Editor-in-chief, IAP Drug Formulary and ASSC Consultant, Dept of Child and Adolescent Medicine, Aster Medcity, Kochi

Dr Jeeson C Unni

There are a plenty of good apps which a paediatrician needs to have on their mobile.

Over the past 10 years, our smartphones and tablets have transformed the way in which we practice medicine. It has been some time since I reviewed medical applications for mobile devices, and many paediatricians have requested an update. I have therefore put together my recommendations for the best applications for paediatric providers - apps that I have been using for years (applications that are associated with “connected” devices, such as pulse oximeters, blood pressure cuffs, otoscopes and so on, are not detailed in this article). 
I would classify the various mobile apps as follows:
Almost everything in one app  
Medscape
IAP Drug formulary
Pediatrics On Call
Calculators and convertors
Ped(z) – Pediatric calculator
IAP
Indian Pediatrics app 
Immunisation app
IAP Growth Charts app
E-vote IAP
 Differential Diagnosis
Isabel 
Pediatrics On Call
Medline search
PubMed
Other useful apps
Diagnostic tool for Autism Spectrum Disorders 
PedNeuroAiims Diagnostics  
Bone Age Anthropometric Calculator
Pedi Crisis 2.0
Totsguide
Track And Act
ADR PvPI
GCS app
Surakshith
Bal Suraksha
Journals
Read by QxMD
Podcasts
Podcast Republic

There are a plenty of good apps which a paediatrician needs to have on their mobile.
Medscape from WebMD is one of the leading medical resources used by physicians, medical students, nurses, and other healthcare professionals for clinical information. Since 2009, Medscape has released an annual update of its Medscape Mobile app. The mobile app is used by over 3 million health care professionals, and was the #1 most downloaded free app in the medical category of iTunes for 2010.
The app, as with previous versions, offers evidence-based disease reference and updated medical news and education resources. An independent team of 7700 physicians and pharmacists author and review the content, so current information is ensured. Medscape has recently released its 4.0 version, that includes several new features. 
The IAP Drug Formulary (IAP DF), first published in 2004-2005, has completed 13 yrs of service to the paediatric specialists in India and abroad, being the only exclusively paediatric formulary in the world other than the British National Formulary for Children (BNFc). The IAP DF 2019 now contains recommendations for drug therapy for >500 paediatric ailments and detailed, well researched information for more than 630 medications licensed for use in neonates, children and adolescents.
The user interface of the IAP DF mobile apps has been improved to make it more user friendly. It is available for the iOS (iPhone, iPad), Android, Symbian and Blackberry platforms. The quarterly Web Updates get automatically updated on the mobile app.  
The Pediatric Oncall apps contains calculators (growth calculators, renal calculators, pregnancy calculator, blood group calculator, drug dose calculator, critical care calculators, diarrhoea calculator and biochemical test calculators), drug dosing, MCQs, video podcasts, poison information, an image gallery and more. One can take part in Grand rounds (Teaching Files), get a differential diagnosis in the Diagnostic Aid section, ask an expert panel of doctors a medical query in the Post Query section, or watch videos on the latest topics.

Ped(z) – Pediatric calculator as an app for Android and iPhone/iPad is available for free. The features of the app are the same as on the website (except for the plotter, which is missing in the app). Calculates percentiles, z-scores and many more things a paediatrician needs.
IAP - Indian Pediatrics mobile app -  the official publication of the Indian Academy of Paediatrics (IAP) – the app allows the user to browse the contents of the current issue of IP, search articles by author or keyword, and search contents of past issues up to 2002.
IAP Immunization app of the IAP Advisory Committee on Vaccines & Immunization Practices (ACVIP) – contains recommended vaccines. An immunization schedule and vaccine reminders are also available on this app. 
IAP Growth Charts app - This smart app can plot entered data efficiently on reference graphs and analyse the data, to give an instant diagnostic interpretation. This app aims to give a completely new outlook to growth monitoring practice in India by digitalizing growth charts.

Isabel app is a differential diagnosis generator designed to ensure no diagnosis is overlooked. It is a web-based program designed for clinicians making a diagnosis, as well as medical students and residents honing their skills. It can assist in thinking through all possibilities in any case with diagnostic uncertainty.
The differential diagnosis process begins by entering patient data (age, gender, travel history) into the “clinical features” box on the homepage. Next, the user enters any abnormal clinical features, including the chief complaint, labs, vitals and comorbidities. Clicking the “Get Checklist” button generates a ranked diagnoses list organized by a color bar or “Likelihood Indicator”: dark orange designating “most likely”, through to light yellow representing “less likely.” The list can be sorted by specialty or by red flags (conditions requiring immediate attention). Another tab lists drugs whose side effects may cause the symptoms.
Clicking on a possible diagnosis brings up a separate window – the “knowledge window” – containing evidence-based information on that particular condition. 
Isabel Pro, which boasts a 96 percent accuracy rate, applies artificial intelligence pattern recognition software onto a database of more than 10,000 diagnosis presentations for all ages and specialties.

PubMed app - The free “PubMed Mobile” option provides access to a mobile friendly, simplified PubMed version which connects to the most up-to-date journal citations and abstracts from the complete PubMed database.

the Child Neurology Division of the Department of Pediatrics, All India Institute of Medical Sciences, New Delhi.  The app can diagnose autism in children between the ages of 1 and 14 yrs, with the help of questions such as whether the child talks about his/ her achievements and emotions without being asked, whether they like to play alone or in a group, or whether they make eye contact whilst talking.
PedNeuroAiims Diagnostics app - Diagnostic Tools for Epilepsy, Autism, Attention-deficit/hyperactivity disorder (ADHD) and Neuromotor impairment (NMI). The free PedNeuroAiims Diagnostics app guides doctors through a set of questions and observations to diagnose whether a child has one of the above conditions. Each child that the paediatrician assesses using this tool can be archived in the app.  
Totsguide - Totsguide is an online self-education portal for parents, doctors and guardians to take complete care of their child’s development. One has to pay for features such as Track and Act and SCoPE. 
Pedi Crisis 2.0 - The Society for Pediatric Anesthesia (SPA) presents Pedi Crisis 2.0! As an interactive version of the SPA critical event checklists, crucial details for these 26 paediatric critical events are now just a ‘tap’ away on your pocket device! Quick reference drug dosage summaries are available for each emergency. Enter the patient’s weight to automatically calculate patient-specific dosages.
Surakshith: App on personal safety for 6-18 years olds. Children learn Body Safety Rules, Safe - Unsafe Touch, how to identify Safe Adults. Stories help children learn No - Go - Tell mantra & understand that the perpetrators of abusive actions are solely responsible for them. Blame & shame are discussed. The Internet Safety & Digital Citizenship features are for older children.
Suraksha App by Bengaluru City Police SOS is a fully integrated personal safety app with Policing. The App turns your smartphone into a discreet personal safety device for use during an emergency. A call to the police service can be triggered by simply activating the SOS button, featured as an icon on your cell phone.
ADR PvPI - PvPI adverse drug reaction reporting android application. Using PvPI adverse reporting android application, healthcare professionals or consumers can instantly report any suspected Adverse Drug Reaction to PvPI from any part of India.
The Pharmacovigilance Program of India (PvPI) was launched with a broad objective to safeguard the health of the 1.27 billion people of India. Adverse drug Reactions (ADRs) are reported from all over the country to PvPI, which also work in collaboration with the global ADR monitoring centre (WHO-UMC), Sweden, to contribute in the global ADR data base. PvPI monitors ADRs among the Indian population and helps the regulatory authority of India (CDSCO) in taking decisions for the safe use of medicines. PvPI invites all health care professionals and patients/consumers to join in their mission to promote patient safety.
ABG Calculator: Blood Gas App - With an intuitive design and easy-to-read display, ABG Calculator makes it a breeze to answer even the most complex blood gas problems in seconds.
Features:
- Fast, accurate input using the dial
- Expected Lab values
- Easy Reset
This ABG app has been “stress tested” against 100+ triple acid-base ABG problems.
Bone age assessment app - This application allows one to evaluate, by visual comparison of a radiograph, what is the age of a paediatric patient, based on the maturity status of his/her bones.
The Glasgow Coma Scale Android App is an must-have app. An intuitive interface leads a great list of features. The Glasgow Coma Scale Android app is routinely used in the ER and during ICU rotations, for assessing brain injury. The scale checks for a patient’s motor response, verbal response, and eye opening, and objectively creates a score. This app includes options to select grade or severity of symptoms based on eye, verbal, or motor response. The app has an intuitive user interface with instructions in a Q&A format. The app is free and performs quick calculations. It lacks medical information correlating GCS scores with disease conditions, but is perfect for ER and neurology rotations.

