Dens evaginatus (DE) is a developmental anomaly. It is an extra cusp or tubercle that protrudes from the occlusal surface of posterior teeth, as well as the lingual surface of anterior teeth. Tubercles are susceptible to pulp exposure from wear or fracture because of malocclusion;leading to pulpal complications early after eruption.DE may also complicate the process of daily routine oral health care. A 13-year-old girl presented to our polyclinic with sinus track at the posterior lower left buccal gingiva forthe past1 year. All 35 teeth were sound. At the lingual side, there was an accessory tooth in close relation to tooth 35 covered with supra-gingival inflammation. Electric pulp test (EPT) showed that tooth 35 was already non-vital. Intra-oral periapical (IOPA) radiograph and cone beam computed tomography (CBCT) scan showed an abnormal-appearing root which shows DE with radiolucency at the periapical area. Root canal treatment was performed using crown down pressureless (CDP) technique and obturation was done using lateral condensation technique. The tooth was restored using composite restoration. DE is a rare condition. It is quite a challenge for dental practitioner since the diagnosis is difficult and the treatment options are limited. In the case of DE with necrotic pulp and periapical abscess, root canal treatment is a treatment of choice. We have to accept and appreciate any gift from Allah SWT whether it is good or bad, no equal divine creation except from Allah SWT.Therefore we need to take care of our oral hygiene to prevent diseases. In every disease, there is a cure; we thus need to try to do the best to find the cure and to not easily get rid of the tooth and replace it with a denture. Early diagnosis of DE can lead to proper treatment which can result in high success rate if it is followed by adequate restoration.
Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids is a rare inflammatory Central Nervous System disorder prominently affecting the brainstem. We present an adolescent’s reflection on this condition complicated with Epstein-Barr virus induced CNS Lymphoma. Case report: A 16-year-old boy presented about 5 years ago with a balance problem. He was diagnosed by MRI after ongoing debate whether this is juvenile multiple sclerosis. He initially responded to methylprednisolone but developed acute deterioration requiring 8 cycles of Infliximab and Methylprednisolone. He then confirmed cerebellar lymphoma 2 years later hence commenced on chemotherapy and radiotherapy after posterior fossa decompression. He gradually losses his motor skills, left hemiparesis and spasticity. He needs tracheostomy and gastrostomy due to poor bulbar function. Now, he is fully dependent and requires chest physiotherapy and suctioning and cystostomy for urinary incontinence. He has multiple PICU admissions due to recurrent aspiration, acute cerebellar oedema and post posterior fossa decompression. He worries about family breakdown as mum is his sole carer and having regular nightmare. He changed school to meet his care demands and unsure how to adapt to new environment. Social experience makes him anxious due to lack of understanding about his condition. He is evidently having low self-esteem and confidence. Discussion: Children palliative care team has been involved since diagnosis to support him and family. He is understandably depressed and clinical psychologist input bear minimal impact. He is fully aware of the current situation and his wish to rap Eminem’s songs like he used to do it before. Conclusion: Early aggressive treatment in CLIPPERS aim to prevent neuroaxonal loss. However, due to bellicose nature of the condition, the prognosis is relatively poor. Managing adolescent expectation after gradual deterioration is challenging especially, he is aware that achieving ‘normality’ is impossible as the treatment advancement still in limbo.
Acute onset quadriparesis can be a manifestation of a variety of neurological, metabolic or autoimmune conditions. Rarely, it could be one of many clinical presentations of juvenile dermatomyositis which belongs to the group of idiopathic inflammatory myopathies of childhood. We report the case of a 9-year old girl who presented with global myopathy over a two-month period. Case report: A 9-year-old girl referred with a 2 months history of generalised muscular pain and weakness. There were no significant history of preceded illness, trauma or excessive strenuous exercises. She had no other systemic complaints such as fever or skin rash. Past medical history was unremarkable except for recurrent tonsillitis. Physical examination revealed a child with normal alertness and behaviour. She has notable generalised swelling of all four limbs. Her neurological examination revealed normal tone however her power was definitely reduced in all four limbs (Graded 3/5). She demonstrated signs of proximal myopathy. Subsequent investigations revealed high Creatinine Kinase (CK) levels of 6470U/L, ESR 84mm/hr with deranged transaminases and positive serum mycoplasma and CMV IgM. Her MRI brain and spine along with lumbar puncture results were normal. She was referred to tertiary centre for further evaluation as her weakness progressed. An MRI muscle demonstrated marked inflammation in all four limbs including paravertebral muscles. Her muscle biopsy showed inflammatory myopathy leading to a diagnosis of juvenile dermatomyositis (JDM). She is now showing sustained clinical improvements following a course of immunoglobulin and corticosteroids therapy. Discussion: The diagnosis of JDM is generally considered in patients with rash-associated muscle weakness. Essentially, it involves assessment of muscle, skin, lung and cardiac involvement on top of baseline list of investigations that has been outline by Single Hub and Access point for pediatric Rheumatology in Europe (SHARE). This case reflects that JDM is possible despite non-existent of skin involvement.
