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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 8  |  Issue : 3  |  Page : 178-179

Congenital midline cervical cleft in a neonate born to diabetic mother – An unusual association


1 Department of Pediatrics, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India
2 Department of Neonatology, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India

Date of Web Publication6-Aug-2019

Correspondence Address:
Dr. Ashish Jain
Department of Neonatology, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcn.JCN_11_19

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  Abstract 


Congenital midline cervical cleft is a rare developmental anomaly of the neck region with only 200 cases reported till date. This case report highlights an unusual association of congenital midline cervical cleft and maternal diabetes. We report a neonate born to a mother with uncontrolled type 2 diabetes who presented with a midline neck lesion characterized by a proximal skin tag, middle mucosal surface and a distal blind sinus causing micro retrognathia with no other visible malformation. Most of the cases reported till date have a sporadic occurrence. This is the first possible report of this anomaly in a neonate of diabetic mother. This raises a possibility of being a part of diabetic embryopathy, however occurrence by chance cannot be completely ruled out.

Keywords: Branchial arch anomaly, midline cervical cleft, neonate of diabetic mother


How to cite this article:
Sait H, Jain A, Kapoor S. Congenital midline cervical cleft in a neonate born to diabetic mother – An unusual association. J Clin Neonatol 2019;8:178-9

How to cite this URL:
Sait H, Jain A, Kapoor S. Congenital midline cervical cleft in a neonate born to diabetic mother – An unusual association. J Clin Neonatol [serial online] 2019 [cited 2019 Oct 22];8:178-9. Available from: http://www.jcnonweb.com/text.asp?2019/8/3/178/264032




  Introduction Top


Congenital midline cervical cleft (CMCC) is a very rare congenital malformation involving the anterior region of the neck.[1] This condition was first reported by Luschka (1848) and subsequently documented by Bailey (1924) following which only 200 cases have been reported in literature. It is diagnosed clinically by the presence of a nipple-like projection formed by a hood of skin superiorly and fibrous or chord covered by reddened epithelium in the middle and inferiorly located sinus. Embryologically, it is postulated to be an impaired fusion of distal branchial arches in the midline.[2] Previous studies[3],[4] have reported branchial arch anomalies such as preauricular tags and branchial cleft cyst in neonates with diabetic embryopathy. However, the occurrence of CMCC in a neonate born to diabetic mother has not yet been reported.


  Case Report Top


Our case presented at birth with midline cervical defect. A 34+6-week-old preterm male, an outcome of a third-degree consanguineous marriage and spontaneous conception, was born vaginally to a third gravida mother. He weighed 3375 g at birth with a head circumference of 33 cm (50th–90th percentile). He was born through clear liquor with Apgar scores of 9 at 1, 5, and 10 min. The first sibling was also a preterm and expired at 6 months of age. The mother was not booked in our institution and had uncontrolled type 2 diabetes for the past 3 years. However, the mother denied taking any medication for the same in the past. In addition, the mother also had hypothyroidism for the past 5 years and was on Eltroxin 100 μg daily. The rest of the family history was insignificant.

On examination at birth, the child was plethoric and had a hairy pinna. Neck examination revealed a hood of skin in the upper part and reddened mucosa in the middle leading to a sinus in the lower part causing apparent microretrognathia with no discharge [Figure 1]. There was no airway or feeding difficulty, and the cry was normal. Examination of rest of the neck, face, and oral cavity revealed no other sinus/cleft. Examination of the cardiovascular system was normal. An ultrasound of the neck revealed no other associated abnormalities. Pediatric surgery referral was taken, and the neonate is awaiting a Z-plasty repair.
Figure 1: The characteristic three components of congenital midline cervical cleft – nipple-like skin tag at the cephalic end, a midline longitudinal mucosal surface, and a distal sinus

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  Discussion Top


CMCC being a rare anomaly constitutes 2% of the congenital cervical malformations.[5] It is sporadic in nature, and it is more commonly found in females.[6] In our case, the neonate was a male. Scanty serous discharge is usually present which ceases over time.[7] While many theories have been postulated, impaired midline fusion of the first and second branchial arches is most accepted.[8] It is associated with microgenia, bifid mandible, thyroglossal duct cyst, and ectopic bronchogenic cyst.[9] A thorough search of these associated lesions is mandatory in a patient with CMCC. In addition to cosmetic abnormality, secondary complications such as neck contracture resulting in torticollis and micrognathia and sternal growth abnormalities warrant early correction of this condition.[10]

Children born to mothers with diabetes have a two- to four-fold increase in the risk of congenital malformations (Pederson et al. 1964). Grix et al.[3] postulated an increased occurrence of branchial arch anomalies in children of diabetic mothers. This was later supported by Johnson and Fineman[4] in their report on two children born to diabetic mothers, where both of them had preauricular tags. However, there is a lack of literature stating the association of CMCC with diabetic embryopathy. Hence, this case report presents a rare occurrence of CMCC in neonates born to diabetic mothers.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Franzese C, Hayes JD, Nichols K. Congenital midline cervical cleft: A report of two cases. Ear Nose Throat J 2008;87:166-8.  Back to cited text no. 1
    
2.
Kara CO, Kara IG. Congenital midline cervical cleft. Otolaryngol Head Neck Surg 2006;135:953-4.  Back to cited text no. 2
    
3.
Grix A Jr., Curry C, Hall BD. Patterns of multiple malformations in infants of diabetic mothers. Birth Defects Orig Artic Ser 1982;18:55-77.  Back to cited text no. 3
    
4.
Johnson JP, Fineman RM. Branchial arch malformations in infants of diabetic mothers: Two case reports and a review. Am J Med Genet 1982;13:125-30.  Back to cited text no. 4
    
5.
Derbez R, Nicollas R, Roman S, Estève A, Triglia JM. Congenital midline cervical cleft of the neck: A series of five cases. Int J Pediatr Otorhinolaryngol 2004;68:1215-9.  Back to cited text no. 5
    
6.
Gardner RO, Moss AL. The congenital cervical midline cleft. Case report and review of literature. Br J Plast Surg 2005;58:399-403.  Back to cited text no. 6
    
7.
Bajaj Y, Dunaway D, Hartley BE. Surgical approach for congenital midline cervical cleft. J Laryngol Otol 2004;118:566-9.  Back to cited text no. 7
    
8.
Saha VP, Pal S, Saha S, Misra S. Congenital midline cervical cleft: A 7 year prospective study. Indian J Otolaryngol Head Neck Surg 2013;65:367-70.  Back to cited text no. 8
    
9.
Erçöçen AR, Yilmaz S, Aker H. Congenital midline cervical cleft: Case report and review. J Oral Maxillofac Surg 2002;60:580-5.  Back to cited text no. 9
    
10.
Eastlack JP, Howard RM, Frieden IJ. Congenital midline cervical cleft: Case report and review of the English language literature. Pediatr Dermatol 2000;17:118-22.  Back to cited text no. 10
    


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