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Podcasts are ready for download multimedia audio or video files. The audio files usually take the MP3 format. 
The term ‘podcasts’ is an amalgam of ‘iPod’ and ‘broadcasts’. These files or collection of files are accessed via syndication feeds through RSS readers, and so, unlike broadcasts, they are not necessarily streamed at a certain time but rather can be subscribed to and accessed at any time using a device. NEJM, for example, offers some headlines and news in the form of podcasts and RSS feeds

Medical Apps relevant to Paediatricians

#111827

Specialist, medical microbiology, Aster Medinova Diagnostic Centre, Burdubai

Naseel Salim

Specialist, Ear Nose and Throat, Aster Hospital, Mankhool

Amit Goel

Microbiology technologist, Aster Medinova Diagnostic Centre, Burdubai

Smrithi Sajeesh

HalaFarrag Hassan

Scedosporium apiospermum is a ubiquitous fungus. The clinical importance of this fungus is mostly seen with immunocompromised hosts and rarely in immunocompetent patients. It can present with a broad clinical spectrum. Hereby, we report a case of Scedosporium apiospermum infection-causing rhinosinusitis, a rare clinical entity in an immunocompetent female.

A 63 yr old female presented with a complaint of left side facial pain (maxillary region) which had been present for 1yr. There was no history of nasal blockage, nasal discharge, fever or headache. The patient was also given an opinion from a dentist but experienced no improvement. On examination, the nasal cavity was found to be clear, with no significant nasal secretions or discharge. There was significant maxillary sinus tenderness on the left side on palpation.
An X-Ray-PNS showed complete haziness in the left maxillary sinus. A CT-PNS scan (plain) showed that the nasal septum was deviated towards the right side, with a bony spur towards the right side. Bilateral inferior turbinate hypertrophy was evident. Mucosal thickening was seen in the left maxillary sinus, with occlusion of the ostium, and sinus wall thickening and sclerosis suggestive of chronic left maxillary sinusitis. Possible erosion of the floor of the maxillary sinus was seen at the site of an absent molar tooth. Mucosal thickening was seen in the right sphenoid sinus. The patient underwent left side functional endoscopic sinus surgery (FESS) only. The left maxillary sinus was opened and found to be full of a fungal element, which was sent for fungal culture. The sinus was cleaned completely.
On biopsy, direct KOH mounts revealed the presence of hyaline thin septate hyphae. The fungal culture from the sinus tissue biopsy was identified as Scedosporium apiospermum. The patient is kept on regular follow-up and continues with a daily alkaline nasal douche. As per follow-up visits, the left maxillary sinus is healing well. 

https://firebasestorage.googleapis.com/v0/b/article-app-d985d.appspot.com/o/blogmedia%2F1732269289913fig%201.jpg?alt=media&token=bcf5e279-ee0f-4059-833e-9f08f7a80ff9

Allergic fungal rhinosinusitis (AFRS) is a distinct type of chronic rhinosinusitis (CRS), accounting for between 5 and 10 percent of all CRS cases. AFRS is believed to result from chronic, intense allergic inflammation directed against colonizing fungi. Patients with AFRS are immunocompetent and show evidence of allergy to one or more fungi. Definitive diagnosis is usually confirmed after sinus surgery.
The diagnostic criteria for AFRS vary among authors, but most widely accepted are the 5 criteria described by Bent and Kuhn6: (1) type 1 hypersensitivity to fungi; (2) nasal polyposis; (3) characteristic radiographic finding of serpiginous areas of high attenuation; (4) eosinophilic mucin; and (5) a positive fungal stain. 
Scedosporium spp. are found in soil and polluted or stagnant waters. Although these organisms are commonly considered agents of phaeohyphomycosis, the hyphae are hyaline. Infection occurs most often in the upper and lower extremities, but may occur on any exposed parts of the body. 
Scedosporium spp. can infect subcutaneous tissues, muscle, tendon and bones, as well as lungs, sinuses, eyes, the central nervous system and other body sites. Scedosporium spp. are the most common cause of fatal lung and central nervous system infections complicating near-drowning accidents in immunocompetent victims. Disseminated infection is most commonly seen in immunocompromised patients. Invasive Scedosporium lung disease in immunocompromised patients may clinically resemble invasive pulmonary aspergillosis. Patients with cystic fibrosis have a high frequency of colonization by Scedosporium spp. or chronic infection in the form of fungus balls in the lower respiratory tract, but seldom with progression to locally invasive disease or dissemination.  
Diagnosis of infections caused by Scedosporium spp. is made using radiological and microbiological tests. In microscopy, the appearance of this fungus is similar to common hyaline hyphomycetes, therefore the final diagnosis of scedoporiosis is based on isolation of the fungus in culture7. In the present case, the final diagnosis of this infection was made on the basis of tissue biopsy culture isolation of Scedosporium apiospermum.
Accurate and prompt diagnosis of S. apiospermum is essential, as this organism can be often misidentified as other moulds such as Aspergillus spp. and Fusarium spp. These three genera are angioinvasive, and have non-pigmented, slender, septate hyphae 2–4 µm in width. Scedosporium spp. can be differentiated by their more irregular branching pattern, compared to the orderly dichotomous 45° angle branching observed in Aspergillus, and also by their production of ovoid conidia with truncated bases, which can be confused with yeast. 
Identification of the fungus by culture is crucial because of the variable susceptibility of S. apiospermum to amphotericin B and other antifungal agents. Scedosporium spp. are inherently resistant to amphotericin B and frequently, though unpredictably, resistant to azoles8. The broad-spectrum azole voriconazole is the drug of choice for S. apiospermum infections. 
Prompt clinical suspicion in patients of chronic sinusitis with suspicious signs and symptoms and timely sampling of the adequate patient specimens and processing of samples by microscopy and culture examination will help early diagnosis and management of these patients. Successful treatment involves a combination of surgical and medical management.

