- Email: [email protected]
Surgical management of midline cervical cleft
International Journal of Pediatric Otorhinolaryngology 127 (2019) 109657
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Surgical management of midline cervical cleft a,c
Jill N. D'Souza , Taher Valika a b c
a,b,∗
, John Maddalozzo
T
a,b
Division of Otolaryngology, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 East Chicago Avenue, Chicago, IL, 60611, USA Department of Otolaryngology, Feinberg School of Medicine, Northwestern University, USA Depart of Otolaryngology, Texas Children's Hospital, 17580 I-45 South, Suite WM410, The Woodlands, TX, 77384, USA
A R T I C LE I N FO
A B S T R A C T
Keywords: Midline cervical cleft Recurrence Z-plasty
Introduction: Midline Cervical Cleft (MCC) is a midline cervical congenital anomaly that manifests as a vertical cutaneous/subcutaneous defect with abnormal dermal elements as well as an underlying fibrous cord that extends from the sternum to the mentum of the mandible, which can lead to "wry neck" and hypoplastic mandible. The goal of surgical correction of MCC is to provide adequate healthy tissue coverage, as well as restore contour of the anterior neck. The primary treatment modality for midline cervical cleft is surgical. We describe a technique involving complete excision of the fibrous cord, and use of double z-plasty flap in order to create a tension-free closure and restore contour to the anterior neck. Methods: Using a database search method, children with the clinical diagnosis of midline cervical cleft treated between 2006 and 2016 were identified at a pediatric tertiary care center. Chart review was completed to assess for age at surgery, follow up, results, and complications. Results: 12 patients were identified in the Lurie Children's Hospital (LCH) database. 8 patients underwent complete cord excision by the seniorauthor using the double z-plasty (DZ) technique for closure, with no recurrences. 4 patients underwent linear closure by another surgeon, had persistent contracture, and underwent revision using the DZ technique by the senior author, with no recurrence. Average age of surgery was 9.5 months. Most common post op complication was hypertrophic scar (3/12). Recurrence was only seen in the linear closure cases (4/12). Conclusions: Midline Cervical Cleft is a rare entity with less than 200 cases in the literature. We believe the double z-plasty closure and complete excision of the fibrous cord results in reproducible restoration of neck contour and prevents cord recurrence, and should be considered the standard method for surgical excision of MCC.
1. Introduction
mandible to the sternum. There may be an associated draining sinus or conglomeration of abnormal skin located at the inferior or superior aspect of the cutaneous defect. Both the paucity of anterior skin and the fibrous cord contribute to anterior neck contracture or “wry neck” (Fig. 1). It can be associated with inferior draining sinus, though this is not necessary for diagnosis. The embryologic development of this lesion is debated, with most authors favoring failure of branchial arch closure during embryogenesis [2–11]. However, bronchogenic origin has also been suggested [1,12], with no definitive histologic determination at time of this writing. Management of MCC once diagnosed is primarily surgical. The purpose of surgery is to correct the contracture and provide adequate skin coverage to the anterior neck. There has been some debate regarding linear closure versus z-plasty closure [3,12–15]. The purpose of this discourse is to describe a direct and reproducible technique to
Midline cervical cleft (MCC) is a rare diagnosis in the pediatric population. While a clinically distinctive entity, its rarity may lend to some diagnostic challenges by primary care providers who initially encounter this in the neonate or infant, and it may be misdiagnosed as thyroglossal duct cyst, dermoid cyst, or branchial cleft anomaly. A 2015 review by Puscas [1] is the largest series in English language literature to date, and reported approximately 200 cases known worldwide; its incidence has been estimated at 1.7% of congenital anterior neck defects [2], however, due to varying nomenclature and description of these lesions, it is uncertain what the true incidence is. MCC is a midline fusion defect that presents as vertical cutaneous defect with either an absence of, or atrophic, skin. This is always associated with a fibrous band that connects the posterior aspect of the
∗
Corresponding author. Division of Otolaryngology, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 East Chicago Avenue, Chicago, IL, 60611, USA. E-mail addresses: [email protected] (J.N. D'Souza), [email protected] (T. Valika), [email protected] (J. Maddalozzo).
https://doi.org/10.1016/j.ijporl.2019.109657 Received 2 July 2019; Received in revised form 20 August 2019; Accepted 21 August 2019 Available online 23 August 2019 0165-5876/ © 2019 Elsevier B.V. All rights reserved.
International Journal of Pediatric Otorhinolaryngology 127 (2019) 109657
J.N. D'Souza, et al.
