to achieve painless and function of the foot. CASE PRESENTATION: We report a 14 years old male presented with complaints of progressive enlargement 2nd, 3rd, 4th and 5th toes of the right foot since birth, associated with inability to wear shoes. One month prior to admission he experienced gradual onset painful forefoot and toes that was increasing in severity with time associated with inability to walk normally. He is the first born in a family of four children and his other siblings are healthy with no anomalies. On clinical evaluation, he was health with stable vitals, with enlarged 2nd, 3rd, 4th and 5th toes of the right foot with no tenderness with intact neurovascular status. On clinical and radiological evaluation he was diagnosed with congenital megadactyly of the right foot, a multidisciplinary team including orthopedic surgeons and prosthetics team agreed to do trans-metatarsal, then partial foot prosthesis fabrication. He underwent trans-metatarsal amputation of the affected foot and tissue sample was taken for histopathology. The histological findings revealed bone enlargement with increased proliferation of subcutaneous adipose tissues with increased fibrous septae together with thinning of the epidermis, features suggestive of lipomatosis. The wound site healed very well after 14 days stitches were removed and the child was scheduled for follow-up after six weeks, 12 weeks and 6 month post-surgery. On the last visit he was free from pain on his right forefoot and toes, able to wear fabricated partial foot prosthesis and shoes normally, walk with no incapacitation. DISCUSSION: Our case report is unique due to the involvement of the multiple toes of the right foot with syndactyly at third and fourth toes and its management is challenging because there is no uniformity in its surgical treatment, in our case trans-metatarsal amputation was done and the patient progressed well after six months of follow up. CONCLUSION: Foot megadactyly is uncommon congenital malformation, most common on the right foot. Regardless of the dilemma on treatment, the trans-metatarsal amputation and a fabricated prosthesis to our patient fulfilled the goals of painless right foot and able to wear shoes and walk normally with no impairment.

Spinal ganglioneuroma with extension to the brachial plexus. The challenges for total surgical excision, a case report.

INTRODUCTION: Ganglioneuromas are benign neurogenic tumors that arise from the sympathetic ganglia. They are less aggressive compared to the more immature neuroblastomas and ganglioneuroblastomas but can grow to exert mass effect on surrounding tissues. PRESENTATION OF CASE: A 7 years old girl who presented with progressive quadriplegia for 4 months. On examination, she had a right supra-clavicular mass with reduced power in the right hand than the left. Power in the lower limbs was also reduced with hyper-reflexia, clonus and Babinski positive. Laboratory investigations were unremarkable and Chest X-Ray showed a widened mediastinum. Magnetic Resonance Imaging (MRI) scan revealed an extra-medullary spinal tumor at C6/C7 extending laterally on the right through the C6/7 neuro-foramen to the para-spinal tissue and brachial plexus. A C6/7 laminectomy with Spinal cord decompression by partial resection of the tumor was done. Histology of the resected tissue showed ganglioneuroma. DISCUSSION: The presentation of Ganglioneuromas is usually asymptomatic until they are huge enough to exert mass effect on surrounding tissue. Most are located in the posterior mediastinum, retro-peritoneum and neck. Due to this, it may be very challenging to achieve total resection especially when they surround major vessels or nerves. A multi-disciplinary approach is needed for the best surgical outcomes but this is not always realized in our setting. CONCLUSION: In resource limited settings, more collaboration and training is needed to realize appropriate management of complex surgical conditions. Although complications are not uncommon, total surgical excision is necessary to prevent recurrence and progression of Ganglioneuromas.

HIV positive patient with bilateral osteonecrosis of the femoral head, a case report.