Machado CM, Martins MA, Heins-Vaccari EM, Lacaz Cda S, Macedo MC, Castelli JB, et al. Scedosporium apiospermum sinusitis after bone marrow transplantation: Report of a case. Rev Inst Med Trop Sao Paulo. 1998;40:321–3.

Eckburg PB, Zolopa AR, Montoya JG. Invasive fungal sinusitis due to Scedosporium apiospermum in a patient with AIDS. Clin Infect Dis. 1999;29:212–3.

Chanqueo L, Gutiérrez C, Tapia C, Silva V, Razeto L, Misad C, et al. Scedosporium apiospermum rhinosinusal infection in an immunocompetent host. Rev Chilena Infectol. 2009;26:453.

Giri S, Kindo AJ, Rao S, Kumar AR. Unusual causes of fungal rhinosinusitis: A study from a tertiary care centre in South India. Indian J Med Microbiol. 2013;31:379–84.

Rynga D, Capoor MR, Varshney S, Naik M, Gupta V. Scedosporium apiospermum an emerging pathogen in India: Case series and review of literature. Indian J Pathol Microbiol. 2017;60:550–5.

Bent  J, Kuhn  FA. Diagnosis of allergic fungal sinusitis.  Otolaryngol Head Neck Surg 1994;111580-111588. 

Larone DH, Hayden RT, Walsh T.  Larone’s Medically Important Fungi: A Guide to Identification. Washington, American Society for Microbiology; 2018.

  Lackner M, de Hoog GS, Verweij PE, Najafzadeh MJ, Curfs-Breuker I, Klaassen CH. Species-specific antifungal susceptibility patterns of Scedosporium and Pseudallescheria species. Antimicrob. Agents Chemother. 2012;56(5):2635–2642.

Scedosporium apiospermum as a rare cause of Allergic Fungal Rhinosinusitis (AFRS)

MS-ORTHO, Department of Orthopedic Surgery, Aster MIMS Hospital, Kottakkal, Kerala, India.

Dr. Muhammad Hassan

Dr. Faizal M Ikbal

D-ORTHO, Department of Orthopedic Surgery, Aster MIMS Hospital, Kottakkal, Kerala, India.

Dr. Abdul Hakkeem

, Dr. Janees MC

Dr. Muhammad Mohsin Edavath

MS-ORTHO,Department of Orthopedic Surgery, Aster MIMS Hospital, Kottakkal, Kerala, India.

Dr. Arif PK

Shaft humerus, Fracture, Plate fixation, Intramedullary nail, Operative management


Background: 
Materials & methods: This prospective observational study consisted of 32 participants, who were recruited to the study groups sequentially until the desired sample size was reached in each group. Participants were allocated to each of the surgical procedures based on their personal preferences. Patients were evaluated both clinically and radiologically at the end of 1 month, 3 months, 6 months and 1 year from the day of surgery. The study group (plating vs nail) was considered as an explanatory variable. Time to union, and functional outcome, as assessed by American shoulder and elbow society score, were considered primary outcome variables of interest. The need for bone grafting was considered as a secondary outcome variable.

Background

Both study groups were comparable with respect to all baseline variables. The proportions of subjects showing signs of radiological union were 100% and 92.86% in the plating and nailing groups respectively. The proportion of subjects with radial nerve involvement was slightly higher in the plating group compared to the nailing group (12.5% vs 7.14%), but the difference was not statistically significant (P = 0.626). There was no statistically significant difference in the proportion of subjects needing bone grafting (18.75% in plating vs 14.29% in nailing group, P = 0.743). The mean time to union was slightly longer in the plating group compared to the nailing group (15.44 ± 2.39 vs 13.93 ± 1.85 weeks, P = 0.067), which was not statistically significant. Functional outcomes at the end of the follow up period, assessed by mean ASES score (26.44 ± 2.31 in plating vs 26.64 ± 1.65 in nailing group), was comparable between the two intervention groups.


Results

Radiological and functional outcomes following humeral shaft fracture treatment with plate osteosynthesis and intramedullary nailing were comparable. 


Conclusions: 

Fractures of the humeral shaft are common orthopaedic injuries, which result in a significant burden to society from lost productivity and wages.1 Fractures of the humerus comprise approximately 5 to 8% of all extremity fractures.2 Non-union rates for diaphyseal humerus fractures treated non-operatively range from 0 to 13%, with the incidence increasing to 15 to 30% for operatively treated fractures.2, 3 There is a bimodal distribution of humeral shaft fractures by age, with peaks primarily in young male patients aged 21–30 years, and a larger peak in older females from 60–80 years of age.4 


Absolute indications for surgical intervention for humeral shaft fractures are specific circumstances including open fractures, associated neurovascular injury, proximal and distal articular extension, patients with multiple injuries or polytrauma, floating elbow, progressive radial nerve deficits, significant soft tissue injury (unable to brace), pathologic fractures and failed non-operative management.5 Other relative indications include obese patients, patients with associated brachial plexus injuries due to the loss of muscle co-contraction and its ability to maintain bony alignment, and non-compliant patients.6 

For patients with surgical indications, two different models are available: compression plate and intramedullary nailing (with open and closed approaches). Each has its advantages and disadvantages.7,8  With plate and screw fixation, we may achieve more rigid fixation; however, in intramedullary nailing, fracture site soft tissue manipulation is possible. With plate and screw fixation, we may achieve more rigid fixation; however, in intramedullary nailing, fracture site soft tissue manipulation is much reduced.7,8 Plating with rigid fixation, which is known to provide accurate anatomic reduction, can reduce the risk of mal-union, but requires wide intra-operative exposure, associated with soft-tissue stripping. Furthermore, the over-stripping of soft tissue at the fracture site also lowers the blood supply, which might raise the risk of non-union or infection.9


Closed nailing preserves the periosteal blood supply and promotes fracture union, by utilizing the osteogenic potential of the pluripotent cells in the fracture haematoma. Thus, closed intramedullary nailing supports the concept of biological fixation. Moreover, closed nailing procedures are associated with fewer complications, such as reductions in blood loss, infection rate and hospital stay. This treatment method has been the subject of controversy since its inception, because of concerns of damage to medullary circulation, possibilities of fat embolism, complications arising from application of incorrect technique, and lack of understanding of the biomechanical principles of intramedullary interlocking nail fixation (IINF). 10 Clinical series of fractures stabilized with humeral nails often report shoulder problems related to the insertion site, possible technical difficulties, more radiation exposure intra-operatively, and a higher rate of revision surgery.11


Choosing to plate or nail a humeral shaft fracture is becoming more a matter of patient preference or surgeon’s choice, based on potential complications and surgeon familiarity. A meta-analysis that previously favoured plating over nailing was recently updated, and noted equivalent outcomes in rates of non-union, infection, nerve palsy, reoperation, and total complications between humeral plating and nailing.9-12 However, few studies have definitively stated that IM nailing was a better surgical option for the management of humeral shaft fracture.13 Hence, this study compared the functional outcomes of humeral shaft fractures treated by two procedures - closed intra-medullary interlocking nailing and open plate osteosynthesis. 



