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Journal of Radiology Case Reports

Congenital Midline Cervical Cleft

Case Report

Javier Villanueva-Meyer1*, Christine Glastonbury1, Peter Marcovici1

Radiology Case. 2015 Mar; 9(3):7-11 :: DOI: 10.3941/jrcr.v9i3.2202

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1. Department of Radiology and Biomedical Imaging, University of California - San Francisco, San Francisco, California, USA

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  ABSTRACT
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Congenital midline cervical cleft is a rare anomaly that typically presents in the neonatal period as a thin suprasternal vertical band of erythematous skin with a nipple-like projection superiorly, which may exude fluid. We present the clinical and pathophysiologic features and the imaging findings of this uncommon, and rarely described entity in a newborn girl.








  CASE REPORT
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A large for gestational age female was born to a healthy gravida 6 para 5 woman at 41 weeks of gestation. The prenatal course was unremarkable and, although at birth the infant was found to have meconium stained amniotic fluid, there were no neonatal complications. She was transferred to our institution for further evaluation of a one centimeter vertically-oriented linear band of erythematous, atrophied skin in the suprasternal neck with a 2 mm nipple-like protrusion cranially and a blind-ending sinus tract caudally (Fig. 1 Preview this figure

Figure 1: Clinical image
A two-day old girl with congenital midline cervical cleft. FINDINGS: Clinical photograph of a 2-day old girl shows a band of thin, erythematous skin (black arrow) in the midline of the lower neck with a sinus tract caudally (white arrow).
). No other dysmorphic features were present and there was no family history of congenital abnormality or consanguinity.

Initial radiologic evaluation with a targeted ultrasound to the soft tissues of the neck demonstrated an apparently blind-ending midline suprasternal sinus tract arising from the subcutaneous soft tissues (Fig. 2 Preview this figure
Figure 2: Ultrasound
A two-day old girl with congenital midline cervical cleft. FINDINGS: Transverse US (A) and longitudinal color Doppler US (B) images demonstrate a blind-ending, caudally-oriented tract (white arrows, A) without associated vascularity. TECHNIQUE: Transverse and longitudinal scan planes of a neck US (A and B). Ultrasonography was performed using an 18 MHz linear transducer (GE Logiq).
).

Given the infant`s stridor on exam however, there was persistent concern for a fistulous connection of the sinus tract. Rigid bronchoscopy revealed an intact trachea without fistulous communication or evidence of tracheomalacia.

A targeted magnetic resonance imaging examination was performed at 3 tesla (Discovery, GE Healthcare, Waukesha, WI) including T2 with and without fat saturation and pre- and postgadolinium enhanced T1-weighted sequences. This demonstrated a T1 hypointense, T2 hyperintense tract with peripheral post-contrast enhancement at the base of the neck measuring 7 mm in craniocaudal length and 3 mm in width. This tract extended from the skin surface at the inferior aspect of the lesion to the superior cortex of the sternal manubrium in a caudal direction. There was no cortical disruption or involvement of the manubrium. Additionally, although the tract was noted to course toward the thyroid gland, the thyroid gland appeared separate and intact. There was no associated soft tissue mass or fluid collection (Fig. 3 Preview this figure
Figure 3: Magnetic Resonance Imaging
A two-day old girl with congenital midline cervical cleft. FINDINGS: Magnified sagittal T1-weighted (A), fat-saturated T2-weighted (B), and post-gadolinium fat-saturated T1-weighted (C) images show a T1 hypointense, T2 hyperintense, and peripherally-enhancing cranio-caudally oriented tract (white arrow). The tract courses from the skin in the midline at the base of the neck toward the manubrium of the sternum. TECHNIQUE: 3.0 Tesla MRI (GE Discovery) Image A: Sagittal T1 TR/TE - 600/8 ms, 1.5 mm slice thickness, noncontrast; Image B: Sagittal T2 TR/TE - 3000/62 ms, 1.5 mm slice thickness, noncontrast; Image C: Sagittal T1 TR/TE - 600/8 ms, 1.5 mm slice thickness, Gadolinium contrast 0.2 mL/kg.
). These MRI findings confirmed the diagnosis of a congenital midline cervical cleft.

The neonate continued to do well and, after thorough evaluation by pediatric surgery, was discharged home with scheduled outpatient follow-up for surgical management of the congenital midline cervical cleft.

  DISCUSSION
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Etiology and demographics
Congenital midline cervical cleft is a rare entity that was first described in the English surgical literature by Bailey in 1924. Subsequently there have been fewer than 50 case reports in the English pediatric surgical literature documenting this congenital anomaly [1]. To date this entity has not been reported in the radiologic literature. The exact incidence is unknown, however, it comprises up to 2% of all neck anomalies and appears to be more common in Caucasian females [2,3].