INTRODUCTION AND IMPORTANCE: Avascular necrosis (AVN), also known as osteonecrosis, refers to the death of bone tissue due to the lack of blood supply. Osteonecrosis in HIV can be a complication of the ART's or the disease itself. CLINICAL PRESENTATION: 47 years old male, HIV positive for 10 years on Antiretroviral-therapy had gradual onset of bilateral hip pain for 6 months, progressively and sharp in nature, aggravated by movement and relieved by resting with reduced range of movement, wheel chair dependent 2 months. No history of fever, cough, night sweats or weight loss. No history of trauma, steroid use or hormonal therapy and no history of alcohol intake. On Examination he had bilateral inguinal and hip tenderness, pain on movement, with reduced flexion and extension of the hip. His viral-load was 27copies/ml of blood. Complete blood count was unremarkable. Serum lipid panel had no evidence of hypertriglyceridemia. He was diagnosed with bilateral femoral heads Avascular-necrosis. Bilateral ceramic with polyethylene liner uncemented total hip arthroplasty was done. No complications observed, in 6 months of follow up he had Harris hip score of 90 and he had returned to his activities without hip pain complaints/complications. DISCUSSION: The management of AVN is usually total hip arthroplasty, but other surgical treatment includes, hemiarthroplasty, core-decompression and girdle stone arthroplasty, the latter has poor outcomes in-terms of quality of life. CONCLUSION: Understanding causes and mechanism of AVN is crucial for effective management and treatment, particularly when addressing cases such as in our patient with HIV induced osteonecrosis of both femoral heads, surgical treatment should aid on relieving pain and improving patient's quality of life.

Dural ectasia with cauda equina syndrome, a rare case report.

INTRODUCTION AND IMPORTANCE: Dural ectasia, which is often idiopathic, is seen both in patients with neurofibromatosis and Marfan's syndrome. In neurofibromatosis, the ectasia is most often seen in the thoracic region but can occur at any point along the dura. A complication such as cauda equina syndrome is usually rare. CLINICAL PRESENTATION: A 48 year old male complaining of recurrent throbbing headache, for 3 years, 2 years ago he developed progressively lower back pain, associated with numbness and tingling sensation of the lower limbs. A year ago he experienced defecation and urinary incontinence. On further questioning the patient reported to have first degree relative with neurofibromatosis. On examination he has multiple café au laite on the trunk, back and left arm, and plexiform on the left palm, mild right deviation on thoracic region on the back. Lower limb muscle power grade 4/5 bilaterally, sensation was intact. Laboratory work up Full blood counts, electrolytes, renal and liver function tests were normal, MRI of the lumbar spine demonstrate L3/L4 and L4/l5 mild disc bulge with no significant narrowing of the primary canal and no evidence of existing nerve root impingement, increase antero-posterior diameter of dura sac involving L5-S1, with a Dural Sac Diameter of S1 increased compared to that of L4 with mild scalloping of lower lumbar vertebra and pronounced at S1 vertebral body. A diagnosis of cauda equina syndrome and dural ectasia secondary to neurofibromatosis was rendered. Lumbar peritoneal shunting, was reached as a surgical treatment for this patient, but due to inadequate and unavailability of the required shunting equipment, the patient was managed conservatively with anti- inflammatory medications, lumbar CSF tapping, genital hygiene and counselling. 3 months of follow up, the patient was able to walk, with power 5/5 to both lower limbs, however fecal and urine incontinence persisted. DISCUSSION: this case was particularly unusual due to the combination of cauda equina syndrome and dural ectasia, Dural ectasia is seen with various conditions including Marfan syndrome, Ehlers-syndrome, neurofibromatosis 1, Ankylosing spondylitis, trauma, scoliosis or tumors it may also have no clear cause. In most cases patients with dural ectasia are asymptomatic few may present with low back pain, radicular pain in the buttocks or legs and headache and rarely caudal equina syndrome. The management of dura ectasia may be conservative for asymptomatic patient and for a symptomatic patient surgery such as stabilization, marsupialization and lumbar peritoneal shunt. CONCLUSION: Dural ectasia with cauda equina syndrome are rarely complication of neurofibromatosis. Familiarity with its classic imaging and clinical features as described in this case report can help its early detection and management.