The study was a prospective observational study conducted in the Department of Orthopedic surgery, Aster Mims Hospital-Kottakkal, between July 2019 and July 2020. The study population consisted of patients with fractures of the shaft of humerus (Middle and Proximal 3rd), aged 18 years or above.  The participants with open fractures, fracture distal third of humerus, pathological fractures polytrauma, fractures associated with neuro vascular injury, established non-union from previous fracture, and patients with multiple fractures, were excluded from the study. 


The participants were divided into two groups: The intramedullary nailing group or the 2-plate osteosynthesis group. The ethics committee of the hospital approved the study, and each subject gave written informed consent. 


Sample Size: Sample size was calculated assuming the expected mean ASES score (µ1 – µ0) in each of the treatment groups as 23.9 and 21.7, as per the study by Hashmi PM et al.14 The other parameter considered for sample size (N) calculations was a common standard deviation (σ1, σ0)  of 2, giving 5% alpha error and 80% power of study. Here, the constant value of u was 0.84 for 80% power and the value of v was 1.96 for a significance level of 5% .The sample size was calculated using the following formula.

The sample size as per the above mentioned calculation was 14 subjects in each group. To account for loss to follow up, it was decided to include an additional 10% of subjects. Hence, the final sample size decoded was 16 subjects in each group. 


The study participants were recruited to each study groups sequentially until the sample size was reached in each group. Participants were allocated to either of the surgical procedures, as per patient preference. The relative advantages and disadvantages of each of the two treatment methods were explained to the participants, based on the best available evidence. The cost of treatment was also explained. Based on their choice, they were allocated to either of the two groups. 


Patients were evaluated both clinically and radiologically at the end of 1 month, 3 months, 6 months and 1 year from the day of surgery. Clinical and radiological evaluation used the following measures:


Subjective pain or tenderness at the fracture site (scored with visual analog scale) 
Functional activities at the shoulder and elbow joint. 
Documentation of radial nerve injury and Tinel's sign 
Presence of callus and bony trabeculae bridging the fracture site in follow up radiograph. 
American shoulder and elbow society score.


Functional outcome (age, ASES score, shoulder abduction ROM, elbow flexion ROM, time to union) were considered as primary outcome variables. Study group (plating vs nailing) was considered as an explanatory variable.


All quantitative variables (age, ASES score, shoulder abduction ROM, elbow flexion ROM, time to union) were checked for normal distribution within each category of explanatory variable, using visual inspection of histograms and normality Q-Q plots. A Shapiro- Wilk test was also conducted to assess normal distribution.  A Shapiro- Wilk test p value of >0.05 was considered as normal distribution.


For normally distributed quantitative parameters, the mean values were compared between study groups using an independent sample t-test (2 groups). Categorical outcomes were compared between study groups using a Chi square test /Fisher's Exact test. (If the overall sample size was < 20 or if the expected number in any one of the cells is < 5, Fisher's exact test was used.) 
P values of <0.05 were considered statistically significant. IBM SPSS version 22 was used for statistical analysis.15 


Humeral shaft fracture is unique amongst the long bone fractures in its tolerance of below-anatomical reduction. Shortening up to 3 cm, rotation of up to 300 and angulation of up to 200 are all considered acceptable. Due to this fact, most humerus fractures are still managed conservatively and have good functional results. The most common indication of operative intervention is an inability to achieve acceptable reduction, followed by associated vascular lesions, open fractures, radial nerve palsy, polytrauma patients, floating elbow and pathological fractures.16


  Intramedullary nailing in humerus fractures is a less invasive procedure, which maintains the biology and gives a good, stable fixation. It is also assumed to result in quicker union, less blood loss and less chance of radial nerve injury. Even though many studies have reported on IMN fixation and plate fixation,13, 17-21 controversy still exists over the best method of fixation. In this study, we aimed to compare the closed intra medullary interlocking nailing and open plate osteosynthesis in terms of the functional outcomes of Humeral shaft fractures treated by these two procedures.  A total of 30 subjects were included in the analysis. 
Among the study population, plating was conducted 

chosen in 14 subjects (46.67%). The proportions of males were  68.75% among plating subjects, and 71.43% in the nailing group. There was no significant difference in age and gender between the two study groups (P > 0.05); The mean age of subjects in plating group was 35.63 ± 15.43 years and in the nailing group, 36.14 ± 11.8 years. Similar study settings have been reported in a few existing studies.17-20 A study by Fan et al. (2015)19 recruited a total 0f 60 participants with humeral shaft fractures, which were equally distributed to two study groups to receive IMN (N=30) and LCP (N=30), with similar mean ages of 39.3 ± 10.8 years in the IMN group and 39.2 ± 10.3 years in the LCP group. In the IMN group, the proportion of males were 60%, and in the plating group, 63.3%. In A 2014 study by Wali et al.,17 25 patients with humeral shaft fractures were selected for an  ILN group and 25 to receive DCP. Similar to our study, the majority of participants in the two study groups were males (85% and 80%), with mean ages of 37.28 and 37.72 years respectively. In Yin et al. (2013)18, again, no statistically significant difference was observed in age and gender between the two study groups.


In our findings, the proportion of subjects showing signs of radiological union was 100% among plating subjects and, in the nailing group, 92.86%. Union rates in our study were comparable with other studies.13,17,19-20  In the study reported by Fan et al. (2015)19, union rates were reported as 96.7% and 93.3% in the IMN and LCP groups respectively. Wali et al.’s study (2014)17 was consistent with our findings, with 92 % union rates and only 8% non-union cases. The study findings of Putti et al. (2009)20 reported union rates of 100% and 94% in their IMN and DCP groups respectively. Changulan, et al. (2007)13 reported a 85.7% union rate in an IMN group compared to 87.5% in a DCB group.


The mean ASES score was 26.44 ± 2.31 in our plating group and 326.64 ± 1.65 in our nailing group, which was not statistically significant (P = 0.784) in our study population, as has been reported by similar studies.13,17,20 Results were similar in the study by Wali et al. (2014),17 with a mean AECS score of 43.2 in the ILN group and 44.1 in the DCP group, which also was not significant. Mean AECS scores observed were 45.2 and 45.1 across IMN and DCP groups respectively in a study by Putti et al. (2009)20, which was not significantly different. In Changulani et al. (2007)13, mean AECS scores were observed as 44 min and 45 min in IMN and DCP groups respectively.


The proportion of radial nerve involvement among the plating subjects was 12.5% and in the nailing group this proportion was 7.14%. There was no statistical significance in the difference in radial nerve involvement between the two groups (P value=0.626), which is similar to the findings of a few existing studies 13, 17-20. In the study by Fan et al. (2015)19, radial nerve palsy was reported in 3 (10%) patients from the LCP group, but no cases in the IMN group. Similarly, Wali et al. (2014)17 reported radial nerve palsy in two cases from a DCP group and no cases in an IMN group. As per the findings of Yin et al. (2013)18, radial nerve injury was reported in 4 cases from an LCP group, but no cases were found in an IMN group. In the study by Changulani et al. (2007)13, only one case of radial nerve palsy was found in a DCP group and no cases in an IMN group. However, a contrary study by Putti et al. (2009)20 reported two cases of transient radial nerve palsies but none in a DCP group.


