Pediatric Surgery International

, Volume 25, Issue 9, pp 811–813

Congenital midline cervical cleft with an underlying bronchogenic like cyst

Authors

  • Srinivas Vure
    • Department of Paediatrics and Child HealthCanberra Hospital
  • Karl Pang
    • Department of MedicineUniversity of Glasgow
  • Lavinia Hallam
    • Department of PathologyThe Canberra Hospital
  • M. Lui
    • Department of PathologyThe Canberra Hospital
    • Department of Paediatrics and Child HealthCanberra Hospital
    • The Australian National University
Case Report

DOI: 10.1007/s00383-009-2419-6

Cite this article as:
Vure, S., Pang, K., Hallam, L. et al. Pediatr Surg Int (2009) 25: 811. doi:10.1007/s00383-009-2419-6

Abstract

Congenital midline cervical cleft (CMCC) is an uncommon malformation. We report a case of a baby girl aged 3 days with a CMCC associated with a cyst reported as a bronchogenic cyst (BC). The pathology is not specific. The association of BC and CMCC is extremely rare and only five cases have been found in the literature. We report our case and review the relevant literature.

Keywords

Midline cervical cleftBronchogenic cystBranchial arches

Introduction

Congenital midline cervical cleft (CMCC) is an uncommon malformation of the ventral neck, which is noticeable at birth. Surgical excision is performed for cosmetic reasons, prevention of cervical contractures and prevention of complications of BCs.

BCs are rare developmental anomalies of the tracheo-bronchial tree. They are uncommonly found in the neck, and fewer than 70 cases have been reported [1]. BCs have been described with CMCCs but are only identified on histological examination. We report an extremely rare case of a CMCC with an epithelial lined track reported as BC histologically.

Case report

A baby girl born at 39 weeks gestation by Caesarean section for failure to progress in labour was referred to the Paediatric Surgery Clinic at the age of 3 days with an obvious CMCC. No previous antenatal diagnosis was made. The cleft appeared to be floored by shiny, thin, pink membranous layer which is suggestive of mucosa or dysplastic skin. There was a small superior “hood” and an opening at the inferior end which had discharged clear fluid (Fig. 1). An ultrasound scan of the neck was performed at 3 months, which confirmed a normal thyroid gland, but showed a non-specific area of cystic change measuring 3 × 5 × 2 mm inferior to the cleft in the midline. This was not a thyro-glossal cyst on either imaging or clinical characteristics. We decided to perform a surgical excision of the CMCC and cyst.
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Fig. 1

Congenital midline cervical cleft before surgery

Surgery

Procedure

Cord paralysis has been noted previously in this condition [2], although it was not present in this case. Intubation for anaesthesia was graded as 2–3 in difficulty.

Lines of excision including Z-plasty incisions were marked on the skin, and dissection of skin and the associated tract proceeded in a routine manner. The inferior tract was followed to the anterior surface of the sternum and excised. The Z-plasty flaps were fashioned and are shown in the illustrations (Fig. 2a, b). The procedure was uneventful and later postoperative course has been likewise uneventful. Appearance of scars at 1-year follow-up is very satisfactory.
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Fig. 2

a Z-plasty flaps were mobilized, rotated. b Z-plasty skin closed and secured

Pathology

Two pieces of roughly ellipsoid shaped skin were submitted for histopathology. These comprised the pink skin ellipse shown in Fig. 1, and the track leading inferiorly from the punctum also seen in Fig. 1.

The sections of the tract showed a benign cyst lined partly by pseudostratified columnar epithelium and partly by stratified squamous epithelium. A few small groups of mucous glands were seen in the surrounding tissue. There was no cartilage or smooth muscle in association with the mucosa. The pathology was not specific, but was considered as a BC.

Discussion

CMCC is a rare malformation of the anterior neck. Despite many hypotheses, the aetiology is still unclear. However, it is likely due to the impaired or delayed midline fusion of the first or second branchial arches [3]. The fault in fusion may be related to underlying mechanical factors [4] and vascular anomalies resulting in ischemia and necrosis [5, 6]. Apart from cosmetic importance, unmanaged CMCC could result in infection and contractures, the midline cleft pulling down on the submental region of the jaw, and affecting growth and neck mobility.

BCs are congenital malformations of the ventral foregut. BCs are lined by columnar or cuboidal epithelium and are surrounded by tissues similar to the bronchus including mucous glands [7]. Between the third and sixth week the primitive foregut separates to form the ventral trachea and oesophagus [8]. Budding from the ventral foregut forms the bilateral tracheo-bronchial tree, and any disturbance in this development may result in anomalies including bronchogenic cysts. BCs have been described outside the embryonic field and are more difficult to explain embryologically in these locations. Abnormal buds could migrate and settle down in different intra- and extra-thoracic locations. Common locations are in the intrathoracic and intrapulmonary regions, and in the mediastinum. They are rarely found in the abdomen [9] and cervical area [10].

The association of a CMCC with a branchial or other type of cyst is unusual [11]. CMCC in the presence of a BC is extremely rare and only five cases have been found in the literature [10, 1215]. None of the patients in the previously reported cases had any significant cystic structures in association with a CMCC at presentation. Histological examination was essential to confirm the association of CMCC with BC in all the reported cases. Histology in our case revealed the presence of pseudostratified columnar epithelium with mucous glands surrounding the cyst suggesting a bronchogenic origin. This finding was similar to what the previous five reports found, however smooth muscle and cartilage adjacent to the midline cervical cleft were also reported in previous reports [1015].

The correlation between a CMCC and a BC is not clearly understood, although there are some embryological hypotheses to explain the dual pathology. French et al. hypothesised that a disturbance occurs at day 32 of gestation, when the head is in close proximity to the pericardial region [11]. The development of the dual anomalies may occur separately at the same time when the developing respiratory and digestive systems begin to separate. The delayed or impaired fusion of the branchial arches and the abnormal budding of the ventral foregut together produced a CMCC and a BC, respectively.

Mendis and Moss discussing the embryology in a 2007 paper [16], note that fewer than 100 cases of CMCC have been reported, and suggest that the cleft consists of combined branchial and bronchogenic components.

BCs are rare in adults and majority of the cases are diagnosed in the paediatric population. Surgical excision of the CMCC and BC is important not only to prevent skin contractures and poor cosmetic appearance, but also to prevent unpredictable complications of the malformation.

Although this case was reported as a bronchogenic cyst, the lack of cartilage and smooth muscle must leave this as a presumptive rather than an established diagnosis. At present we regard the embryology as an open question, and add the present case to the previously observed cases for consideration.

Copyright information

© Springer-Verlag 2009