Although the embryology of the head and neck is thought to be well understood, the pathogenesis of congenital midline cervical clefts remains speculative. The most widely accepted explanation for the development of this anomaly is impaired fusion of the first or, more commonly, second branchial arches in the midline as well as improper interaction between the ectoderm and mesoderm [4]. In normal embryogenesis the branchial arches grow medially and merge cephalad to caudal. Prior to fusion, mesodermal tissue migrates between the arches and pushes ectoderm outward to flatten the ventral furrow [1,2]. In the setting of congenital midline cervical clefts some mechanical factor or vascular anomaly is thought to result in ischemia or necrosis and interfere with these normal processes. Other less accepted explanations include increased pressure in the cervical region from the developing pericardial roof, amniotic adhesions, or exteriorization of a thyroglossal duct cyst. There are no associated risk factors [5].

Histologic examination has revealed stratified keratinized squamous epithelium lacking skin appendages such as sweat or sebaceous glands. The dermis has alternating hypertrophy and atrophy and there is a mild lymphocytic inflammatory infiltrate in the subcutaneous tissues [1]. An associated sinus tract is lined by a pseudostratified epithelium with seromucinous salivary glands; however, there have been several reports of respiratory epithelium and bronchial glands associated with the sinus tract [2].

Clinical and imaging findings
The midline cervical cleft is usually detected at birth as a thin vertical band of erythematous skin with a nipple-like projection superiorly. This lesion can be found anywhere along the midline from the chin to the suprasternal notch. An associated blind-ending sinus tract in the caudal aspect may occasionally have minimal secretion. The midline cervical cleft heals by scarring and resultant fibrous cord formation that may lead to a neck contracture and eventually torticollis or micrognathia in early childhood [3]. Additionally, there have been reports of associated lower lip, mandible, chin, and tongue defects [1].

Prior to the widespread use of ultrasound and MRI, radiographs of the neck classically demonstrated bony spurring at the mandible in advanced cases due to traction from the underlying fibrous cord [4]. Ultrasound of the neck shows a non-vascular, blind-ending sinus tract from the skin surface. The thyroid gland is normal. MRI is helpful in defining the lesion and its course as well as to exclude other differential considerations such as thyroglossal duct cysts or branchial cleft anomalies. MRI usually demonstrates skin thinning and a peripherally-enhancing, T1 hypointense, and T2 hyperintense linear tract without disruption of any bony or cartilaginous structures or involvement of the thyroid gland.

Treatment and prognosis
Treatment is accomplished surgically, by resection of the skin tag and fibrotic tissue with closure of the soft tissue defect by Z-plasties. Although there is no consensus on age of treatment, surgical intervention is recommended early before scarring leads to contracture, neck webbing, or mandibular hypoplasia [1]. Multiple series have documented good outcomes, however, there have been reports of recurrence [3].

Differential diagnosis
Differential diagnosis includes thyroglossal duct cysts or branchial cleft anomalies. A thyroglossal duct cyst that, although a midline T2 hyperintense lesion that may have faint peripheral enhancement, characteristically appears cystic and it is typically much higher in the neck, being more midline when related to the hyoid and more lateral when in the infrahyoid neck. In addition, a thyroglossal duct cyst does not usually have an associated sinus tract unless it is superinfected [1,2,5]. 2nd or 3rd branchial cleft cysts or pyriform sinus fistulae are typically off midline in the lateral neck [6].

  TEACHING POINT
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The congenital midline cervical cleft is a rare entity that is usually diagnosed clinically, however, the radiologist may be asked to evaluate this anomaly to further characterize anatomy and exclude other differential considerations. Recognition of an anterior cervical midline skin defect with an underlying blind-ending sinus tract that is T1 hypointense, T2 hyperintense, and demonstrates peripheral contrast enhancement on MR allows early surgical intervention to prevent future complications.








  FIGURES
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Display figure 1 in original size

Figure 1: A two-day old girl with congenital midline cervical cleft. FINDINGS: Clinical photograph of a 2-day old girl shows a band of thin, erythematous skin (black arrow) in the midline of the lower neck with a sinus tract caudally (white arrow).

Figure 1: Clinical image (Open in original size)
A two-day old girl with congenital midline cervical cleft. FINDINGS: Clinical photograph of a 2-day old girl shows a band of thin, erythematous skin (black arrow) in the midline of the lower neck with a sinus tract caudally (white arrow).

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Display figure 2 in original size
Figure 2: A two-day old girl with congenital midline cervical cleft. FINDINGS: Transverse US (A) and longitudinal color Doppler US (B) images demonstrate a blind-ending, caudally-oriented tract (white arrows, A) without associated vascularity. TECHNIQUE: Transverse and longitudinal scan planes of a neck US (A and B). Ultrasonography was performed using an 18 MHz linear transducer (GE Logiq).

Figure 2: Ultrasound (Open in original size)
A two-day old girl with congenital midline cervical cleft. FINDINGS: Transverse US (A) and longitudinal color Doppler US (B) images demonstrate a blind-ending, caudally-oriented tract (white arrows, A) without associated vascularity. TECHNIQUE: Transverse and longitudinal scan planes of a neck US (A and B). Ultrasonography was performed using an 18 MHz linear transducer (GE Logiq).

