Volume 4, Issue 3 , Pages 123-128, September 2009
Double opposing Z-plasty for congenital midline cervical web: A case report
Article Outline
Summary
Congenital midline cervical clefts (CMCCs), rare congenital anomalies of the cervical neck, are unaesthetic and can lead to secondary deformities of the mandible. Repair usually involves excision of the lesion followed by primary closure, W-plasty, or Z-plasty. We repaired a CMCC in a 4-year-old girl using double opposing Z-plasty of the subcutaneous tissues and platysma to increase the vertical length of the skin and form a deeper cervicomental angle. The surgery substantially improved appearance and function, producing a sharper cervicomental angle and reduction in cleft chin. Scarring was minimized with postsurgical treatments. However, because of the patient's age, an open-bite deformity remained. Therefore, we recommend surgical correction for CMCC in the first year of life.
Keywords: Cervical cleft, CMCC, Z-plasty
1. Introduction
Congenital midline cervical clefts (CMCCs) are a very rare congenital anomaly of the ventral neck thought to be caused by failure of the distal branchial arches to fuse in the midline. The characteristic clinical findings consist of webbing of the neck with a cutaneous protuberance, a subcutaneous fibrous cord, and a distal sinus tract. These anomalies are anatomically situated in the midline between the submental region and suprasternal notch [1], [2]. The treatment is complete excision with reconstruction of the defect by primary closure, W-plasties, or, most commonly, with single or multiple Z-plasties. The reconstruction is both cosmetic and functional, with the goal of deformity removal, reformation of the cervicomental angle, and prevention of sequelae from traction on the mandible. We present a case of congenital midline cervical web that we repaired using the double opposing Z-plasty technique, which uses multiple Z-plasties of the skin layer and an opposing Z-plasty of the subcutaneous tissue and platysma layer, rather than the more common single-layer Z-plasty closure. This technique, which was first described by Daw and Patel [3], has the intended benefit of improving the cervical mental contour. Here we provide step-by-step documentation of this technique.
2. Case report
A 4-year-old girl was referred to the Head and Neck Surgery Department, Kaiser Permanente Oakland Medical Center, for a midline cervical contracture that was present since birth. There was no history of cervical pain, infection, or drainage. An evaluation by a medical geneticist found no associated congenital abnormalities. The patient had been a full-term infant with a normal delivery and had no significant family or past medical history. On examination, the cranial vault, orbits, nose, and midface were normal in appearance. A ventral midline cervical cleft with a 1.5-cm dehiscence of the skin at the cervicomental junction was noted (Fig. 1A). A cervical sinus tract was present (Fig. 1D), with a blind pouch 1
cm proximal to the manubrium. No drainage was noted or expressed. A fibrous cord was palpable and visible from the inferoposterior mandibular symphysis to the manubrium (Fig. 1A). There was midline webbing with dorsiflexion of the head (Fig. 1B). The lesion did not change with swallowing or tongue protrusion. The chin was diminished, with the anterior extent deflecting inferiorly, creating an open-bite (Fig. 1C). There was a small palpable midline cleft of the mandible. There were no associated abnormalities of the laryngopharynx, lips, oral cavity, tongue, thyroid, or sternum. Cardiac and pulmonary examinations were normal.
Fig. 1.
Preoperative photographs. (A) An open-bite deformity and cleft chin. (B) Desquamating epithelium and fibrous chord. (C) Webbed neck with dorsiflexion of the head. (D) Lacrimal probe in sinus tract. S, superior and I, inferior.
