Category Archives: Clinical

Trichotillomania

Clinical, General paediatrics, OPD

Or repetitive hair pulling.  Previously classified as an impulse control disorder, ie a sense of tension that is only “satisfied” when hair is pulled out. However, many children do not get this tension and gratification so in DSM-V trichotillomania is included among obsessive-compulsive and related disorders.

Dutch cohort mostly girls, literature says no gender difference!   Nail biting can co-exist, as can stereotypies.  Many kids will also eat their hair once it is pulled out.  Most common age of onset is in early adolescence (9-13 years), but frequently occurs in early childhood, even as early as 12 months of age.  Triggering factors identified include concerns about physical appearance, family and school issues, and concurrent illness.  Parents sometimes also pull their hair, so maybe (partly) learned.

Two distinct types of trichotillomania described: automatic and focused

  • Automatic – outside of own awareness, may not recall actual pulling, but may admit to ‘playing with their hair’ or may have been noted to pull their hair in a distracted state.  Children tend to fall into this category.
  • Focused – aware, in response to negative emotion or urges

Parents often miss the hair pulling and only present when hair clumps noticed on surfaces (esp bed –  presumably due to pulling in sleep) or bald patches appear.

On Examination

Exclamation mark hairs (thin proximally, at scalp, normal distally), usually thought of being evidence of alopecia areata, may be seen, so not very predictive.  Pull test – gentle traction on about 20 hairs in 3 different locations.  Positive if more than 5 hairs extracted – suggests active alopecia areata.  You may miss dormant alopecia, but in that case hair regrowth should occur.

[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4857813/]

Stereotypy

Clinical, General paediatrics, Neurology, OPD

Where children present with abnormal movements, consider:

Stereotypies are repetitive non-functional movements, typically hand flapping or twisting, body rocking, head banging/nodding, grimacing, arm flapping. As with tics, there is often a family history, and there is an association with obsessive compulsive tendencies.

They can be present in children with normal development, but are a feature of neurological disorders especially autism spectrum disorder and sensory impairment.  In these children, the movements are part of a period of introspective absorption, they make prefer such activity to conventional social interactions.

There are a number of differences from tics, although they can co-exist:

  • Sterotypy presents younger, eg under 2 yrs.  Tics present from 4 onwards.
  • Tics can vary over time, so grimacing moves on to  shoulder jerks, then moves on to clearing throat.  Stereotypy movements are unchanging.
  • Stereotypy movements are rhythmic, rather than just a single jerk
  • Tics are brief, stereotypy can be prolonged
  • Tics have a premonitory sensation (although only older children may be aware)
  • Tics can be suppressed with effort.  Children with stereotypy can be distracted but may resent it! (Similarly self gratification)

Excitement and stress are triggers for both.  Over time, the child usually becomes aware of social disapproval and may suppress the behaviour except in secret!

[Ulster Med J 2014;83(1):22-30]

 

 

 

Sacral dimple

Clinical, Congenital, General paediatrics, Neurology

Typical sacral dimples are <5mm in diameter, within 25mm of anus and located in midline.  Rate of spinal  dysraphism (bifida occulta) less than 1%.

Higher risk if do not fulfill these criteria. Lipomas, deviated/bifurcated crease are the most likely to be associated with dysraphism.  Otherwise you expect at least 2 or more cutaneous markers (hair tuft, haemangioma, Mongolian spot, skin tag/tail).

Reports of high frequency of hair tufts in diastematomyelia probably refer to more striking lesions (“faun tails”).

Royal College of Radiology has policy  – ignore sacral dimples unless atypical, or in combination with other lesions.

USS if neonate, but MRI if US abnormal or equivocal, where neurological signs (bladder, bowel, lower limb) or lesion discharging.

[Arch Derm 2004]

Episodic autonomic symptoms

Clinical, General paediatrics, Neurology, OPD

Intraosseous needle technique

Clinical, General paediatrics

Proximal humerus preferred in adults! Less pain, nearer heart. Lay elbow across abdomen. Half way between axilla and lateral aspect, feel for neck. 45 deg angle.

All sizes are same gauge, just length different. Judge soft tissue depth with thumb. If you can’t see first black line then prob too short for safety!  Don’t put patient into MRI!

