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Chiropractic Journal of Australia : CJA December 2013
Chiropractic Journal of Australia Volume 43 Number 4 December 2013 125 refexes, muscle tone, and muscle stretch refexes were unremarkable. The patient was referred to a Paediatrician. Whilst waiting for this referral appropriate corrections were made to the shoulder, upper cervical and cranial regions. There was later confrmation of left lambdoid craniosynostosis, with surgery booked for the following month. Case Two: OH, aged 9 weeks, presented with a "lump" on back of the head, with concerns regarding delayed resolution of the "molding process" during birth. There was a mild right head preference and concern about possible head shape effects. There had been a GP examination at 6 weeks, with no concerns identifed. The pregnancy history was unremarkable. Labour was at 40.3 weeks, with spontaneous contractions, and 23 hours of phase 1 labour. There was failure to progress resulting in emergency caesarian. The birth weight was 3.790 kg, length 52.5 cm, head circumference 35.0 cm, and Apgar scores were 9 (1 minute) and 10 (5 minutes). OH was breastfed, with good attachment and good suck. There was arching/extension fussiness occurring after 15 minutes, worse on the right. There was no choking, gagging or coughing throughout the feed. Night sleep was normal for age, however day sleeps were short (10-60 min) and OH was easily woken and unsettled throughout the day. OH was comfortable with prone (tummy) time, nappy changes and getting in/out of the car seat. There was a dislike of dressing near the shoulder/neck. The systems review was unremarkable, except for some issues with straining, with frequency between bowel motions of up to 10 days. There was a family history of allergies to penicillin, and congenital heart defects. On examination there was a large, rounded protrusion at the posterior occipital base. There was no clear ridging at the lambdoid suture, but the palpation at this region felt atypical. Subluxation patterns were present at the occiput and sacrum. A positive scapulohumeral refex was present, indicating irritation of upper cervical nerve roots.12 Muscle tone, primitive refexes and muscle stretch refexes were all normal for age. Examination of the hips, shoulders and other extremities were all unremarkable. Due to the unusual presentation of posterior occipital base prominence and atypical palpation at the lambdoid suture, the patient was referred to the plagiocephaly department of a major hospital via their GP. On phone follow-up the mother had been given a diagnosis of "fused skull plates" and there had been a booking the next month for surgery with a neurosurgeon. DISCUSSION There are several ways to classify craniosynostosis. In the "simple" type, only one suture is involved -- in the "complex" type, multiples sutures are involved. In "Primary" craniosynostosis there are no associated conditions, in "secondary" craniosynostosis, there is an underlying disorder (e.g. thyroid, haematologic, metabolic).13 In "Syndromic" craniosynostosis there are other genetic conditions associated, with concomitant orthopaedic, neurologic and system anomalies (see Table 1). RECOGNISING CRANIOSYNOSTOSIS O’NEIL • STEWART Table 1 SYNDROMIC CRANIOSYNOSTOSIS AND ASSOCIATED SIGNS: CONDITION KEY SIGNS OTHER SIGNS Apert Syndrome23 Coronal suture synostosis Wide-set eyes, Shallow orbits Mid-face hypoplasia Choanal stenosis Megalocephaly Cognitive impairment Cardiac and renal anomalies Syndactyly Crouzon Syndrome23 Brachycephalic, Craniosynostosis Hypertelorism, Proptosis Maxillary hypoplasia Beaked nose, Cleft palate Hydrocephalus Chiari 1 malformation Hind-brain herniation Ear and cervical spine pathology Carpenter Syndrome44 Acrocephaly - variable Synostosis of multiple sutures Syndactyly, Polydactyly Kyphoscoliosis Congenital heart disease Mental retardation Hypogenitalism, cryptorchidism Umbilical hernia, Genu valgum Muenke Syndrome45 Genetically determined Uni/bi-lateral coronal sutures Facial anomalies (in 50%) Thimble-like middle phalanges Sensorineural hearing loss Mental retardation Saethre- Chotzen Syndrome46 Craniosynostosis Flat forehead Straight nasal bridge Low Frontal hairline Ptosis, Hypertelorism Arched or cleft palate Pfeiffer Syndrome47 Craniosynostosis Ocular proptosis Brachydactyly, Syndactyly Hydrocephalus Hearing loss, Choanal atresia Seizures, Mental retardation The incidence of craniosynostosis as a group in Australia and overseas varies from 2.6/10,000 to 6.4/10,000, with increasing incidence.14-16 When looking at each type of craniosynostosis, the rate of sagittal synostosis is approximately 2.8/10,000 births, with increasing incidence.14 Approximately 72% of cases are sporadic.19 The rate of
CJA September 2013