2.4. Surgical technique The surgical approach (Figs. 2–4) requires complete excision of the cord and associated fibrous cord with double Z-plasty repair to restore anterior neck contour: 1. The surgical approach requires complete excision of the cutaneous defect. This should be from the approximate level of the hyoid to the sternal notch (around 7–10 cm) 2. After appropriate undermining the fibrous cord is excised from the mentum to sternal notch. 3. The line of excision (vertical/anterior neck), is then horizontally divided at the approximate midline and a double Z-plasty is planned and executed. 4. The limbs of the Z-plasty should be designed about the midpoint of the cutaneous defect. The limbs should be adequate in length for repair and lack of tension, approximately 3 cm in length, leading to effortless transposition and alignment in the anterior neck. 5. Penrose drain placed at inferior edge of wound in all cases
Fig. 1. Illustration defining a midline cervical cleft. The lateral view reveals the skin contracture as a result.
3. Results
correct MCC, involving the use of a double z-plasty flap, in order to create a tension-free closure, restoring contour to the anterior neck, and preventing contracture.
There were no recurrences using the double Z-plasty technique as executed using the design and the methods described. The four cases which required reoperation had not undergone the Z-plasty technique. These patients had no evidence of contracture after excision of cord and closure using double z-plasty technique. The average age of patients at diagnosis was within the first month of life. All children underwent ultrasound of the neck prior to surgery. Surgical intervention was completed at an average age of 9.5 months. The most commonly reported complication was hypertrophic scar (3/ 12). Small wound dehiscence was noted in one case. None of these required return to OR. All cases had excellent cosmetic outcome with restoration of anterior neck contour. Post op follow up was on average 6 weeks after surgery, with the longest follow up date 7 months postoperative (Fig. 5).
2. Materials and methods 2.1. Study design and setting Using a database search method, children with the clinical diagnosis of midline cervical cleft treated between 2006 and 2016 were identified. Database search was undertaken at Lurie Children's Hospital, a tertiary care pediatric referral center.
2.2. Study participants
4. Discussion
12 individual patients were identified in the Lurie Children's Hospital (LCH) database through database search and chart review (Table 1). Of the 12 patients identified, 8 were completed by the senior author using the double z-plasty closure. Two patients underwent linear excision and closure by another surgeon at LCH. The remaining two were completed at other institutions, without a z-plasty closure.
MCC is an extremely rare anomaly of the anterior cervical neck seen in the pediatric population. This is present from birth, though frequently not recognized at that time. Its exact incidence is unknown, though a 1985 review by Gargan found they comprised 1.7% of congenital anterior neck lesions [16]. MCC is thought to occur through the failure of branchial arch fusion. In normal embryologic development, fusion of the arches occurs in a cephalad to caudal direction beginning in the fourth week of gestation, with failure at any point resulting in a midline defect. During fusion, mesodermal elements migrate between the arches, and flatten ventral furrow by exteriorizing ectodermal tissue. In the case of MCC, a failure of the first (mandibular) or second
2.3. Outcome measures Main outcome measure was recurrence of MCC. Secondary measures included return to operating room, restoration of anterior neck contour, and cosmesis. Table 1 Characteristics/outcomes of patients undergoing MCC repair. Patient
Age at Surgery (months)
Recurrence
Prior Surgery at OSH
Need for Revision
Post Op Complications
1 2 3 4 5 7 7 8 9 10 11 12
5 9 13 11 5 5 9 9 13 10 13 13
no no yes no no yes no no unknown no yes no
no no yes no no yes no no no no no no
no yes yes no no yes no no unknown no yes no
hypertrophic scar hypertrophic scar none none none none none wound dehiscence unknown none hypertrophic scar none
2
International Journal of Pediatric Otorhinolaryngology 127 (2019) 109657
J.N. D'Souza, et al.
Fig. 2. Double z-plasty closure with long limb technique.
Fig. 3. Intraoperative double z plasty design planning. Fig. 5. Midline cervical cleft. A,B) Preoperative view of fibr.