Contra indications: local infection, previous attempt, fracture. Osteoporosis is less of an issue with EZIO driver, cf Cook’s needle.

Hard flush initially to help flow. Pain of infusion is severe – often worse than pain of insertion.  Consider lidocaine (without adrenaline) 0.5mg/kg IO over 2 mins, then allow 1 min dwell time, BEFORE flush.

Compartment syndrome is major complication (due to extravasation) – diproportionate pain, esp on passive stretching.  Later pallor, paraesthesiae, pulseless.

72hr life.  Removal can be tricky – secret is to remove extension tube and attach syringe directly to hub, for better grip.

Capillary refill time (CRT)

Clinical, General paediatrics

In children over 7 days of age, the upper limit of normal CRT is approximately 2 s when measured on a finger, and 4 s when measured on the chest or foot, irrespective of whether the child is feverish or not. Longer pressing times and ambient temperature outside 20°C–25°C are associated with longer CRT.

Evidence suggests that the use of stopwatches reduces variability between observers.

Recommend following standardised CRT method: press on the finger for 5 s using moderate pressure at an ambient temperature of 20°C–25°C. A capillary refill time of 3 s or more should be considered abnormal.  Other timings apply to other sites.

[Systematic review – 21 studies on 1915 children.  Arch Dis Child doi:10.1136/archdischild-2014-307079]

Blood Culture

Clinical, General paediatrics, Infectious disease

Essential investigation in sepsis, particularly where unusual organisms or deep seated infection eg endocarditis.

The volume of the sample is important. Small volumes have higher false negative rate, and are slower to become positive – 6ml superior to 2ml [j Peds 1996].

Traditionally, thought to be most effective when done at time of pyrexia, but there is little evidence for this.  In a study of 1,436 adult patients with bacteremia and fungemia, the likelihood of documenting bloodstream infections was not significantly enhanced by collecting blood specimens for culture at the time that patients experienced temperature spikes. Nor was there any benefit for any subgroup eg patient age, gender, white blood cell count and specific cause of bacteremia. [J Clin Microbiol. 2008 Apr; 46(4): 1381–1385.  doi:  10.1128/JCM.02033-07]

Also traditionally, considered negative at 48 hours. Canadian study of 98 positive blood cultures in babies up to 90 days of age found 96% of true pathogenic cultures were positive at 24 hours, with 100% positive at 36 hours. Mean time to positivity was 14.4 hours in pathogenic bacteria and 23.2 hours in contaminants.  [DOI: 10.1093/jpids/piv078]

US study of 256 non-critically ill babies up to 60 days of age found median time to positive blood cultures of 16.6 hours for pathogens cf 25.1 for contaminants, for CSF cultures 14 hours for pathogens cf 40 for contaminants. 82% of pathogens positive within 24hrs for both blood and CSF [can’t see figures for 36/48hrs yet, full text embargoed?][Hosp Peds 2020]

Another US study of 392 cases (outside of PICU) found 96% of pathogens positive by 36hrs (95% CI 95-98), and 99% at 48hrs. But not clear how many of these would have been well enough to go home at 36 hours.  Estimated that observation >36hrs would identify 1 bacteraemic infant for 1250-2778 infants [Biondi, JAMAped2014]. Note that there were significant differences between organisms (E coli faster, staph slower), and between sites (sample volumes? time to inoculation?). No correlation with degree of fever, interestingly.

Current Western Australian guideline, FeBRILe3, appears to be safe.

Canadians have published a new position statement too.

In a tertiary neonatal unit, 72 hours was considered necessary. [ADC Fetal & Neonatal 2001]

Plagiocephaly

Clinical, Common, General paediatrics, OPD

Differential

The clinical criteria for a unilateral lambdoid synostosis consist of an ipsilateral occipital flattening, a depressed ipsilateral ear lobe (inferior movement) and a parallelogram-like shape in the posterior view. All three of these signs were present in the eight synostotic infants. Furthermore, all children had developed a compensatory contralateral parietooccipital bulging that led to a slanted tree top-like shape of the head at follow-up. Normal posterior view (ie ears level) and anterior movement of the ear excludes LS [but photo looks like ipsi anterior movement in LS – is it contralat in PP??? No mention of anterior bossing, not obvious in photo].