In this study, the proportions of subjects needing bone grafting were 18.75% and 14.29% in the plating and nailing groups respectively, showing no significant difference (P = 0.743). Similar proportions have been observed in most prior studies.13, 17, 20 Wali et al. (2014)17  reported bone grafting completed as a secondary procedure in two patients in each study group (ILN and DCP). In the findings of Putti et al. (2009)20, bone grafting was necessary in only one case in the DCP group and none in an IMN group. Changulani et al. (2007)’s study findings 13 show that bone grafting was required in 9.5% of cases in an IMN group, and 4.5% in a DCP group.             


In our study, the mean elbow flexion ROM in plating group was 133.75 ± 7.19, and in the nailing group, this was 135.71 ± 10.89. The mean ROM of shoulder abduction in the plating group was 156.25 ± 16.68, and in the nailing group, was 161.43 ± 8.64. The differences between elbow flexion ROMs and shoulder abduction ROMs were not statistically significant between groups. Similar to our study findings, the meta-analysis by Benegas et al. (2014)22 reported that patients' shoulder movements after DCP treatment were superior to those after IMN fixation (RR  =  9.27 95% CI: 2.22–38.72) Ma et al. (2013)21 study also supported our study findings. There was no significant difference found with regard to shoulder function according to the University of California, Los Angeles, scale between the minimally invasive plate and locking intramedullary nail (31.4 points vs 31.2 points, P ¼ .98). There was also no difference in elbow function (94.8 points vs 94.1 points, P ¼ .96).


In the current study, our reported mean time to union in the plating group was 21.13 ± 3.74.19 days, and in the nailing group, was 19.07 ± 2.79 days (P Value=0.560). However, the study by Changulani et al. (2007)13  reported a mean union time for an IMN group of 6.3 weeks, and 8.9 weeks for a DCP group, a statistically significant difference (P<0.001). The study findings of Wali et al. (2014)17 reported that the mean duration of union in remaining patients in ILN group was 13.60 (SD 4.32) weeks, and in the DCP group was 15.2 (SD 5.65) weeks, which was also statistically significant (P=0.376). Fan et al. (2015)19’s study documented average union time as 6.7 weeks in an IMN group and 10.6 weeks in an LCP group, with a statistically significant difference (P <0.001). In the study by Yin et al. (2013)18, the average bone healing time was (11.77 +/- 0.75) weeks in an LCP group, and (11.38 +/- 0.82) weeks in an IMN group. The difference was not statistically significant. (t = 1.705, P = 0.095).


There was no significant difference in radiological and long-term functional outcomes following humeral shaft fracture treatment with plate osteosynthesis and intramedullary nailing. No delay was observed in time to union.



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1.	Walker M, Palumbo B, Badman B, Brooks J, Van Gelderen J, Mighell M. Humeral shaft fractures: a review. J Shoulder Elbow Surg. 2011;20(5):833-44.


2.	Volgas DA, Stannard JP, Alonso JE. Nonunions of the humerus. Clin Orthop Relat Res. 2004;419: 46-50.


3.	Konda SR, Davidovitch RI, Egol KA. Initial Surgical Treatment of Humeral Shaft Fracture Predicts Difficulty Healing when Humeral Shaft Nonunion Occurs. HSS J. 2016;12(1):13-7.


4.	Tytherleigh-Strong G, Walls N, McQueen MM. The epidemiology of humeral shaft fractures. J Bone Joint Surg Br. 1998;80(2): 249-53.


5.	Denard A, Jr., Richards JE, Obremskey WT, Tucker MC, Floyd M, Herzog GA. Outcome of nonoperative vs operative treatment of humeral shaft fractures: a retrospective study of 213 patients. Orthopedics. 2010;33(8).


6.	Spiguel AR, Steffner RJ. Humeral shaft fractures. Curr Rev Musculoskelet Med. 2012;5(3):177-83.


7.	Kivi MM, Soleymanha M, Haghparast-Ghadim-Limudahi Z. Treatment Outcome of Intramedullary Fixation with a Locked Rigid Nail in Humeral Shaft Fractures. Arch Bone Jt Surg. 2016;4(1):47-51.


8.	Saka G, Saglam N, Kurtulmus T, Ozer C, Ugurlar M, Akpinar F. Interlocking intramedullary ulna nails in isolated ulna diaphyseal fractures: a retrospective study. Acta Orthop 

9.	Wang X, Chen Z, Shao Y, Ma Y, Fu D, Xia Q. A meta-analysis of plate fixation versus intramedullary nailing for humeral shaft fractures. J Orthop Sci. 2013;18(3):388-97.


10.	Murari SP CN, Bhargav K A, Palvai S. . Does Closed Intramedullary Interlocking Nailing in Patients with Humeral Shaft Fractures Has Advantages: Evaluation through a Clinical Study.  Asian J Pharm Clin Res. 2016;10(3):434-8.


11.	Hohmann E, Glatt V, Tetsworth K. Minimally invasive plating versus either open reduction and plate fixation or intramedullary nailing of humeral shaft fractures: a systematic review and meta-analysis of randomized controlled trials. J Shoulder Elbow Surg. 2016;25(10):1634-42.


12.	Heineman DJ, Bhandari M, Nork SE, Ponsen KJ, Poolman RW. Treatment of humeral shaft fractures--meta-analysis reupdated. Acta Orthop. 2010;81(4):517.


13.	Changulani M, Jain UK, Keswani T. Comparison of the use of the humerus intramedullary nail and dynamic compression plate for the management of diaphyseal fractures of the humerus. A randomised controlled study. Int Orthop. 2007;31(3):391-5.


14.	Hashmi PM, Mohib Y, Abbas K. Functional and radiological outcome of Intramedullary nailing vs. plate fixation for humeral shaft fractures. Does treatment method bring any difference? J Pak Med Assoc. 2014;64(12 Suppl 2):S135-8.


15.	Lin J. Treatment of humeral shaft fractures with humeral locked nail and comparison with plate fixation. J Trauma. 1998;44(5):859-64.


16.	Papasoulis E, Drosos GI, Ververidis AN, Verettas DA. Functional bracing of humeral shaft fractures. A review of clinical studies. Injury. 2010;41(7):e21-7.


17.	Wali MG, Baba AN, Latoo IA, Bhat NA, Baba OK, Sharma S. Internal fixation of shaft humerus fractures by dynamic compression plate or interlocking intramedullary nail: a prospective, randomised study. Strategies Trauma Limb Reconstr. 2014;9(3):133-40.


18.	Yin P, Mao Z, Zhang L, Tao S, Zhang Q, Liang X, et al. Effectiveness comparison between locking compression plate fixation and locked intramedullary nail fixation for humeral shaft fracture of types B and C. Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2013;27(12):1457-61.


19.	Fan Y, Li YW, Zhang HB, Liu JF, Han XM, Chang X, et al. Management of Humeral Shaft Fractures With Intramedullary Interlocking Nail Versus Locking Compression Plate. Orthopedics. 2015;38(9):e825-9.