fibrous cord that extends from the sternum to the mentum of the mandible; further illustrating the midline fusion abnormality. It is often associated with an upper nipple-like projection, and an inferior sinus, which may drain mucoid fluid. These elements are not, however, necessary for diagnosis. Presence of fibrous band with resultant neck contracture should encourage the provider to consider diagnosis of MCC. Once diagnosis of MCC is made, evaluation for the presence of other midline defects should be undertaken. Cleft of the tongue, lip, and mandible have been described, as well as thyroglossal duct, bronchogenic, and dermoid cysts [1,2,7]. One case of congenital heart defect with MCC has been described [4]. Several imaging modalities are suggested for this purpose. Prior to the advent of modern imaging techniques, x-ray evaluation of the neck was undertaken, which classically demonstrated bony spurring at the mandible in advanced cases, thought to be due to traction from the fibrous tract [6]. More commonly, ultrasound or magnetic resonance imaging (MRI) of the neck are used for characterization of the lesion and exclusion of concurrent lesions. Ultrasound of the neck typically shows an avascular, blind-ending sinus tract from the skin surface, along with fibrous band contained within the dermis [6,8]. MRI of the neck typically demonstrates thinning of the epidermis, and a linear T1 hypointense/T2 hyperintense tract, without disruption of underlying bony or cartilaginous structures [6]. It is our practice to use ultrasound exclusively during initial diagnosis, with MRI reserved for cases where associated lesions are identified during screening ultrasound. After completion of imaging studies, the question of management of the lesion arises. The natural history of MCC is that it grows commensurate with the child, and lengthening and increased prominence of the cord occurs as the neck elongates [1]. Limitation of extension may occur, and obliteration of normal cervicomental angle can be seen. Early intervention is recommended in order to prevent long-term
Fig. 4. Intraoperative double z plasty closure.
(hyoid) arches can lead to persistent midline furrow, and subsequent fibrous cord formation, with or without inferior sinus tract [2]. Other hypotheses include exteriorization of thyroglossal duct remnant, or bronchogenic origin [12], however our senior author (JM) believes that failure of branchial arch fusion is the most likely etiology. Histologic findings are generally unremarkable, with the most consistent finding being absence of epithelial adnexa in the dermis, fibrosis replacing superficial musculature, and areas of atrophy/hypertrophy of the dermis along with dyskeratosis [2]. MCC is present at birth, though its diagnosis may not be made until early infancy and neck mobility is noted to be impaired. It is recognized as a cutaneous/subcutaneous defect that classically is represented by complete absence of dermal tissue or dysmorphic skin oriented vertically and of varying length. It is also associated with an underlying 3
International Journal of Pediatric Otorhinolaryngology 127 (2019) 109657
J.N. D'Souza, et al.
neonate or infant presenting with midline fibrous cord connecting submentum to posterior/inferior aspect of mandible. Once this diagnosis is made, complete surgical excision of the fibrous cord should be undertaken by one year of age, with use of double z-plasty closure technique in order to reproducibly minimize recurrence as well as provide optimal cosmetic outcome with restoration of anterior neck contour.
contracture of the neck as well as hypoplasia of the mandible. We advocate for early intervention, ideally before one year old, to prevent mandibular and contracture issues. Additionally, the fibrous cord has not yet matured into a mature scar at this age. Management of MCC is primarily surgical, and multiple techniques have been described. All authors agree that complete excision of the lesion is essential, as mere transection of the cord frequently results in recurrence and persistent contracture [1]. Debate exists regarding skin closure [1,14]. Some authors advocate for use of linear closure, particularly in early infancy when fibrous cord has not yet transformed into mature scar [2,3]. A linear closure is feasible when the cutaneous lesion is small and the adjacent tissue plentiful, allowing for a lax linear closure. This still requires wide undermining with dissection and excision of the fibrous band to prevent a recurrence. As most patients are now managed surgically beyond the stages of early infancy, a linear closure is not a reasonable option. Other reports describe varying techniques with W- and Z-plasty [1,6,8,13,15]. In a previous article by senior author (JM), consideration of linear excision and closure had been given to small defects with lax adjacent tissues [2]. However, in this series, recurrence with persistent contracture was demonstrated in four infants that had undergone linear excision. The purpose of this article is to describe our use of the double z-plasty with long limb technique for prevention of contracture and restoration of anterior contour of the neck. Z-plasty is a common technique and versatile surgical maneuver, allowing the surgeon to lengthen a contracted scar, reorient scar direction, break up a straight line, and, essentially, shift soft tissue contour [5]. The double z-plasty technique involves creation of two triangular transposition flaps that are rotated optimally to close a central defect. Use of the z-plasty allows a gain of 75% length with optimal design using an angle of 60°. More obtuse angles have demonstrated tissue distortion and formation of dog-ear deformity, while angles less than 20° present viability concerns due to impaired blood supply. In our experience, we have found the double z-plasty to be optimal as it allows for optimal tissue rotation and viability. The danger with a multiple z-plasty is not making, or not being able to make the limbs of the Z long enough. This has the potential to compromise the extra tissue mobilized to correct the contracture. It has been the observation that when multiple Z-plasty is employed (> 2 limbs), that the size of the flaps is inadequate to mobilize enough tissue to correct the neck contracture. Additionally, the double z-plasty allows the limbs to fall into the natural skin crease. The single z-plasty does not always lend itself to this. The most common complication encountered with our technique was hypertrophic scarring. In our experience, this can be ascribed to the nature of the defect, in which it represents an open lesion and therefore likely has a subclinical infection. The lesion also possesses ectodermal and mesodermal components which predispose to scar formation (fibrous cord). Although the Z-plasties were closed without tension, there also may be a tensile component to the scar formation secondary to mobility and stretching of the neck after excision. Proper tissue handling techniques and wide undermining provide the opportunity to optimize post-surgical healing. The novelty of our technique is the consistent use of double z-plasty with long limb length, in order to provide tension free closure (Fig. 2). The study is the largest utilizing the double z-plasty with no evidence of recurrence. Thus, use of the double z-plasty technique using the guidelines we provide allows for optimal results. This technique is a reproducible and reliable technique to prevent recurrence of the cord, provide excellent cosmetic outcome, and should be considered the standard surgical method for management of MCC.