German study – all LS cases obvious clinically. Where positional plagiocephaly was doubted, USS demonstrated patent sutures.

[Arch Dis Child 2015;100:152-157 doi:10.1136/archdischild-2014-305944]

Monitoring

Measure the oblique diameter left (ODL) and oblique diameter right (ODR) lines are drawn from points located 40° either side of the antero-posterior (AP) line. 40° is typically where deformation most notable.  Express as difference (the Oblique diameter difference (ODD) = ODL−ODR) or else ratio between the ODL and the ODR (oblique diameter difference index, or ODDI).

[European Journal of Pediatrics March 2006, Volume 165, Issue 3, pp 149-157]

Treatment

Dutch RCT of 6 months of helmet therapy (n=84 infants aged 5 to 6 months with moderate to severe skull deformation, exclusions were prems, muscular torticollis, craniosynostosis, or dysmorphic features). Full recovery was achieved in 10 of 39 (26%) participants in the helmet therapy group and 9 of 40 (23%) participants in the natural course group (odds ratio 1.2, 95% confidence interval 0.4 to 3.3, P=0.74). All parents reported one or more side effects.

[van Wijk RM BMJ 2014; 348 (); g2741]

Some evidence for bedding pillows (but SUDI risk?) and stretching exercises.

Haemangiomata

Clinical, Common, Dermatology, General paediatrics

2018 classification (ISVVA.org) – rather functional and lacking in poetry!

Basically benign tumours, involving blood vessels.  Seen in 12% of all infants  – more common in girls, whites, premature infants, twins and are babies born to mothers of higher maternal age!  Mostly seen in head and neck region, including the face, but can be anywhere.

Tumours distinguished from malformations.

Infantile Haemangioma

Cutaneous/mucosal haemangiomata usually develop after birth, appearing in the first 8 weeks of life.  They then develop and grow for 6-12 months, often resembling a strawberry.  Most then start to reduce and fade gradually, although it can take up to 9 years.   Often there will be complete disappearance with no cosmetic defect, but there may well be scarring, telangiectasia, or loose fibro-fatty tissue.

Can be further differentiated by depth (superficial tend to be raised and bright red, deep are generally darker red or even purple/blue, they can also be mixed) and extent/pattern (focal or segmental).

Typically they are in the skin and soft tissues, but can sometimes affect the liver or airways.  Associated with GLUT-1 positive staining on biopsy.

Congenital Haemangioma

Much less common. Present at birth and do not progress, although they may grow proportionally with child.  Oval or round, plaques or exophytic.  Some rapidly involute during the first year of life but otherwise they are permanent.

Vascular malformations

Grow slowly compared with vascular tumours.  Usually present at birth but perhaps inconspicuous until child grows.  Can involve arteries, veins, lymphatics in various combinations.

Capillary malformations most common – dilated capillaries, classic port wine stain (naevus flammeus).  Darken over time, do not regress.  Can be associated with bone or soft tissue overgrowth. Multiple can be associated with underlying AVM!

Nevus simplex is the classic “stork bite” at the nape, eyelid or forehead at birth. Lighter, regress.

Venous malformation more ill defined, bluish, easily compressible.  Multifocal tend to be autosomal dominant.  Some syndromes eg Blue rubber bleb naevus syndrome (widespread, including palms/soles).

Lympoedema and cystic hygroma are the lymphatic versions.

Others

  • Pyogenic granuloma – reaction to trauma, well demarcated, raised or even pedunculate
  • Telangiectasia eg Hereditary haemorrhagic telangiectasia (HHT)
  • Angiokeratoma – characteristic of tubersclerosis
  • PHACE syndrome (post fossa malformations, haemangiomata, arterial anomalies, cardiovascular defects, eye anomalies – but also midline defects)
  • Tufted angioma and Kaposiform haemangioendothelioma – similar histologically, but latter bruised, purpuric appearance, infiltrate into muscle/adipose tissue and associated with Kasabach-Merritt syndrome (consumptive coagulopathy).

[Seminar intervent radiol 2017][Ped dermatology 2016]