Congenital midline cervical cleft leading to contracture of neck.

Congenital mid-line cervical cleft (CMCC) is a rare congenital anomaly of the neck. The first description of this abnormality was documented in 1924 by Bailey.[1] The treatment of CMCC is surgical excision of the lesion and subsequent repair and surgery can be performed whenever the child is considered fit for general anaesthesia, usually before the second year of life.[2] However, earlier repair is recommended in more severe cases.[3] We came across a rare clinical presentation of mid-line cervical cleft that presented in adulthood with contracture of the neck.

Impaired fusion of branchial arches is considered as the most accepted theory explaining the aetiology of CMCC[2] [Diagram 1]. Various mechanisms of improper fusion have been proposed, including[1] ischaemia and necrosis secondary to mechanical factors and vascular anomalies,[2] failure of the mesenchyme to penetrate the midline, resulting in poor interaction between the mesoderm and ectoderm, and pressure exerted on the cervical area by the pericardial roof.[3]

Diagram 1

The development of branchial arches

A 20-year-old female presented with a midline skin lesion in the anterior part of neck since birth. The patient complained of gradually increasing restriction of neck mobility and aesthetic deformity due to the skin lesion. The midline skin lesion over the anterior neck was 8 cm in length and 1 cm in width. A nipple-like protuberance of skin was noted superiorly, which continued as a sinus tract inferiorly [Figure 1a]. This is in accordance with other cases reported so far.[2] The neck mobility was restricted to an extension of 30 degrees above the normal forward gaze [Figure 1b]. However, the patient had no difficulty in carrying out her activities of daily living. No other head and neck abnormalities were noted. A fistulogram revealed the sinus tract ending in a blind pouch just above the sternal notch. The lateral neck skiagram and computed tomography (CT) of the head and neck were normal. No associated congenital anomalies were found on detail systemic examination.

Figure 1

(a) Preoperative photograph showing superior nipple-like projection of skin with a sinus tract below. (b) Preoperative lateral view

The presentation of CMCC is typically having three elements – a small nipple-like structure in the superior portion, a cleft in the middle portion, and a small sinus tract in the inferior portion.[3] In accordance with earlier case reports, the present case also presented with all these three elements. The only unusual aspect was that the patient presented in adulthood.

The lesion was excised by an elliptical incision taken all around it. Per-operatively, a clearly outlined sinus tract was found in the supra-platysmal plane with band-like fibrosis involving the platysma in the midline. The sinus tract was dissected free from the surrounding structures and complete excision of the sinus tract was performed [Figure 2]. Platysmal band was released and normal neck extension was achieved on table. The skin was closed with multiple Z-plasties [Figure 3]. The postoperative course was uneventful and the sutures were removed on day 7. The patient was however lost to long term follow-up. Our personal communication with the referring physician suggests that the patient was symptom free and happily married.

Figure 2

Intraoperative view showing the supraplatysmal sinus tract

Figure 3

Postoperative view showing the multiple Z plasty closure

The indications for surgery in CMCC are aesthetic, prevention of cervical contractures, associated clefts of lip and mandible, and other associated congenital defects. Simple excision or transection of the fibrous band leads to recurrence of scarring and fibrosis.[4] Simple closures are not cosmetically pleasing in the long run due to recurrent scarring. Straight line closure also leads to a higher incidence of neck contracture recurrences.[2] It is therefore recommended that the defect should be closed using multiple Z-plasty technique.