Financial disclosure The authors have no financial relationships relevant to this article to disclose. Funding source No external funding support. Conflicts of interest The authors have no conflicts of interest relevant to this article to disclose. Statement of ethics Research was approved by Ann & Robert H. Lurie Children's Hospital of Chicago Institutional Review Board and conducted in an ethical manner with respect to patient data and privacy. Appendix A. Supplementary data Supplementary data to this article can be found online at https:// doi.org/10.1016/j.ijporl.2019.109657. References [1] L. Puscas, Midline cervical cleft: review of an uncommon entity, nt. J. Pediatr. Otolaryngol. (2015), https://doi.org/10.1155/2015/209418 2015:209418. [2] J. Maddalozzo, A. Frankel, L. Holinger, Midline cervical cleft, Pediatrics 92 (2) (1993) 286–287. [3] X. Sinopidis, H. Kourea, A. Panagidis, V. Alexopoulos, S. Tzifas, G. Dimitriou, G. Georgiou, Congenital midline cervical cleft: diagnosis, pathologic findings, and early state treatment, Case Rep. Pediatr. 1 (2012) 951040. [4] S. Hirokawa, H. Uotani, H. Okami, K. Tsukada, T. Futatani, I. Hashimoto, A case of congenital midline cervical cleft with congenital heart disease, J. Pediatr. Surg. 38 (7) (2003) 1099–1101. [5] K. Kadokhar, S. Patel, J. Maddalozzo, Congenital Midline Cervical Cleft, Otolaryngology 3 (2013) 132, https://doi.org/10.4172/2161-119X. 1000132. [6] J. Villanueva-Meyer, C. Glastonbury, P. Marcovici, Congenital midline cervical cleft, J. Radiol. Case Rep. 9 (3) (2015) 7–11. [7] B. Crippa, M. Bedeschi, G. Cantarella, L. Colombo, V. Agosti, I. Amodeo, M. Fumagalli, I. Mazzola, F. Mosca, Congenital. Anom. 55 (2015) 112–115. [8] R. Goldfisher, P. Bawa, Z. Ibrahim, J. Amodio, Clinical and imaging features of a congenital midline cervical cleft in a neonate: a rare anomaly, Case Rep. Pediatr. (2015), https://doi.org/10.1155/2015/439596 2015:439596. [9] R. Smith, P. Barth, J. Castillo, B. Millman, W. Wood, Congenital midline cervical cleft: a report of 3 cases, Ear Nose Throat J. 85 (2) (2006) 119–120. [10] C. McInnes, A. Benson, C. Verchere, J. Ludemann, J. Arneja, Management of congenital midline cervical cleft, J. Craniofac. Surg. 23 (1) (2012) e36–38. [11] D. Mendis, A. Moss, Case series: variations in the embryology of congenital midline cervical clefts, Acta Chir. Plast. 49 (3) (2007) 71–74. [12] Y. Bajaj, D. Dunaway, B. Hartley, Surgical approach for congenital midline cervical cleft, J. Laryngol. Otol. 118 (2004) 566–568. [13] J. Daw, P. Patel, Double-opposing Z-plasty for correction of midline cervical web, J. Craniofac. Surg. 14 (5) (2003) 774–777. [14] S. Achard, X. Leroy, P. Fayoux, Congenital midline cervical cleft: a retrospective case series of 8 children, nt. J. Pediatr. Otolaryngol. 81 (2016) 60–64. [15] C. Cochran, R. DeFatta, A. Brenski, Congenital midline cervical cleft: a practical approach to Z-plasty closure, Int. J. Pediatr. Otorhinolaryngol. 70 (3) (2006) 553–559. [16] T. Gargan, M. McKinnon, J. Mulliken, Midline cervical cleft, Plast. Reconstr. Surg. 76 (1985) 223–229.