Thoracolumbar junction translation injury in a patient with ankylosing spondylitis, a case report.

INTRODUCTION: The thoracic spine is stable because of kyphotic alignment, rib cage, and costovertebral joints. Any compression or kyphosis in the thoracic spine always causes spinal cord injury. CLINICAL CASE: A 47-year-old male with complaints of back pain 1 day prior to admission, after he sustained a motorbike crush and landed on his back. The pain, radiates to both limbs, associated with severe spasms, numbness, and weakness in his lower extremities, however no incontinence. No other associated injuries were reported. 25 years ago he had a history of tuberculosis of the spine with progressive deformity of the back, he was treated medically without surgery. On examinations: Gibbus at T11-L1, with hyper-pigmented post-inflammatory skin and an easily palpable spine, power 1/5 right and 2/5 left lower limbs, Sensation and bulbocarvenosus reflex were intact. Upper limbs were neurologically intact. All laboratory investigations including FBP, ESR, Electrolytes, renal and liver function tests were all within normal range. After radiological imaging, a final diagnosis of Spinal Cord Injury, ASIA C. AO classification type T12-L1:C/T9-L1:A4/N3/M2 was made. He was kept on a thoracolumbar corset 6 weeks after being initiated on spine protocol. He was discharged 8 weeks this time patient had no back pain but no improvement was noted neurologically. After a year of thoracolumbar corset and physiotherapy, he reported no more back pain, no numbness to lower limbs, and power 3/5 right and 4/5 left lower limbs, with intact sensation. However, no changes were observed radiologically. CLINICAL DISCUSSION: Due to the instability of fracture-dislocation, surgical treatment is recommended to realign the spine but for this case with back deformity and fractured vertebra bodies, it is best not to temper with reduction and fixation as it would further worsen the neurological deficit of the patient, during maneuvers while doing the reduction. CONCLUSION: Fracture-dislocation of the thoracic spine can impact the physical and mental well-being of the patients. Surgical fixation and instrumentation are ideal but in cases where surgical intervention would further impair the neurological function of the patient conservative management is the goal.

Invasive sclerosing epitheloid fibrosarcoma in the lumbar spine- A rare case report.

INTRODUCTION AND IMPORTANCE: Sclerosing epitheloid fibrosarcoma is a rare variant of low grade sarcoma with specific histologic and immunohistochemical features, and often associated with a poor prognosis. CLINICAL PRESENTATION: We report a case of a 35-year old male who presented with 2 year history of lower back pain, radiating to both lower limbs, weakness to both lower limbs and weight loss more than 5kgs in the past 6 months, no past history of trauma, drenching night sweats, no TB contact history, no incontinence and no any comorbidities. He reported no history of alcohol intake or cigarette smoking, no history of exposure to radiations or similar presentation to his family and no features suggestive of metastasis. On examination - he had maximum midline tenderness L3-S1, no gibbous, upper limbs neurologically intact and power 3/5 to both lower limbs, sensation intact. Computed tomography scan and magnetic resonance imaging revealed an ill-defined osteolytic mass spanning L3 to L5, vertebral plana of L4 and involvement of the left paraspinal muscles. The tumor extended to the extradural space and was also abutting on the distal great vessels. Our intervention entailed laminectomy, tumor debulking and posterior instrumental fusion of the spine. Histopathology findings were in keeping with an invasive sclerosing epitheloid fibrosarcoma. He had adjuvant chemotherapy with good outcome. DISCUSSION: This case was unique due to its lumbar column location, abutting the distal vessels and despite its low grade, it illustrates the malignant potential which responded well to adjuvant chemotherapy. CONCLUSION: Invasive SEF is an aggressive tumor that requires early diagnosis. Multi-modal treatment with surgical resection, adjuvant chemotherapy can improve patient survival and quality of life.