20.	Putti AB, Uppin RB, Putti BB. Locked intramedullary nailing versus dynamic compression plating for humeral shaft fractures. J Orthop Surg (Hong Kong). 2009;17(2):139-41.


21.	Ma J, Xing D, Ma X, Gao F, Wei Q, Jia H, et al. Intramedullary nail versus dynamic compression plate fixation in treating humeral shaft fractures: grading the evidence through a meta-analysis. PLoS One. 2013;8(12):e82075.


22.	Benegas E, Ferreira Neto AA, Gracitelli ME, Malavolta EA, Assuncao JH, Prada Fde S, et al. Shoulder function after surgical treatment of displaced fractures of the humeral shaft: a randomized trial comparing antegrade intramedullary nailing with minimally invasive plate osteosynthesis. J Shoulder Elbow Surg. 2014;23(6):767-74.

Functional Outcomes Following Fixation of Shaft of Humerus Fractures - Intramedullary Nailing Versus Plate Osteosynthesis:  A Comparative Study

Department of Pediatric Neuroscience, Aster DM Healthcare, Al Qusais, Dubai, The United Arab Emirates 

Vivek Mundada

Department of Psychology, Al Noor Training Centre for Persons with Disabilities, Dubai, The United Arab Emirates 

Tanuka Gupta

Depart of Social Services, Al Noor Training Centre for Persons with Disabilities, Dubai, The United Arab Emirates 

Awit Pamfilo

Shijimol Joseph

Shameem Banu

Introduction: The COVID-19 pandemic and the lockdown imposed have affected most of us, including children with complex care needs. Because of various reasons including changed routine and social isolation, the physical and psychological health of these children have been affected. 


Method: This was an observational study with the objective of understanding and assessing the prevalence and spectrum of new-onset symptoms that children with complex care needs have been facing during lockdown, mainly due to their disrupted routine, including subsequent school closure. We selected a cohort of children who usually attend ‘Al Noor Training Centre for Persons with Disabilities’, which is a specialized center in Dubai, the United Arab Emirates, where they receive educational and therapeutic services from multidisciplinary team members. During the lockdown period, due to the COVID-19 pandemic, these children had to stay home and continue their sessions through a tele-rehabilitation program. All new symptoms presented and observed during these sessions were reported by the parents and interventionist to the research team. 


Result: 14.6% of the participants presented with a new-onset symptom. Amongst the different symptoms which were mainly reported by the parents were sleep impairment, headache, behavioural issues, non-epileptic seizures, and motor and vocal tics. All of the children had some form of sleep impairment.


Conclusion: Children with special care needs were at a higher risk of having negative impacts on their well-being during the lockdown period induced by the COVID-19 pandemic. Identifying such symptoms in these children can be difficult, but is crucial to managing them effectively.


The current COVID-19 pandemic has caused significant social, economic and health consequences across the world. Factors including social isolation and economic effects have also affected the mental and psychological health of many individuals. Children are one of the vulnerable groups to be affected.


Children’s clinical services and social activities have been disrupted in many countries as the result of the temporary closures of medical centres, schools, and caregiving agencies in the lockdown period. Children with neurodisabilities have faced additional challenges as a result of their functional limitations and changes to their daily routine1. For example, a child with autism may find it difficult to cope up with the new environment and exhibit behavioural symptoms like agitation. There could be added negative impacts on skill development due to the suspension of speech therapy or occupational therapy type sessions. It is not uncommon for these children to present with somatic symptoms such as headaches or abdominal pain.


With this observational study, we wanted to understand the prevalence as well as the spectrum of new-onset symptoms that children with complex care needs have been facing during the COVID-19 pandemic; mainly due to their disrupted structured routine, including a lack of ongoing support from their respective therapists as the result of lockdown and closure of medical facilities as well as special need schools.


For this observational study, the cohort of children receiving tele-rehabilitation in ‘Al Noor Training Centre for Persons with Disabilities’ in Dubai, United Arab Emirates was chosen. This school caters to the complex care needs of children with developmental and intellectual disabilities, and children have access to various services including physiotherapy, occupational therapy, speech and language therapy and psychology. The study primarily aimed to understand and assess the prevalence and spectrum of new-onset symptoms that these children with complex care needs have been facing during the lockdown, mainly due to their disrupted routine including subsequent school closure.


Between March 2020 and September 2020, a total of 164 children who usually attended the school daily received tele-rehabilitation from various therapists, depending on their ongoing care needs. Participants who reported any new symptoms, which was the inclusion criteria, were studied further for the purpose of the study. Participants who did not show any new symptoms were excluded from the study.  The newly reported symptoms were assessed by the respective specialists, or the child was referred to the relevant physician or specialist for formal assessment and management.


Out of the total 164 participants, 24 (14.6%) were assessed for any new-onset symptoms as such symptoms were only reported in these 24 participants. These new symptoms were either reported by the parents of the respective participants or were observed by the therapists during the tele rehabilitation. All the participants who had such new symptoms during this period were seen by the relevant specialists for the formal assessment and diagnosis of these symptoms. Of these children, 


8.4% were in the age group of 5-10 years, while 25% were aged 10-14 years. A third of these participants (33.3%) were between 14 and 18 years, and similarly, 33.3% were above 18 years. (Fig 1)


In terms of their background clinical diagnoses, 10 participants (41.7%) had Autism Spectrum Disorder with intellectual disability, 20.8% had Cerebral Palsy with intellectual disability, while 8.3% had diagnosis of Fragile X syndrome with intellectual disability. Predominantly, 91.6% of the new onset symptom episodes were observed in children who had intellectual disability. (Table 1)


All 24 participants (100%) had some form of sleep impairment, and were more vulnerable to disruption of sleep. Two-thirds of them had sleep-onset association type, and the rest had limit-setting type behavioural insomnia. All of the children were later referred to the clinical psychologist after the confirmed diagnosis by the sleep medicine specialist. The surge of behavioral problems was observed in 19/24 participants (79%). These included behavioural patterns such as increased aggression, agitation, being violent towards family members and headbanging episodes. 4/24 (16%) participants needed evaluation by a psychiatrist, while all of them were seen by the clinical psychologist. A third of these participants (33.3%) were diagnosed to have some form of anxiety and vocal tics respectively (Fig 2).


The next most common symptom was a headache. 62.5% of the participants included in the study had new onset symptom of headache. In all cases this was reported by their parents based on the observation. Following the assessment by the paediatric neurologist, all of these children were diagnosed to have tension-type headaches. None had any secondary type headache.


Two of the participants (8.3%) presented with new-onset seizure-like episodes which were assessed by the paediatric neurologist. These were diagnosed as non-epileptic seizures. Both of the cases had pre-existing epilepsy, but the new-onset seizures had a different semiology than their habitual seizures. Similarly, new-onset motor tics were observed in 2/24 male participants (8.3%), while vocal tics were seen in eight participants (33.3%), of which 5 (20.8%) were male. All of these children were subsequently assessed by a paediatric neurologist and neuropsychologist. (Fig 3)


Widespread outbreaks of infectious diseases, such as COVID-19, are associated with psychological and mental health issues2. Children are not indifferent and are prone to the dramatic impact of the COVID-19 epidemic. Understanding their reactions and emotions is essential to address their needs properly. These could be challenging, especially in children with complex care needs. These children can have difficulty in understanding the complexity of the situation and are faced with missing ongoing learning and therapy support as the result of lockdown. Parents can find it difficult to cope during such times. The lockdown also appears to have affected the parents in the form of increased anxiety and stress. They may therefore struggle to identify any such new symptoms in their children3.