5. Conclusion Diagnosis of midline cervical cleft should be considered in any
4
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Surgical management of midline cervical cleft a,c
Jill N. D'Souza , Taher Valika a b c
a,b,∗
, John Maddalozzo
T
a,b
Division of Otolaryngology, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 East Chicago Avenue, Chicago, IL, 60611, USA Department of Otolaryngology, Feinberg School of Medicine, Northwestern University, USA Depart of Otolaryngology, Texas Children's Hospital, 17580 I-45 South, Suite WM410, The Woodlands, TX, 77384, USA
A R T I C LE I N FO
A B S T R A C T
Keywords: Midline cervical cleft Recurrence Z-plasty
Introduction: Midline Cervical Cleft (MCC) is a midline cervical congenital anomaly that manifests as a vertical cutaneous/subcutaneous defect with abnormal dermal elements as well as an underlying fibrous cord that extends from the sternum to the mentum of the mandible, which can lead to "wry neck" and hypoplastic mandible. The goal of surgical correction of MCC is to provide adequate healthy tissue coverage, as well as restore contour of the anterior neck. The primary treatment modality for midline cervical cleft is surgical. We describe a technique involving complete excision of the fibrous cord, and use of double z-plasty flap in order to create a tension-free closure and restore contour to the anterior neck. Methods: Using a database search method, children with the clinical diagnosis of midline cervical cleft treated between 2006 and 2016 were identified at a pediatric tertiary care center. Chart review was completed to assess for age at surgery, follow up, results, and complications. Results: 12 patients were identified in the Lurie Children's Hospital (LCH) database. 8 patients underwent complete cord excision by the seniorauthor using the double z-plasty (DZ) technique for closure, with no recurrences. 4 patients underwent linear closure by another surgeon, had persistent contracture, and underwent revision using the DZ technique by the senior author, with no recurrence. Average age of surgery was 9.5 months. Most common post op complication was hypertrophic scar (3/12). Recurrence was only seen in the linear closure cases (4/12). Conclusions: Midline Cervical Cleft is a rare entity with less than 200 cases in the literature. We believe the double z-plasty closure and complete excision of the fibrous cord results in reproducible restoration of neck contour and prevents cord recurrence, and should be considered the standard method for surgical excision of MCC.
1. Introduction
mandible to the sternum. There may be an associated draining sinus or conglomeration of abnormal skin located at the inferior or superior aspect of the cutaneous defect. Both the paucity of anterior skin and the fibrous cord contribute to anterior neck contracture or “wry neck” (Fig. 1). It can be associated with inferior draining sinus, though this is not necessary for diagnosis. The embryologic development of this lesion is debated, with most authors favoring failure of branchial arch closure during embryogenesis [2–11]. However, bronchogenic origin has also been suggested [1,12], with no definitive histologic determination at time of this writing. Management of MCC once diagnosed is primarily surgical. The purpose of surgery is to correct the contracture and provide adequate skin coverage to the anterior neck. There has been some debate regarding linear closure versus z-plasty closure [3,12–15]. The purpose of this discourse is to describe a direct and reproducible technique to
Midline cervical cleft (MCC) is a rare diagnosis in the pediatric population. While a clinically distinctive entity, its rarity may lend to some diagnostic challenges by primary care providers who initially encounter this in the neonate or infant, and it may be misdiagnosed as thyroglossal duct cyst, dermoid cyst, or branchial cleft anomaly. A 2015 review by Puscas [1] is the largest series in English language literature to date, and reported approximately 200 cases known worldwide; its incidence has been estimated at 1.7% of congenital anterior neck defects [2], however, due to varying nomenclature and description of these lesions, it is uncertain what the true incidence is. MCC is a midline fusion defect that presents as vertical cutaneous defect with either an absence of, or atrophic, skin. This is always associated with a fibrous band that connects the posterior aspect of the
∗
Corresponding author. Division of Otolaryngology, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 East Chicago Avenue, Chicago, IL, 60611, USA. E-mail addresses: [email protected] (J.N. D'Souza), [email protected] (T. Valika), [email protected] (J. Maddalozzo).
https://doi.org/10.1016/j.ijporl.2019.109657 Received 2 July 2019; Received in revised form 20 August 2019; Accepted 21 August 2019 Available online 23 August 2019 0165-5876/ © 2019 Elsevier B.V. All rights reserved.
International Journal of Pediatric Otorhinolaryngology 127 (2019) 109657
J.N. D'Souza, et al.