Spinal tuberculosis in children is an established preventable disease in developing countries. Complications are devastating due to its aptitude to cause bone destruction, spinal deformity and paraplegia. Case report: We present an eight-month old girl with isolated gross motor regression and evolving spastic paraplegia. It highlights the challenge we encountered due to delay in garnering the pertinent investigation. She presented to clinic with history of legs weakness and loss of rolling after a period of prolonged febrile illness. Both parents are medical practitioners. Mother had history of SVT during pregnancy in spite on anti-arrhythmic treatments. Her father is a thalassaemia carrier. A thorough examination revealed gross motor delay and upper motor neuron signs. She had raised inflammatory markers, anaemia and thrombocytosis with persistent low-grade temperature. CT brain with contrast showed meningeal enhancement. Full septic work up revealed the CSF result reflecting partially treated meningitis. She was treated with third generation cephalosporin and acyclovir. Mother claimed exposure to TB patients hence Mantoux test was recommended which came positive. Her chest x-ray, sputum culture, CSF culture and NAA studies came non-conclusive for pulmonary tuberculosis. MRI for brain and spine showed features of tuberculous spondylodiscitis of T4-T5 vertebrae with associated subligamentous paravertebral spread and epidural extension causing spinal cord compression and T3-T6 hydro-syringomyelia. After multidisciplinary team discussion, patient started on intensive antituberculosis regimen with good initial response. Discussion: Clinically lower limbs power improved with good antigravity movement. Laboratory and radiological investigations have improved inflammatory markers and dropping trend thrombocytosis, and spinal gibbous stay stationary with improvement in plain radiology. She is under regular follow up awaiting serial MRI. Conclusion: The challenge in diagnosis of extra pulmonary tuberculosis in infants is getting them excluded early. High index of suspicion along with radiological investigation is vital to aid the diagnosis and establishment of treatment to expect a good outcome.
In Malaysia, a course of vaccination DTaP/IPV/Hib was introduced in 2008, replacing the 2006 DwPT-HBV/Hib+OPV vaccines. Severe systemic adverse reactions after diphtheria, tetanus and pertussis vaccination are uncommon. Cardiac complications are rarely reported and is most probably implicated to the pertussis component. We describe a rare case of acute myocarditis that developed 60 hours after DTaP/IPV/Hib vaccination. Case report: A 2-month old infant presented to emergency department after her first diphtheria, tetanus and pertussis vaccination due to severe respiratory distress and cyanosis. She had her BCG and two Hepatitis B vaccinations previously with no major side effects. Parents reported that she was feverish for 48 hours post vaccination with no other associated symptoms. Prior to presentation, she went floppy and was immediately brought to hospital. On arrival, she was tachypnoeic and cyanotic with hypoperfusion and hypotensive. She was also noted to have hepatomegaly. She was grunting and her level of consciousness deteriorated. She was immediately intubated and her first blood gas showed profound metabolic acidosis with pH 6.6, base excess -24mmol/L, lactate 14mmol/L and bicarbonate 4mmol/L. She required fluid boluses and inotrope infusion was commenced. She received antibiotics and sodium bicarbonate to correct her acidosis. Her echocardiography showed global hypokinesia, CK 3018 and positive Troponin. She was treated with immunoglobulin for myocarditis and on high frequency oscillation for 4 days before being extubated on day 11 of admission. All her viral serology and cultures came back negative. Discussion: Cardiac complications after diphtheria, tetanus and pertussis and other vaccinations are exceptionally uncommon. This patient developed sudden onset cardiogenic deterioration after an expected fever-like illness post vaccination. Given her viral screening and cultures were negative, this make acute myocarditis post vaccination a remote possibility. We concur that evaluation of cardiac state should be considered in recently vaccinated infants who manifest with cyanosis, hypoperfusion and drowsiness.
APDE is an acquired, transient bleeding disorder characterised by normal platelet counts with eosinophilia. It was previously known as ‘nonthrombocytopenic purpura with eosinophilia’. We report a case of a 3-year-old boy with prolonged history of spontaneous unexplained bruising which was initially investigated by SCAN team for non-accidental injury (NAI). Case report: A 3-yearold boy attended clinic with a 4-month history of recurrent bruising. Parents were unsure of preceded illness, but he remained well with no history of trauma. He has unremarkable medical history and father is a thalassaemia carrier. Upon assessment in clinic, he was subsequently referred for suspected NAI and SCAN team led to a police report with a plan to review in 2-months. Parents later decided to bring the child for further medical assessments which revealed multiple bruises over both thighs, back of shoulder, loin and trunk with varying sizes and ages. They were all non-tender on palpation with no recognisable shapes or patterns. Blood results showed normal liver functions with slightly prolonged APTT 39.8 secs. He has normal platelet count with significant eosinophilia 2.2 x 10^9/L and occasional reactive lymphocytes. Reflecting this result, plan for platelet functions and von Willebrand tests were made. However, due to costs, we decided to treat the child with antihelminthic agent for possible parasitic infestation. He had 3 days course of albendazole and no further bruises appeared after 5 days of completing treatment. Discussion: The clinical presentation of APDE can mimic Idiopathic Thrombocytopenic Purpura in many ways yet normal platelet counts often leads to a delay in diagnosis. Reassuringly, the course of APDE is benign and no treatment is often required. Conclusion: Investigations however are costly, therefore treatment with anti-helminthic agent would be an alternative option in providing assurance to family and medical practitioners dealing with suspected cases.