There have been many published studies on the mental health consequences of children during the COVID-19 pandemic. Many children are presenting with various somatic symptoms such as tension-type headaches, tics, non-epileptic seizures, etc. Even children with complex care needs have been facing similar issues. The lockdown has been known to cause negative effects on morale, behaviour, and social interactions in these children. Although parents are performing therapies for such children at home, they could struggle to cope as they often lack the necessary professional expertise and usually rely on the support of their schools and therapists for any such issue4. 


With this study, we wanted to understand the physical issues that such children could be facing apart from their background physical and mental health needs. Such new symptoms could affect not only the children but their parents also. Moreover, these symptoms needed newer interventions which could be possible only after the appropriate identification and diagnosis of them.


Any new symptoms need to be carefully assessed as it can be very difficult to accurately diagnose them in children with complex care needs. Parents can play an important role in this process. For example, diagnosis of headaches according to the ICHD-3 classification for headaches can be challenging in such children5. Such diagnosis, however is important for appropriate management. The results of our study have also revealed that sleep is almost invariably affected in children with complex care needs. A changed routine during the lockdown may be the reason for their sleep impairment. 


It is important to understand and address any new symptom effectively as there may be an underlying psychological issue that needs attention. Children with neurodisabilities are prone to the long-term and negative effects of psychological stress, which is the result of environmental factors like the COVID-19 pandemic. They can eventually face high levels of stress, anxiety, and depression as a result6. If properly supported by healthcare professionals and the members of a multidisciplinary care team, children and their families can appropriately overcome any long-term distress. Such support can be provided through tele-health measures including tele-rehabilitation and tele-consultation. These could be appropriate and helpful ways to address the gap in the care of such children in situations such as lockdown7.



The authors would like to acknowledge all the class teachers and the parents of the children attending Al Noor Centre for Persons with Disabilities, Dubai, United Arab Emirates


The study protocol was approved by the internal ethical committee of Al Noor Training Centre for Persons with Disabilities Committee on research on 26th February 2020 (Ref no 146/2020-2021/DD).



The parents of the children involved in this research have given their written informed consent to use their children’s data for research.



All data generated or analyzed during this study are included in this article and its supplementary material files. Further enquiries can be directed to the corresponding author.


The authors have no conflicts of interest to declare

All authors contributed to the study’s conception and design. Material preparation, data collection, and analysis were performed by VM, TG, AP, SJ, and SB. The draft of the manuscript was written by VM. All the authors have read and approved the final manuscript

Lee J. Mental health effects of school closures during COVID-19. Lancet Child Adolesc. Health. 2020;4(6):421.

Achterberg M, Dobbelaar S, Boer OD, Crone EA. Perceived stress as mediator for longitudinal effects of the COVID-19 lockdown on wellbeing of parents and children. Sci Rep. 2021;11(1):1-4.

Cacioppo M, Bouvier S, Bailly R, Houx L, Lempereur M, Mensah-Gourmel J, Kandalaft C et al. Emerging health challenges for children with physical disabilities and their parents during the COVID-19 pandemic: The ECHO French survey. Ann Phys Rehabil Med. 2021;64(3):101429.


Arnold M. Headache classification committee of the international headache society (IHS) the international classification of headache disorders. Cephalalgia. 2018;38(1):1-211.


Wang G, Zhang Y, Zhao J, Zhang J, Jiang F. Mitigate the effects of home confinement on children during the COVID-19 outbreak. Lancet. 2020;395(10228):945-7.


Fazzi E, Galli J. New clinical needs and strategies for care in children with neurodisability during COVID-19. Dev Med Child Neurol. 2020;62(7):879.

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Impact of COVID-19 Pandemic Lockdown on Children with Complex Care Needs: An Experience from one Centre  in the United Arab Emirates

Aster Specialty clinic, Department of Neurology, International city, Dubai, UAE

Vishal Pawar

Medinova Diagnostic center, Department of Radiology, Dubai, UAE

Prasanna Kulkarni

Cerebellar strokes are one of the sinister causes of ‘acute vestibular syndrome’(AVS)1. In an emergency setting, it is crucial to differentiate between central and peripheral causes of AVS. The head impulse, nystagmus, and test of skew (HINTS) protocol helps in differentiating between the two2. Stroke at some specific locations can present with a positive head impulse test (HIT)3,and signs such as gaze-evoked nystagmus (with fixation) or skew deviation can be absent. In such a scenario, other central signs can help to differentiate a central from a peripheral cause.

We are presenting a case wherein the HINTS score favoured a peripheral lesion, but other central signs, including centripetal nystagmus without fixation and severe postural instability, helped in diagnosing the stroke. A 66-year-old hypertensive and diabetic man presented with a history of sudden onset rotatory vertigo, associated with nausea and vomiting, which had lasted for three days. The vertigo was spontaneous, and was associated with an imbalance of gait. He complained of swaying to either side on standing. The ocular alignment was normal during an alternate cover test. He showed no spontaneous nystagmus with visual fixation in light. The bedside HIT was positive to the left, with catch-up saccades. The patient also showed significant postural instability, with a tendency to fall to the right while standing. The findings of the HINTS testing are shown in Table 1. 

Video-oculography (Cyclops, Medtech. Bengaluru, India) showed normal saccades (Fig 1A, 1B). Smooth pursuit movement was saccadic in both the horizontal and vertical planes (Fig 2A,2B). The patient additionally showed centripetal nystagmus during lateral gazes in darkness, i.e. right-beating nystagmus during leftward gaze, and left-beating nystagmus during rightward gaze. 
An urgent CT of the brain (Fig-3) showed a hypodense lesion in the territory of the right medial posterior inferior cerebellar artery (m-PICA). MRIs confirmed the diagnosis (Fig-4). 


The patient was managed on an antiplatelet and a statin. He showed a significant recovery in 10 days. Positive HIT implies a deficit in the vestibulo-ocular reflex (VOR)4. Thus, a positive HIT in our case suggested the possibility of a peripheral lesion. However, positive HITs were also reported in patients with central lesions e.g. in patient with unilateral cerebellar lesion5. Indeed, in a cross-sectional study, three of eleven patients with acute vestibular syndrome had a stroke and positive HIT3. The authors proposed three different mechanisms defining the positive HIT in these particular cases. For vestibulo-cerebellar stroke, a positive HIT was considered to be the result of mass effect on the vestibular nucleus or eighth nerve root-entry zone in the pons. In a pontocerebellar stroke, a positive HIT was speculated to be due to labyrinthine infarction. In extensive case of Ponto-cerebello-labyrinthine stroke, a positive HIT was thought to be due to the infarction of the vestibular nucleus or eighth nerve root-entry-zone in the lateral pons2. However, positive HITs have also been observed in lesions restricted to the cerebellar nodulus or flocculus.6,7 A lesion involving the nodulus could have been the cause of the positive HIT in our patient. Thus, contrary to conventional perception, the presence of a positive HIT cannot be exclusively relied upon, and additional clinical features must be considered.