2.4. Surgical technique The surgical approach (Figs. 2–4) requires complete excision of the cord and associated fibrous cord with double Z-plasty repair to restore anterior neck contour: 1. The surgical approach requires complete excision of the cutaneous defect. This should be from the approximate level of the hyoid to the sternal notch (around 7–10 cm) 2. After appropriate undermining the fibrous cord is excised from the mentum to sternal notch. 3. The line of excision (vertical/anterior neck), is then horizontally divided at the approximate midline and a double Z-plasty is planned and executed. 4. The limbs of the Z-plasty should be designed about the midpoint of the cutaneous defect. The limbs should be adequate in length for repair and lack of tension, approximately 3 cm in length, leading to effortless transposition and alignment in the anterior neck. 5. Penrose drain placed at inferior edge of wound in all cases
Fig. 1. Illustration defining a midline cervical cleft. The lateral view reveals the skin contracture as a result.
3. Results
correct MCC, involving the use of a double z-plasty flap, in order to create a tension-free closure, restoring contour to the anterior neck, and preventing contracture.
There were no recurrences using the double Z-plasty technique as executed using the design and the methods described. The four cases which required reoperation had not undergone the Z-plasty technique. These patients had no evidence of contracture after excision of cord and closure using double z-plasty technique. The average age of patients at diagnosis was within the first month of life. All children underwent ultrasound of the neck prior to surgery. Surgical intervention was completed at an average age of 9.5 months. The most commonly reported complication was hypertrophic scar (3/ 12). Small wound dehiscence was noted in one case. None of these required return to OR. All cases had excellent cosmetic outcome with restoration of anterior neck contour. Post op follow up was on average 6 weeks after surgery, with the longest follow up date 7 months postoperative (Fig. 5).
2. Materials and methods 2.1. Study design and setting Using a database search method, children with the clinical diagnosis of midline cervical cleft treated between 2006 and 2016 were identified. Database search was undertaken at Lurie Children's Hospital, a tertiary care pediatric referral center.
2.2. Study participants
4. Discussion
12 individual patients were identified in the Lurie Children's Hospital (LCH) database through database search and chart review (Table 1). Of the 12 patients identified, 8 were completed by the senior author using the double z-plasty closure. Two patients underwent linear excision and closure by another surgeon at LCH. The remaining two were completed at other institutions, without a z-plasty closure.
MCC is an extremely rare anomaly of the anterior cervical neck seen in the pediatric population. This is present from birth, though frequently not recognized at that time. Its exact incidence is unknown, though a 1985 review by Gargan found they comprised 1.7% of congenital anterior neck lesions [16]. MCC is thought to occur through the failure of branchial arch fusion. In normal embryologic development, fusion of the arches occurs in a cephalad to caudal direction beginning in the fourth week of gestation, with failure at any point resulting in a midline defect. During fusion, mesodermal elements migrate between the arches, and flatten ventral furrow by exteriorizing ectodermal tissue. In the case of MCC, a failure of the first (mandibular) or second
2.3. Outcome measures Main outcome measure was recurrence of MCC. Secondary measures included return to operating room, restoration of anterior neck contour, and cosmesis. Table 1 Characteristics/outcomes of patients undergoing MCC repair. Patient
Age at Surgery (months)
Recurrence
Prior Surgery at OSH
Need for Revision
Post Op Complications
1 2 3 4 5 7 7 8 9 10 11 12
5 9 13 11 5 5 9 9 13 10 13 13
no no yes no no yes no no unknown no yes no
no no yes no no yes no no no no no no
no yes yes no no yes no no unknown no yes no
hypertrophic scar hypertrophic scar none none none none none wound dehiscence unknown none hypertrophic scar none
2
International Journal of Pediatric Otorhinolaryngology 127 (2019) 109657
J.N. D'Souza, et al.
Fig. 2. Double z-plasty closure with long limb technique.
Fig. 3. Intraoperative double z plasty design planning. Fig. 5. Midline cervical cleft. A,B) Preoperative view of fibr.