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Display figure 3 in original size
Figure 3: A two-day old girl with congenital midline cervical cleft. FINDINGS: Magnified sagittal T1-weighted (A), fat-saturated T2-weighted (B), and post-gadolinium fat-saturated T1-weighted (C) images show a T1 hypointense, T2 hyperintense, and peripherally-enhancing cranio-caudally oriented tract (white arrow). The tract courses from the skin in the midline at the base of the neck toward the manubrium of the sternum. TECHNIQUE: 3.0 Tesla MRI (GE Discovery) Image A: Sagittal T1 TR/TE - 600/8 ms, 1.5 mm slice thickness, noncontrast; Image B: Sagittal T2 TR/TE - 3000/62 ms, 1.5 mm slice thickness, noncontrast; Image C: Sagittal T1 TR/TE - 600/8 ms, 1.5 mm slice thickness, Gadolinium contrast 0.2 mL/kg.


Figure 3: Magnetic Resonance Imaging (Open in original size)
A two-day old girl with congenital midline cervical cleft. FINDINGS: Magnified sagittal T1-weighted (A), fat-saturated T2-weighted (B), and post-gadolinium fat-saturated T1-weighted (C) images show a T1 hypointense, T2 hyperintense, and peripherally-enhancing cranio-caudally oriented tract (white arrow). The tract courses from the skin in the midline at the base of the neck toward the manubrium of the sternum. TECHNIQUE: 3.0 Tesla MRI (GE Discovery) Image A: Sagittal T1 TR/TE - 600/8 ms, 1.5 mm slice thickness, noncontrast; Image B: Sagittal T2 TR/TE - 3000/62 ms, 1.5 mm slice thickness, noncontrast; Image C: Sagittal T1 TR/TE - 600/8 ms, 1.5 mm slice thickness, Gadolinium contrast 0.2 mL/kg.


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Display figure 4 in original size
Figure 4: A two-day old girl with congenital midline cervical cleft. FINDINGS: Serial axial T1-weighted images (A-C) show a hypointense tract (white arrows) that enhances on corresponding axial post-gadolinium T1-weighted images with fat saturation (D-F). The tract extends cranio-caudally from the skin surface toward the manubrium of the sternum. TECHNIQUE: 3.0 Tesla MRI (GE Discovery) Images A, B, C: Axial T1 TR/TE - 600/8 ms, 1.5 mm slice thickness, noncontrast. Images D, E, F: Axial T1, TR/TE - 600/8 ms, 1.5 mm slice thickness, Gadolinium contrast 0.2 mL/kg.

Figure 4: Magnetic Resonance Imaging (Open in original size)
A two-day old girl with congenital midline cervical cleft. FINDINGS: Serial axial T1-weighted images (A-C) show a hypointense tract (white arrows) that enhances on corresponding axial post-gadolinium T1-weighted images with fat saturation (D-F). The tract extends cranio-caudally from the skin surface toward the manubrium of the sternum. TECHNIQUE: 3.0 Tesla MRI (GE Discovery) Images A, B, C: Axial T1 TR/TE - 600/8 ms, 1.5 mm slice thickness, noncontrast. Images D, E, F: Axial T1, TR/TE - 600/8 ms, 1.5 mm slice thickness, Gadolinium contrast 0.2 mL/kg.

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Display figure 5 in original size
Figure 5: Summary table for congenital midline cervical cleft.

Figure 5: Table (Open in original size)
Summary table for congenital midline cervical cleft.

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Display figure 6 in original size
Figure 6: Differential diagnosis table for congenital midline cervical cleft.

Figure 6: Table (Open in original size)
Differential diagnosis table for congenital midline cervical cleft.

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  REFERENCES
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1. Kakodkar K, Patel S, Maddalozzo J. Congenital Midline Cervical Cleft. Otolaryngology 2013;03(02)
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2. Agag R, Sacks J, Silver L. Congenital midline cervical cleft. Cleft Palate Craniofac J 2007;44(1):98-101 Get full text
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3. King J, Patel RV, Huddart SN. Congenital midline cervical cleft. Journal of Pediatric Surgery Case Reports 2013;1(5):99-101 Available at: www.jpscasereports.com/article/S2213-5766(13)00039-0/pdf
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4. Van der Staak F, Pruszczynski M, Severijnen R, Van de Kaa CA, Festen C. The midline cervical cleft. Journal of pediatric surgery 1991;26(12):1391-1393 Get full text
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5. Mlynarek A, Hagr A, Tewfik TL, Nguyen VH. Congenital mid-line cervical cleft: case report and review of literature. International Journal of Pediatric Otorhinolaryngology 2003;67(11):1243-1249 Get full text
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6. LaRiviere CA, Waldhausen JH. Congenital Cervical Cysts, Sinuses, and Fistulae in Pediatric Surgery. Surgical Clinics of NA 2012;92(3):583-597 Get full text
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  ABBREVIATIONS
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CT = computed tomography
MRI = magnetic resonance imaging
TE = echo time
TR = repetition time
US = ultrasound









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