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J Ped Surg Case Reports 1 (2013) 99e101
Journal of Pediatric Surgery CASE REPORTS
journal homepage: www.jpscasereports .com
Congenital midline cervical cleft
Juliette King, Ramnik V. Patel, Simon N. Huddart*
Department of Pediatric Surgery, University Hospital of Wales, Heath Park, Cardiff CF 14 4XW, Wales, UK
a r t i c l e i n f o
Article history:Received 27 January 2013Received in revised form28 March 2013Accepted 2 April 2013
Key words:Midline cervical cleftMidline cervical sinusCongenital midline cervical anomaliesBranchial arch defectsZ-plasty
* Corresponding author. Tel.: þ44 (0)29 2074 5342;E-mail address: [email protected]
2213-5766/$ e see front matter � 2013 Elsevier Inc. Ahttp://dx.doi.org/10.1016/j.epsc.2013.04.002
a b s t r a c t
We report a case of a neonate with a congenital midline cervical cleft diagnosed postnatally at birth.Classical clinical presentation, fistulogram and microlaryngoscopy established prompt clinical diagnosisand excluded associated anomalies. She underwent operative treatment during early infancy withexcellent cosmetic and functional long-term results.
� 2013 Elsevier Inc. All rights reserved.
Congenital midline cervical cleft (CMCC) is a rare anomaly of theanterior midline of the neck identified at birth. There have beenfewer than 50 reports in the English literature [1]. We presenta classic case of CMCC treated with multiple Z-plasties with excel-lent long-term functional and cosmetic results.
1. Case report
A baby weighting 3100 g was referred to us with a congenitalneck abnormality noted at birth. She was born at term by normalspontaneous vaginal delivery after an uneventful pregnancy. Preg-nancy scans at 12 and 20 weeks were reported normal. Her parentswere healthy and nonconsanguineous. The patient had no airway orfeeding difficulties, and her cry was normal.
On examination, there was a vertically oriented cleft of pinktissue in the anterior midline neck, with an associated nipple likeprojection at the cephalic end and a sinus tract opening caudally,discharging mucoid material (Fig. 1). No other associated malfor-mations were noted.
A fistulogram performed at 2 months of age showed the sinusto be a narrow channel, 0.9 cm in length, coursing toward thesuprasternal notch and ending abruptly. There was no connectionwith other structures.
fax: þ44 (0)29 2074 6322.es.nhs.uk (S.N. Huddart).
ll rights reserved.
The patient was treated surgically at 3 months of age. At micro-laryngo-broncho-scopy; pharynx, larynx, and the tracheobronchialtreewere normal. The cleft with nipple like skin tag at cephalic end,underlying fibrotic tissue and the sinus at the caudal end wereexcised completely, and the vertical elliptical wound was closed bymultiple primary Z-plasty (Fig. 2).
Histological examination of the excised tissue showed the cep-halic skin tag of normal epidermis with occasional bundles ofskeletal muscle. Cleft was lined by stratified squamous epitheliumwithout skin appendages. The sinus tract was lined by pseudos-tratified nonciliated columnar epithelium with occasional area oftubuloalveolar glands of the bronchial type.
The postoperative course was uneventful. At 9 year follow-up,she is well and the cosmetic result is excellent with no restriction ofmovements or any contracture functionally.
2. Discussion
CMCC is a rare congenital fusion anomaly of anterior midline ofthe neck. Exact embryology is unknown but it appears to occur asa result of fusion abnormality of the paired first and second bran-chial arches in themidline during embryogenesis [2]. It is usually anisolated anomaly but associated other malformations have beenreported sporadically with other clefts of the tongue, lower lip,and mandible [3e6]. Two cases at one hospital within a span of10 days have been reported [7]. It occurs predominantly in Cauca-sian females.
Fig. 1. Front view of the lesion. Note: nipple like skin tag at the top, cleft in the middleand the sinus distally.
Fig. 2. Pre per and postoperative planning, performance and closure of multiple Z-plasties. Note: complete excision of the fibrous cord.
J. King et al. / J Ped Surg Case Reports 1 (2013) 99e101100
Clinically, the presenting signs appear along a wide spectrum,ranging from a simple cleft of the soft tissue to an extreme defectassociated with clefting of the mandible and/or sternum anda possible loss of other midline structures, such as portions of thehyoid bone. They usually are detected at birth as cutaneousweeping ulceration with an overhanging skin or a cartilaginous tagin the midline anterior neck.
There is usually a sinus tract protruding downward from thecutaneous component, which in complete clefts may makeconnection with the sternum or mandible. Alternatively, the
external skin tag may cover a short sinus that ends in a blind pouch[8]. Histologically, the skin tag, cleft and sinus tract consist ofmesodermal elements, with fibrous tissue and interwoven skeletalmuscle and occasional glandular structures.
Although most patients are asymptomatic at diagnosis withan apparent cosmetic concern due to ugly appearance of theCMCC, neck contractures and mandibular or sternal growthabnormalities may develop in untreated patients. An exostosisfrom the midpoint of the mandible can form resulting frompersistent traction from the contracting fibrous cord under-neath the cleft. CMCC can prevent full extension of the neck,result in micrognathia and torticollis, predispose patientsto infection, and can coexist with other clefting defects orcysts [1].
Surgical intervention is, therefore, necessary to avoid potentiallong-term complications, such as scarring, contractures andlimitation of neck mobility. Complete surgical excision of the cleftincluding the underlying fibrous cord is recommended procedureof choice preferably in the infancy. The reconstruction involvesmultiple Z-plasty procedure to avoid the formation of hypertro-phic scars and provide enough length to avoid contracture ata later date. This is of cosmetic and functional importance toavoid the inevitable scarring and contractures which follow laterin the life.
Recurrence rates are high and have been reported up to 9years after initial operation. Complete surgical excision of theCMCC lesion and entire subcutaneous fibrous cord similar tochordee correction in hypospadias surgery is necessary tolimit this risk. Simple short sinuses less than 2 cm lengthmay be excised through stair step incisions, with a techniquesimilar to that used for some second branchial clefts.More complicated clefts are excised with a series of Z-plasty
incisions that improves the functional and cosmeticresults.
References
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[3] Van der Staak FH, Pruszczynski M, Severijnen RS, van de Kaa CA, Festen C. Themidline cervical cleft. J Pediatr Surg 1991;26:1391e3.
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