fibrous cord that extends from the sternum to the mentum of the mandible; further illustrating the midline fusion abnormality. It is often associated with an upper nipple-like projection, and an inferior sinus, which may drain mucoid fluid. These elements are not, however, necessary for diagnosis. Presence of fibrous band with resultant neck contracture should encourage the provider to consider diagnosis of MCC. Once diagnosis of MCC is made, evaluation for the presence of other midline defects should be undertaken. Cleft of the tongue, lip, and mandible have been described, as well as thyroglossal duct, bronchogenic, and dermoid cysts [1,2,7]. One case of congenital heart defect with MCC has been described [4]. Several imaging modalities are suggested for this purpose. Prior to the advent of modern imaging techniques, x-ray evaluation of the neck was undertaken, which classically demonstrated bony spurring at the mandible in advanced cases, thought to be due to traction from the fibrous tract [6]. More commonly, ultrasound or magnetic resonance imaging (MRI) of the neck are used for characterization of the lesion and exclusion of concurrent lesions. Ultrasound of the neck typically shows an avascular, blind-ending sinus tract from the skin surface, along with fibrous band contained within the dermis [6,8]. MRI of the neck typically demonstrates thinning of the epidermis, and a linear T1 hypointense/T2 hyperintense tract, without disruption of underlying bony or cartilaginous structures [6]. It is our practice to use ultrasound exclusively during initial diagnosis, with MRI reserved for cases where associated lesions are identified during screening ultrasound. After completion of imaging studies, the question of management of the lesion arises. The natural history of MCC is that it grows commensurate with the child, and lengthening and increased prominence of the cord occurs as the neck elongates [1]. Limitation of extension may occur, and obliteration of normal cervicomental angle can be seen. Early intervention is recommended in order to prevent long-term
Fig. 4. Intraoperative double z plasty closure.
(hyoid) arches can lead to persistent midline furrow, and subsequent fibrous cord formation, with or without inferior sinus tract [2]. Other hypotheses include exteriorization of thyroglossal duct remnant, or bronchogenic origin [12], however our senior author (JM) believes that failure of branchial arch fusion is the most likely etiology. Histologic findings are generally unremarkable, with the most consistent finding being absence of epithelial adnexa in the dermis, fibrosis replacing superficial musculature, and areas of atrophy/hypertrophy of the dermis along with dyskeratosis [2]. MCC is present at birth, though its diagnosis may not be made until early infancy and neck mobility is noted to be impaired. It is recognized as a cutaneous/subcutaneous defect that classically is represented by complete absence of dermal tissue or dysmorphic skin oriented vertically and of varying length. It is also associated with an underlying 3
International Journal of Pediatric Otorhinolaryngology 127 (2019) 109657
J.N. D'Souza, et al.
neonate or infant presenting with midline fibrous cord connecting submentum to posterior/inferior aspect of mandible. Once this diagnosis is made, complete surgical excision of the fibrous cord should be undertaken by one year of age, with use of double z-plasty closure technique in order to reproducibly minimize recurrence as well as provide optimal cosmetic outcome with restoration of anterior neck contour.
contracture of the neck as well as hypoplasia of the mandible. We advocate for early intervention, ideally before one year old, to prevent mandibular and contracture issues. Additionally, the fibrous cord has not yet matured into a mature scar at this age. Management of MCC is primarily surgical, and multiple techniques have been described. All authors agree that complete excision of the lesion is essential, as mere transection of the cord frequently results in recurrence and persistent contracture [1]. Debate exists regarding skin closure [1,14]. Some authors advocate for use of linear closure, particularly in early infancy when fibrous cord has not yet transformed into mature scar [2,3]. A linear closure is feasible when the cutaneous lesion is small and the adjacent tissue plentiful, allowing for a lax linear closure. This still requires wide undermining with dissection and excision of the fibrous band to prevent a recurrence. As most patients are now managed surgically beyond the stages of early infancy, a linear closure is not a reasonable option. Other reports describe varying techniques with W- and Z-plasty [1,6,8,13,15]. In a previous article by senior author (JM), consideration of linear excision and closure had been given to small defects with lax adjacent tissues [2]. However, in this series, recurrence with persistent contracture was demonstrated in four infants that had undergone linear excision. The purpose of this article is to describe our use of the double z-plasty with long limb technique for prevention of contracture and restoration of anterior contour of the neck. Z-plasty is a common technique and versatile surgical maneuver, allowing the surgeon to lengthen a contracted scar, reorient scar direction, break up a straight line, and, essentially, shift soft tissue contour [5]. The double z-plasty technique involves creation of two triangular transposition flaps that are rotated optimally to close a central defect. Use of the z-plasty allows a gain of 75% length with optimal design using an angle of 60°. More obtuse angles have demonstrated tissue distortion and formation of dog-ear deformity, while angles less than 20° present viability concerns due to impaired blood supply. In our experience, we have found the double z-plasty to be optimal as it allows for optimal tissue rotation and viability. The danger with a multiple z-plasty is not making, or not being able to make the limbs of the Z long enough. This has the potential to compromise the extra tissue mobilized to correct the contracture. It has been the observation that when multiple Z-plasty is employed (> 2 limbs), that the size of the flaps is inadequate to mobilize enough tissue to correct the neck contracture. Additionally, the double z-plasty allows the limbs to fall into the natural skin crease. The single z-plasty does not always lend itself to this. The most common complication encountered with our technique was hypertrophic scarring. In our experience, this can be ascribed to the nature of the defect, in which it represents an open lesion and therefore likely has a subclinical infection. The lesion also possesses ectodermal and mesodermal components which predispose to scar formation (fibrous cord). Although the Z-plasties were closed without tension, there also may be a tensile component to the scar formation secondary to mobility and stretching of the neck after excision. Proper tissue handling techniques and wide undermining provide the opportunity to optimize post-surgical healing. The novelty of our technique is the consistent use of double z-plasty with long limb length, in order to provide tension free closure (Fig. 2). The study is the largest utilizing the double z-plasty with no evidence of recurrence. Thus, use of the double z-plasty technique using the guidelines we provide allows for optimal results. This technique is a reproducible and reliable technique to prevent recurrence of the cord, provide excellent cosmetic outcome, and should be considered the standard surgical method for management of MCC.
