The National Paediatric Craniofacial Centre (NPCC) at Temple Street Children’s University Hospital provides specialist cares for over 660 children annually with syndromic and non-syndromic craniosynostosis, craniofacial tumours, craniofacial trauma, facial clefts, craniofacial growth disorders and facial asymmetry, torticollis, positional plagiocephaly and facial nerve disorders. The centre was established in the early 90’s by Professor Michael Earley, Consultant Plastic Surgeon and Mr David Alcutt, Consultant Neurosurgeon. The appointment of Mr Dylan Murray, Consultant Craniofacial and Plastic Surgeon and Mr John Caird, Consultant Neurosurgeon in 2008 brought with it the expertise to manage the full remit of craniofacial disorders in the unit. Before this time complex craniofacial conditions may have required referral to craniofacial centres in the UK for treatment. The service further evolved in 2013 with the appointment of a clinical nurse specialist. Craniofacial MDT clinics are run in Temple Street Children’s University Hospital (TSCUH) and at the satellite centres based in St James Hospital and Our Lady’s Children’s Hospital, Crumlin. A quarterly facial palsy MDT clinic is base at Temple Street Children’s University Hospital (TSCUH).
The reasons for referral of children to the centre are varied. A retrospective audit of the origin and nature of referrals received between the 31st June 2012 and 1st July 2013 was undertaken. This review was approved by the TSCUH audit committee. Almost half (48.6%) of the referrals to the service were from a Neonatologist or Paediatrician. General practitioners or community services comprised the second largest group accounting for 28.4% of the referrals. Irregularities in shape and flattening of the head, including plagiocephaly were common terms used (45.3%). Synostosis (20.7%), syndromic synostosis (2.2%), soft tissue or isolated bony growths (11.2%), facial asymmetry (7.8%) and concerns regarding fontanelles closed or open (5.0%) were other common referrals. In contrast to some craniofacial units in the UK, craniofacial vascular anomalies, positional plagiocephaly and disorders of facial nerve are treated by the NPCC1.
There were three peaks in the age of the children at referral; 4 to 6 months, one to two years, and three years and older. Age at referral is an important factor for some craniosynostosis as it will determine the surgical approach taken. Referrals from GPs were more often older than six months (57.7%). A referral to a Paediatrician instead of directly to the craniofacial services may have delayed referral to the craniofacial clinic but we cannot confirm this as this cohort did not show a proportionally older age of the children referred from Paediatricians.
The referral letter provides vital information required for appropriate triaging. Craniofacial synostosis particularly with signs of raised intra-cranial pressure, craniofacial tumours and craniofacial traumas are categorised as priority referrals and usually seen at the next available clinic. Over the time frame most, 96.1% of children were seen within six month of referral. The children who fell outside this time frame rescheduled their appointments by personal preference. The provisional diagnosis assumed by the referring physician was consistent with the diagnosis at outpatients for 49.7% of the cases; Neonatologists and Paediatricians were consistent in 52.3% of referrals and GPs were consistent in 34.0% of referrals. In the time frame 14.5% of children were determined normal at the clinic appointment. It is possible that children referred with positional plagiocephaly had resolution of their skull flattening at the time of review in clinic and were thus diagnosed as normal.
As is consistent with population density the majority of the referrals were from Dublin (36.6%) with 8.7% from Cork and 5.5% from Galway or Kildare. Multiple births were a feature in 4.4% the referrals. A positive family history of craniofacial conditions was noted for 2.3%. This included Crouzon’s syndrome and macrocephaly.
No preliminary investigations are expected for referral to the centre particularly if it delays the referral. A common mis-endeavour when craniosynostosis is suspected is to perform a plain skull x-ray. This investigation is challenging to report and does not give a definitive diagnosis of craniosynostosis- in the year review of referrals, 3.4% of children were referred with an incorrectly reported x-ray. The investigation of choice is a 3D skull and facial bones CT scan2. Children under the age of 3 years require a full anaesthetic or sedation to obtain good quality views and thus availability of a paediatric anaesthetist is essential. For the same reason, MRI imaging for investigation of soft tissue or intra-cranial abnormalities are best performed with paediatric anaesthetic support and within a multidisciplinary setting with a high patient throughput. With expansion of its case load from 2008 to 2012 the centre has seen a 209% increase in the number of referrals received. This is in part a reflection of increased awareness and diagnosis of conditions in the community and paediatric services. This increase has been reported in other craniofacial units1.
In order to guide referring physicians on important information required and aid in the management of an increasing number of referrals, the NPCC have introduced a structured referral form available on the www.cuh.ie and www.craniofacialireland.ie websites. The form is similar to referral forms used at the craniofacial units in The Hospital For Sick Children Toronto and Great Ormond Street Hospital3,4. The aim is that this referral form will standardise referrals and ensure that there is consistency across the professional bodies. Pre-clinic information regarding patient weight, height and head circumference parameters can be plotted at referral and referenced again at outpatients to monitor growth and development. Radiology images, if available can also be forwarded with referrals and uploaded to the hospital network for discussion at multidisciplinary meetings. Interpreter services can be arranged pre-appointment if required. The craniofacial team may also contact referring physicians if more information is required.
The new referral pathway will enhance communication between the NPCC and referring speciality and the family of children referred. This in turn will aid in the management of the increasing number of referrals. A new information section on the website detailing features of the most common craniofacial disorders will hopefully aid diagnosis and act as a useful guide for referrals.
TA Curran, A McGillivary, D Murray
Department of Plastic and Reconstructive Surgery, Children’s University Hospital, Temple St, Dublin 1
L Comey, Cleft Services Administrator, for assistance with data collection
- White N, Warner RM, Noons P, McAlister EM, Solanki G, Nishikawa H, et al. Changing referral patterns to a designated craniofacial centre over a four-year period. Journal of Plastic, Reconstructive & Aesthetic Surgery 2010;63:921-25.
- Perlyn CAMD, Marsh JLMD, Vannier MWMD, Kane AAMD, Koppel PPD, Clark KWMBA, et al. The Craniofacial Anomalies Archive at St. Louis Children’s Hospital: 20 Years of Craniofacial Imaging Experience. Plastic & Reconstructive Surgery 2001;108:1862-70.
- Children Thfs. How to make a referral. http://www.sickkids.ca/Craniofacial/What-we-do/How-to-make-a-referral/index.html.
- Hospital GOS. Contact the craniofacial unit. http://www.gosh.nhs.uk/medical-information/clinical-specialities/craniofacial-information-for-parents-and-visitors/contact-us/.