3. Description of the procedure
The patient was positioned supine with a shoulder-roll and the neck extended. The borders of the fibrous band were marked out, and two separate Z-plasties were designed to incorporate the cutaneous defect (Fig. 2A). One was placed at the cervicomental angle, and the other was placed inferiorly; in both, the limbs were at approximately 60° from the vertical midline. The skin was incised vertically down to the layer of the platysma to encompass the cleft and any abnormal skin. A lacrimal duct probe was introduced into the sinus tract (Fig. 2B), which extended down to the level of the manubrium (Fig. 2C). The sinus tract was dissected out completely (Fig. 2D). The fibrous band was excised out in its entirety and the attachments were transected at their insertions to the mandible and sternum. In our patient, the fibrous band included a portion of the platysma. A supraplatysmal flap was raised, and a single opposing platysmal Z-plasty was designed and incised at the cervicomental angle (Fig. 3A). The limbs were made at 60° from the vertical midline. The triangles were transposed and sutured into the new position. The previously planned double Z-plasties were incised through the skin and subcutaneous tissues. Key stitches were placed after proper placement of the limbs. Subcutaneous interrupted 4–0 Vicryl sutures were used to reapproximate the skin (Fig. 3B). A dependant Penrose drain was placed. The skin was closed with fast-absorbing gut dermal stitches. A tension-free closure was achieved.
Fig. 2.
Intraoperative photographs before Z-plasty. (A) The skin incision was marked to encompass the abnormal skin, the sinus tract, and the fibrous cord. The first Z-plasty was planned at the cervicomental angle, and the second Z-plasty was planned inferior to the first. Both limbs were at approximately 60° from the vertical midline. (B) A lacrimal probe was placed in the sinus tract. (C) The sinus tract extended down to the level of the manubrium. (D) The sinus tract was completely dissected out. S, superior and I, inferior.
Fig. 3.
Z-plasty and histology. (A) A planned opposing Z-plasty of the underlying platysma and soft tissue. (B) The Z-plasties of the superficial layer have been appropriately placed and the skin closed. (C) The cleft was lined with keratinized squamous epithelium and the sinus tract with cuboidal mucosal epithelium. (D) A cross-section of the sinus tract, lined by cuboidal epithelium and surrounded with collagen bundles (fibrous band, stained blue). The platysma muscle (red bundles) was also involved in the lesion. S, superior and I, inferior.
Histologically, the cleft was lined with keratinized squamous epithelium. The sinus tract was lined with cuboidal epithelium and contained seromucinous glands (Fig. 3C) and was present within collagen bundles representing the fibrous band. The platysma muscle was involved in the lesion with localized fibrosis (Fig. 3D).
The patient had an uneventful postoperative recovery. She had some mild hypertrophic scarring that responded to local steroid injections and topical vitamin E massage. Five months after the operation, a raised erythematous scar was evident (Fig. 4A). At 6 months, the patient had a much improved cervicomental proportion. At 1 year, the cleft chin and open-bite deformity had showed improvement (Fig. 4B). The scar was flattened and showed decreased hyperemia (Fig. 4C), and the cervicomental angle was restored (Fig. 4D).
Fig. 4.
Postoperative photographs. (A) Five months after the operation, raised erythematous scar. (B)–(D) One year after the operation. (B) The cleft chin and open-bite deformity have improved. (C) The scar has flattened and shows decreased hyperemia. (D) Restored cervicomental angle.
4. Discussion
CMCC, also known as medial cleft, median fissure of the neck, congenital midline cervical cord, midline cervical webbing, and pterygium colli medianum [1], is often associated with a midline cervical web. There have been 22 published case reports in the literature on a Medline search between 1950 and 2008. The characteristic findings of a cervical cleft include a cephalic cutaneous protuberance, with a cleft covered by red, desquamating epithelium extending downwards along the midline between the chin and sternal notch. The caudal end of the cleft is often associated with a sinus or fistula tract opening, from which mucoid secretions may drain. A subcutaneous, cord-like, fibrous thickening is present, which may lead to webbing of the anterior neck, cervical contraction, and limited extension of the neck [2], [7], [8]. This fibrous band may cause traction on the growing mandible, leading to a submental bony prominence, mild cleft mandible, microgenia, retrogenia, or an open-bite deformity [1], [7]. Indeed, our patient had limited extension of the neck, a median cleft of the mandible, diminished chin, and open-bite deformity. A number of other head and neck anomalies associated with CMCC have been reported in the literature, including median clefts of the lower lip, chin, or tongue; hypoplasia of the hyoid or thyroid cartilage [2], [5]; and thyroglossal duct cysts and bronchogenic cysts [7]. Other defects associated with CMCC that are outside the head and neck region include cleft sternum, congenital cardiac anomalies, midline abdominal web, and midline hemangioma [2], [5].
