A 32-year-old Saudi Arabian male sickler patient presented to the emergency department with a six-hour history of sudden and continuous shortness of breath. The history was obtained from the mother. The patient’s current presentation was associated with agitation and a sudden change in behavior. The patient was shouting and refusing to communicate with doctors. The mother denied any history of orthopnea, cough or fever during the patient’s current presentation. Past medical history was remarkable for SCD since childhood with infrequent hospital visits. Socially, the patient was single, non-smoker and living with his family.\nOn general examination, the patient looked ill, anxious and sitting on the bed lying forward connected to an oxygen mask. The body mass index (BMI) was 18.5 kg/m2. Vital signs showed blood pressure: 125/80 mmHg, pulse: 110 beats/min, respiratory rate: 28 breaths/min, temperature: 39 degrees Celsius and oxygen saturation: 87% on room air and 94% on 5 liters of oxygen. Respiratory and cardiovascular examinations were normal. Hemoglobin electrophoresis showed a hemoglobin S (Hb S) level of 78%. Laboratory testing for D-dimer showed 12 ng/L (normal range is < 0.5 ng/L). Pulmonary embolism was suspected and chest spiral computed tomography (CT) scan showed bilateral consolidation without acute pulmonary embolism. In consideration of the fever and agitation, the patient received ceftriaxone, vancomycin and diazepam.\nCT scan of the head without contrast showed left frontal subcortical deep white matter hypodensity, most likely representing chronic watershed infarction. Moreover, there was left frontal cortical gray matter hypodensity, most likely representing cerebral atrophy (Figure ). There were no signs of acute hemorrhage or ischemic infarction. Magnetic resonance angiography (MRA) showed severely hypoplastic left ICA and abnormal tortuous collateral vessels around the circle of Willis (Figure ). No arteriovenous malformation or aneurysm was noted.\nBased on the clinical and radiological findings, the final differential diagnosis was suggestive of a progressive vascular occlusive process which could be seen in MMD. Hematology team was consulted for further evaluation. The patient received a blood transfusion three times daily for 16 days. The patient dramatically improved and returned to his regular behavior. The patient was discharged home on aspirin 81 mg daily. The patient was seen four weeks later at the neurology clinic and he was doing well.
A 12-year-old male patient reported to the Department of Pedodontics and Preventive Dentistry with a chief complaint of broken tooth in the upper front tooth region due to trauma, 1 week back. The patient gave a history of prediagnosed case of MMD with hemiplegia of the right side.\nAt the age of 1½ years, the patient suffered from pulmonary tuberculosis and completed anti-tuberculosis treatment for the same. At the age of 4 years, the patient experienced sudden onset of pain and weakness in the right lower limb, which was associated with difficulty in holding objects with the right hand and raising the right arm above the head. The MRI of brain showed focal right frontal infarct with left cerebral atrophy and an attenuated appearance of intracanal arteries suggestive of effects of MMD. Symptomatic medical treatment was provided to the patient. The patient had no past dental treatment exposure. There is no related family history.\nThe patient appeared underweight with a lean body type. The general physical examination revealed hemiplegia of the right side, affecting both upper and lower limbs ().\nThe extraoral examination revealed a symmetrical face with competent lips and normal mouth opening. On intraoral examination, permanent dentition was present with Angle's Class I malocclusion. Ellis Class I fracture was present in the upper right central incisor whereas Ellis Class II fracture was present in the upper left central incisor ().\nThe dental treatment plan focused on providing preventive as well as restorative care to the patient while simultaneously providing proactive counseling to both the parents and the patient. Behavior management was an integral part of our management. Oral hygiene measures were instructed to the patient and modified angled toothbrush with fluoridated toothpaste was prescribed due to the lack of manual dexterity. Along with this, diet modification was done by recommending intake of noncarious dietary food items such as fibrous food, nuts, and fruits. The medical consent was obtained from the pediatrician following which oral prophylaxis and composite restoration of upper central incisors were done ().