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Short Communication
MIDLINE CERVICAL CLEFT: A REPORT OF TWO CASES Somnath Saha, Saibal Misra, Vedula Padmini Saha, A. R. Mondal ABSTRACT: Congenital midline cervical cleft is a rare congenital anomaly. The cardinal diagnostic features are- (1) Fistula’s opening are located caudally (2) Intermittent serous fluid discharge in the early neonatal period (3) Nipple like appearance of the cleft in the superior aspect (4) Widened scar and minimal neck contracture in later life. Two such cases are reported herewith with brief review of literature and it’s surgical management. Key Words: Midline cervical cleft, Associated anomaly, Management
INTRODUCTION Although clefting of lip and palate are not a rarity amongst the congenital malformations, midline cervical clefting are of infrequent occurrence. Facial clefting usually occur due to failure of fusion between facial processes and nature’s attempt to bridge the gap by penetration of mesoderm, which acts as a secondary healing effort. The severity of cleft usually depends on the success of secondary healing effect. Tessier in 1973 presented a classification of craniofacial cleft. In his classification midline clefting of lower lip and mandible coincide with the caudal extension of the no. 0 cleft. Though Tessier had tentatively labelled it as no. 30 cleft. This group includes mandibular process cleft, branchiogenic medial axial B2 cleft and midline branchiogenic syndrome.1 Midline cervical cleft represent a variant of no. 30 cleft of Tessier classification. We are reporting two such cases in which one case was associated with congenital stapedial fixation leading to bilateral conductive deafness. CASE REPORT Case 1 A three-year-old girl presented to OPD with history of discharging midline sinus of the neck since birth (Figure 1). The discharging sinus was located in between cricoid and suprasternal notch. A hood of skin was observed in the superior aspect of the sinus. There was no associated congenital anomaly. A thyroid scan was done and thyroid was found in normal location. Due to its typical appearance of discharging fistula draining caudally with a hood of skin in the superior aspect of fistula, the case was diagnosed as a midline cervical cleft and the tract was excised. The wound was closed by multiple Z plasty. Post-operative period was uneventful.
Figure l: Clinical Photograph of case no. 1 showing a hood of skin in the superior aspect of fistulae
Case 2 A 18 year old male patient presented with a midline cervical scar with history of limitation of motion of neck (Figure 2). The scar resulted from a discharging neck sinus since birth. He also gave history of decreased hearing acuity of both ears without any history of aural discharge. On examination, a widened scar in the midline of neck with a cord of subcutaneous tissue was found which was limiting the
Figure 2: Clinical Photograph of case no. 2 showing a midline cervical scar
Medical College Hospital, Kolkata - 700 073, India Indian Journal of Otolaryngology and Head and Neck Surgery Vol. 57, No. 1, January - March 2005
Midline Cervical Cleft: A Report of two Cases
motion of neck. His neck muscles were hypoplastic. Both tympanic membranes were within normal limits. Pure tone audiometry was suggestive of A-B gap of 50 dB and acoustic reflexes were absent in ipsilateral as well as contralateral stimulation. Tympanometry was suggestive of As type of curve. He was diagnosed as a case of midline cervical cleft with congenital stapedial fixation. The neck contracture resulting from midline cervical sinus was corrected by excision and multiple Z plasty and the postoperative result was satisfactory. DISCUSSION Midline cervical cleft can be considered as a variant of Tessier no. 30 cleft without any major bony defect. It should not be considered as a true cleft as there is no gap between adjacent skin structure.2 In Tessier no. 30 cleft, major deformity i.e. mandibular clefting occurs due to failure of fusion of 1st branchial arch structure, whereas lower neck deformities occur due to alteration of normal fusion of 2nd and 3rd branchial arches. Cleft may extend from alveo-lingual sulcus3 to lower neck even up to sternum. Severe form of midline cleft is usually associated with absence of hyoid bone or improper development of thyroid cartilage. 4 Although some authors described it’s occurrence exclusively in Caucasian female2,5 it is not rare in Asians as evidenced in this review. Serous fluid weeping in the early neonatal period is a common presentation with gradual resolution of the drainage over the next several months.6 The cleft is usually reddened with atrophic and desquamated epithelium.7 The superior aspect of the cleft has a nipple like appearance6 as evidenced from our 1st case. Some author believed that intermittent drainage is due to presence of heterotropic salivary tissue in the inferior aspect.6 Bulging of neck and sternal region during straining is another feature of midline clefting in neck. This is due to thick and hypoplastic strap muscle of neck, which fails to support the trachea and lung.1
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in any of the available literature though deafness due to heterozygous mutation has been reported.10 The differential diagnosis includes other congenital midline sinus of neck and should be differentiated from thyroglossal fistula. The fistula from a midline cervical cleft, in greneralis located caudally, while that due to the thyroglossal duct anomalies, they are located cranially.6 Occasionally a thyroglossal fistula and a midline cleft of neck may coexist. Excision of the contracture and multiple Z plasty is advocated in late cases.2 Early excision is advised to prevent contracture and for cosmetic improvement. REFERENCES 1.
Kawamoto HK Jr, Wang HK, Macomber BW. Rare Craniofacial Clefts-in Reconstructive & Plastic Surgery Vol. 4, 2nd Ed: WB Saunders’ Co: Philadelphia; 1977,p.2116-64.
2.
Fincher SG, Fincher GG. Congenital midline cervical cleft with subcutaneous fibrous cord. Otolaryngol Head Neck Surg 1989;101:399-401.
3.
Chatterjee S, Das S, Khan JA, Bhattachraya RN. An Uncommon case
4.
C.V, Jordon HE. Midline Cleft of the Lower Lip & Mandible,CIeft
of Neck Fistula. J Indian Med Assoc 2002;100:24. sternum & absence of basihyoid. Arch Surg 1935;30:647-56. 5.
Anderson BL, Svendsen EP. Midline cervical clefts. Scand J Plast
6.
Vander Staak FH, Pouszezynski M, Severijnen RS, Van de kaa CA,
Reconstr Surg 1978;12:169-70. Festen C. The midline cervical cleft. J Paediatr Surg 1991;26:13913. 7.
Gottlich E, Lewin ML. Congenital midline cervical cleft of neck N
8.
Surendran N, Varghese B. Midline cleft of lower lip with cleft of
Y State J. Med 1966;66:712-8. mandible and midline dermoid of the neck. J Paediatr Surg 1991;26:1387-8. 9.
French WE, Bale BF. Midline cervical cleft of neck with associated branchial cleft. Am J Surg 1973;125:376-81.
10. Andryk JE, Kerschner JE, Hung R, Aiken J, Conley SF (): Midline Cervical Cleft associated with a Bronchogenic Cyst. Int J Paed Otorhinolaryngology 1999;47:261-4.
The area of the defect eventually heals as widened scar and is often accompanied by a cord of subcutaneous tissue, which forms contracture of neck limiting its motion. 6 If the contracture is severe the chin may held in position of flexion.1 Other associated congenital anomalies include thyroglossal duct cyst, dermoid cyst, bronchogenic cysts and branchial anomalies.8,9,10 Associated stepedial fixation is not reported
Address of Correspondence: Sundaram Apartment 91, Sarat Chatterjee Road Barat Colony, Laketown Kolkata - 700089, India E-mail:
[email protected]
Indian Journal of Otolaryngology and Head and Neck Surgery Vol. 57, No. 1, January - March 2005