The cerebellum plays a significant role in maintaining optimal ocular motor performance.Centripetal nystagmus during eccentric gaze may reflect instability in the gaze-holding networks, similar to the velocity-increasing waveforms of downbeat nystagmus seen in some patients with cerebellar disease. Impairment of postural control is acommon finding in posterior circulation strokes. Thus, significant postural instability, the presence of vascular risk factors and centripetal nystagmus hinted towards a central cause of AVS.  This case highlights that clinical evaluation suggesting a peripheral lesion should never be taken in isolation, and needs to be correlated with other associated central signs.

Lee H. Neuro-otological aspects of cerebellar stroke syndrome. J Clin Neurol2009;5:65-73.

Newman-Toker D, Kattah J, Talkad A, Wang D, Hsieh Y. HINTS to diagnose stroke in the acute vestibular syndrome - Three-step bedside oculomotor exam more sensitive than early MRI DWI. Stroke2009;40:3504-3510.

Newman-Toker D, Kattah J, Alvernia J, Wang D. Normal head impulse test differentiates acute cerebellar strokes from vestibular neuritis. Neurology2008;70:2378-2385.

Armato E, Ferri E, Pinzani A, Ulmer E. Cerebellar haemorrhage mimicking acute peripheral vestibulopathy: the role of the video head impulse test in the differential diagnosis. Acta Otorhinolaryngol Ital 2014;34:288-291.

Baek S, Choi J, Jung J, Kwon D, Park M, Choi J, et al. Abnormal head impulse test in a unilateral cerebellar lesion. J Clin Neurol2015;11:279-282.

Lee J, Song K,  Yu IK,  Lee YH. A case of isolated nodular infarction mimicking vestibular neuritis on the contralateral side. J AudiolOtol 2019 23:167–172.
Park H, Kim J, Strupp M, Zee D. Isolated floccular infarction: impaired vestibular responses to horizontal head impulse. J Neurol2013;260:1576-1582.


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Cerebellar stroke presenting with positive head impulse test and centripetal nystagmus

Department of Pathology, Aster Hospital, Muscat, Oman.

Chhajlani P

Department of Internal Medicine and ICU, Aster Hospital, Muscat, Oman.

Khadar D

Compound sickle cell β thalassemia, chronic anaemia, sickle cell disease, haemoglobin electrophoresis, Hb S, Hb F, delayed diagnosis, Oman.

Hb S- sickle haemoglobin; Hb A- normal adult haemoglobin; Hb F- foetalhaemoglobin; SCD- sickle cell disease; MCV- mean corpuscular volume; MCH- mean corpuscular haemoglobin; MCHC- mean corpuscular haemoglobin concentration; RDW- red cell distribution width; RBC- red blood cell.

Sickle cell diseases are generally diagnosed in infancy or early childhood, with very few cases being undiagnosed until early adult life. Moreover, with a high percentage of Hb S in the blood, the patients present with variety of manifestations related to the disease. We present a rare case of a woman with minimal symptoms and no diagnosis of compound sickle cell β thalassemia up to the age of 40. The only complaint was of intermittent dull pain in the right upper abdominal quadrant. Haemoglobin electrophoresis revealed 92.5% Hb S

Sickle cell disease has a high clinical importance because of its worldwide distribution, prevalence and variety of manifestations.1 It is a genetic haematologic disorder presenting clinically at different years of childhood or early adulthood,2-4 depending on the genotype5,11 and the percentage of Hb S and Hb F in the peripheral blood.5 The genetic status of sickle cell disease includes a homozygous form, a heterozygous form and a double heterozygous form.1 Homozygous sickle cell anaemia cases have a higher percentage of Hb S, and totally lack normal adult haemoglobin (Hb A).1 These cases are typically diagnosed in the first year of life.1 The heterozygous forms have smaller percentages of Hb S and Hb A and rarely have phenotypic expression of the trait.1 The double heterozygous forms have varied amounts of Hb S and the other haemoglobin variant.1 These cases can vary clinically from being asymptomatic to having severe complications.1 Cases with high Hb S typically are diagnosed early in life.

A 40-year-old nulliparous woman presented to the hospitalwith the complaintof right upper quadrant abdominal pain of three-day duration. The pain was dull in nature and she had experienced similar episodes in the past. It was not related to food intake, nausea or vomiting. Her vitals were normal, with no tenderness or organomegaly on abdomen examination. The patient’s haemoglobin was 6.7 gm/dl, with low mean corpuscular parameters as shown in Table 1. The WBC and platelet counts were within normal limits. The serum iron was low (4.3 µmol/L), with normal ferritin (103.9 ng/ml). The total iron binding capacity and serum LDH were within normal limits. The peripheral blood smear showed a microcytic hypochromic blood picture with a few sickle-shaped cells and target cells with mild polychromasia (Figure 1). The corrected reticulocyte count was 6%. A screening test using sodium metabisulphite was positive for sickling. The conjugated bilirubin was slightly raised (5.1 µmol/L), while the total bilirubin was within normal limits. The other liver function tests, renal function tests and urine examination were normal. There were no abnormalities identified on CT abdomen and pelvis. Haemoglobin electrophoresis revealed 92.5% Hb S (shown in Table 2). High performance liquid chromatography (HPLC) showed 84% Hb S (shown in Table 3). With these findings, it was concluded that the case is one of compound sickle cell/ β+ thalassemia (Hb S/β+-Thal). It was found later that the patient’s haemoglobin had been in the range of 6-7 gm/dl for many years.

Case Presentation

The Sultanate of Oman has a high prevalence of hereditary haemoglobinopathies.6 Of these, SCD has a prevalence of 0.46%.7 In 40% of the cases of SCD, the diagnosis is made in the first year of life.8 This is based on the clinical manifestations and severity of the disease.1,8 The
 clinical manifestations result from polymerization of the deoxygenated haemoglobin, which forms the molecular basis of sickling. This, in turn, is dependent upon the concentrations of Hb S and other haemoglobin variants. Thus, the severity of the disease is determined primarily by the genotype. 


In Hb S/β+-Thal, variable amounts of Hb A dilute Hb S and prevent polymerization of Hb S.11 Hb F has an inhibitory effect on sickling,1,13 however, the Hb A and Hb F in our case were 1.2% and 3.3%, respectively, which are minimal in comparison with the 92.5% Hb S. This fails to explain the mild, intermittent symptoms in our case. There have been a few case reports of incidentally diagnosed doubly heterozygous Hb S/ β+-Thal patients in the prior literature, but all have been diagnosed at a younger age than our patient.

Haemoglobin electrophoresis for the patient’s parents, and genetic analysis for the patient, are mandatory to determine the genotype of the disease. Hence, the patient was referred to a government hospital for further evaluation and treatment. The patient has ten siblings, none of which have any symptoms of any haemoglobinopathy.

High Hb S% is generally related to severe clinical manifestations and early diagnosis. However, there can be mild clinical presentation with high Hb S%, which can lead to delayed diagnosis, as in our case. Hence, premarital, prenatal and new-born screening should be carridout to rule out haemoglobinopathies. Newborns with the disease should be offered genetic tests and appropriate treatment.

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