The cephalic skin protuberance of CMCC generally contains normal epidermis and dermis with a core of interfasciculated skeletal muscle. The dermis of the cleft is made of thin, atrophic epidermis with no skin appendages. The underlying fibrous cord is associated with interfasciculated skeletal muscle bundles. It is not clear whether this is part of the underlying platysma or a distinct entity of the CMCC. The histology of the sinus tract in our patient was consistent with other reports that the tract is typically lined with pseudostratified columnar or cylindrical epithelium and often has adjacent seromucinous glands [9]. There have been a few reported cases in which the histology of the fistula resembled bronchogenic epithelium or contained part of a thyroglossal duct [1], [2], [5], [10].
Treatment of CMCC should occur early because of the unaesthetic appearance of the malformation and because it can lead to secondary deformities of the mandible. To prevent recurrence, the complete lesion must be excised, including the attenuated skin and underlying fibrous cord. Webbing of the neck and a decrease in the cervicomental angle are due both to the fibrous band and to a vertical deficiency of the adjacent soft tissue. Therefore, correcting the deformity requires both complete removal of the fibrous cord and a vertical lengthening of the overlying skin, achieved by a Z-plasty. Excising the cord also eliminates traction on the mandible, thereby decreasing the likelihood of microgenia and open-bite deformity. Linear closure results in recurrence of the contracture [11], [12]. A Z-plasty is the transposition of two opposing triangular skin flaps. The scar or contracture is lengthened, released of tension, and reoriented in direction. The degree of change in direction and lengthening depends on the flap angle. The ideal flap angle of 60° will rotate the scar 90° and provide a 75% increase in length. A 30° angle yields 25% increase in length, and a 45° angle yields 50% increase in length. Angles greater than 75° result in tissue distortion and dog-ear deformities, and angles less than 20° result in compromised blood flow and problems with flap viability [13]. The limbs should be equal in length to the central defect. The Z-plasty should be planned to rotate the scar so it lies within a natural skin crease and if possible placed at the cervicomental angle or hidden beneath the mandible [13]. A single Z-plasty should be used if the defect is ≤2cm. In a cleft under extreme tension and where gain in length is the primary concern, a four-flap Z-plasty can increase the vertical length 150%. In the handful of reported cases, the majority of surgeons repaired this lesion using multiple Z-plasties of the skin and subcutaneous tissues. A single paper, by Daw and Patel [3], reported the use of double opposing Z-plasties; a good outcome was achieved in that case. Theoretically, the involvement of the platysma with the fibrous cord could contribute to the web and deficient cervicomental angle. Making an opposing Z-plasty in the platysma increases the vertical length and forms a deeper cervicomental angle [2], [3], [5]. This technique was used in our patient with significant improvement in appearance and function. We used two serial Z-plasties of the skin in our patient, which allowed the upper horizontal scar to recreate the cervicomental angle and be hidden under the mandible. This also resulted in smaller limbs that are less apparent to the eye. Hypertrophic scarring in the oblique scar lines is not uncommon. The tissue should be handled as little as possible and the skin flaps should be adequately released, so there is no tension across the wound. The use of postoperative steroid injections and vitamin E can help soften and flatten the scar.
No consensus exists regarding the optimal surgical management of CMCCs. Although single layered Z-plasty repair of the cutaneous defect and soft tissue deficiency is widely reported, optimal results are limited by hypertrophic scarring and poor definition at the cervicomental angle. In our case, an opposing Z-plasty of the subcutaneous tissues and platysma helped to improve the cervicomental contour, and this method should be considered when planning surgical reconstruction. Atraumatic handling of the tissues and adjunctive measures to address hypertrophic scarring yielded satisfactory results, but delay in presenting for definitive surgery resulted in distortion of the mandible and an open-bite deformity that we were not able to reverse. We advocate that surgical correction be considered by 6–12 months of age.
References
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PII: S1871-4048(08)00069-5
doi:10.1016/j.pedex.2008.09.004
© 2008 Elsevier Ireland Ltd. All rights reserved.
Volume 4, Issue 3 , Pages 123-128, September 2009