Financial disclosure The authors have no financial relationships relevant to this article to disclose. Funding source No external funding support. Conflicts of interest The authors have no conflicts of interest relevant to this article to disclose. Statement of ethics Research was approved by Ann & Robert H. Lurie Children's Hospital of Chicago Institutional Review Board and conducted in an ethical manner with respect to patient data and privacy. Appendix A. Supplementary data Supplementary data to this article can be found online at https:// doi.org/10.1016/j.ijporl.2019.109657. References [1] L. Puscas, Midline cervical cleft: review of an uncommon entity, nt. J. Pediatr. Otolaryngol. (2015), https://doi.org/10.1155/2015/209418 2015:209418. [2] J. Maddalozzo, A. Frankel, L. Holinger, Midline cervical cleft, Pediatrics 92 (2) (1993) 286–287. [3] X. Sinopidis, H. Kourea, A. Panagidis, V. Alexopoulos, S. Tzifas, G. Dimitriou, G. Georgiou, Congenital midline cervical cleft: diagnosis, pathologic findings, and early state treatment, Case Rep. Pediatr. 1 (2012) 951040. [4] S. Hirokawa, H. Uotani, H. Okami, K. Tsukada, T. Futatani, I. Hashimoto, A case of congenital midline cervical cleft with congenital heart disease, J. Pediatr. Surg. 38 (7) (2003) 1099–1101. [5] K. Kadokhar, S. Patel, J. Maddalozzo, Congenital Midline Cervical Cleft, Otolaryngology 3 (2013) 132, https://doi.org/10.4172/2161-119X. 1000132. [6] J. Villanueva-Meyer, C. Glastonbury, P. Marcovici, Congenital midline cervical cleft, J. Radiol. Case Rep. 9 (3) (2015) 7–11. [7] B. Crippa, M. Bedeschi, G. Cantarella, L. Colombo, V. Agosti, I. Amodeo, M. Fumagalli, I. Mazzola, F. Mosca, Congenital. Anom. 55 (2015) 112–115. [8] R. Goldfisher, P. Bawa, Z. Ibrahim, J. Amodio, Clinical and imaging features of a congenital midline cervical cleft in a neonate: a rare anomaly, Case Rep. Pediatr. (2015), https://doi.org/10.1155/2015/439596 2015:439596. [9] R. Smith, P. Barth, J. Castillo, B. Millman, W. Wood, Congenital midline cervical cleft: a report of 3 cases, Ear Nose Throat J. 85 (2) (2006) 119–120. [10] C. McInnes, A. Benson, C. Verchere, J. Ludemann, J. Arneja, Management of congenital midline cervical cleft, J. Craniofac. Surg. 23 (1) (2012) e36–38. [11] D. Mendis, A. Moss, Case series: variations in the embryology of congenital midline cervical clefts, Acta Chir. Plast. 49 (3) (2007) 71–74. [12] Y. Bajaj, D. Dunaway, B. Hartley, Surgical approach for congenital midline cervical cleft, J. Laryngol. Otol. 118 (2004) 566–568. [13] J. Daw, P. Patel, Double-opposing Z-plasty for correction of midline cervical web, J. Craniofac. Surg. 14 (5) (2003) 774–777. [14] S. Achard, X. Leroy, P. Fayoux, Congenital midline cervical cleft: a retrospective case series of 8 children, nt. J. Pediatr. Otolaryngol. 81 (2016) 60–64. [15] C. Cochran, R. DeFatta, A. Brenski, Congenital midline cervical cleft: a practical approach to Z-plasty closure, Int. J. Pediatr. Otorhinolaryngol. 70 (3) (2006) 553–559. [16] T. Gargan, M. McKinnon, J. Mulliken, Midline cervical cleft, Plast. Reconstr. Surg. 76 (1985) 223–229.
5. Conclusion Diagnosis of midline cervical cleft should be considered in any
4