| Abstract|| |
Background: Amniotic band syndrome (ABS) or amniotic deformity, adhesions and mutilations (” ADAM” ) is a spectrum of rare congenital malformations related to an early phase of organogenesis. In the craniofacial region, ABS could manifest in several forms. A rare form of ABS with Tessier Cleft-9 associated with atypical Cleft-2 presenting with a cicatricial ectropion is reported and management of the condition is discussed. Key Points: ABS causing oro-facial deformities is increasingly being reported. Surgical management requires staged correction and is often challenging. As growth is dynamic, evolving problems should be anticipated. Frequent follow-up and early intervention could prevent unfavourable complications. Main Lessons Learnt: ABS has craniofacial components and it requires an adequate understanding of principles of growth and healing to plan corrective surgeries for such complicated and rare conditions.
Keywords: Amniotic band syndrome, amniotic deformity, adhesions and mutilations, Cicatricial ectropion, tessier cleft-9
|How to cite this article:|
Balaji S M, Balaji P. Cicatricial ectropion correction for a case of amniotic band syndrome. Indian J Dent Res 2020;31:644-6
|How to cite this URL:|
Balaji S M, Balaji P. Cicatricial ectropion correction for a case of amniotic band syndrome. Indian J Dent Res [serial online] 2020 [cited 2022 Jan 20];31:644-6. Available from: https://www.ijdr.in/text.asp?2020/31/4/644/298418
| Background|| |
The cause of developmental craniofacial deformities are varied. It could emanate from genetic abnormalities, infections, nutritional deficiencies or mechanical trauma. Of these, local intra-uterine obstruction poses a significant challenge. The most common of this is the amniotic band syndrome (ABS) or amniotic deformity, adhesions, and mutilations (” ADAM” ). It is a spectrum of rare congenital malformations perpetuated by the early, untimely rupture of the amniotic sac. Owing to this, there occurs a series of anatomical alterations accentuated by the appearance of fibrous mesodermal amniotic tissue bands. ABS has varied clinical manifestations at birth. It includes simple ones such as constriction rings to serious ones such as limb and digital amputations. There could be diverse craniofacial malformations and thoracic-abdominal wall anomalies. Such embryological defects often do not conform to a known sequence of embryologic development.,,
Recent trends show ABS to occur in 1 in 1,200–15,000 live births. Streeter in 1930 proposed the intrinsic model that attributed the existence of an early embryo lesion with alterations of the germinal disc, causing an inflammatory response in adjacent amnion causing the fibrous band. Later, Torpin proposed the extrinsic theory. They proposed that the rupture of the amnion during early pregnancy allows the embryo or fetus to enter into the chorionic cavity and to contact the chorionic side of the amnion. In the process, fetal structures could be trapped by the fibrous septum that protrudes into the chorionic cavity. These bands cause compression and adhesions, impeding blood flow causing the abnormalities. Variations in force, site and timing give rise to various anomalies.
The most common craniofacial abnormalities in ABS are corneal and orbital defects, anencephaly, meningocele or encephalocele, palpebral colobomas, nose malformations and facial nerve paralysis, micrognathism, hyperdontia, and cleft lip with or without cleft palate. Treating a case of craniofacial ABS involves teamwork, often necessitating a staged reconstruction, depending on the intensity of defect.,, Restoring anatomical continuity and physiological functions would be the goal of treatment. The purpose of this report is to describe a case of ABS with craniofacial manifestation in a 3-1/2-year-old male child, detailing the systematic, craniofacial and oral clinical characteristics, specific treatment/management with an emphasis on the need for proper procedures.
| Case Report|| |
A 3-1/2-year-old boy was brought to the author's centre with a complaint of constant left lower lid tarsal ectropion with severely infected, hypertrophied conjunctiva and continuous epiphora. The boy was the first child of non-consanguineous parents. His pregnancy was uneventful and delivered by cesarean section. The mother had no medical conditions, no exposure to tobacco, alcohol or any drugs before or during pregnancy. At birth, the boy had conditions and features suggestive of ABS involving the orbit and eye, along with a facial cleft, suggestive of cleft number 9 associated with atypical cleft number 2. Subsequently, the boy had undergone multiple surgeries starting with proptosis correction and lower lid deconstruction at 10 months of age [Figure 1]. Upper and lower eyelid correction was done at 12 months of age. Correction for craniofacial cleft was done at 1-1/2 years of age. Reconstruction of right and left lower eyelid and lateral cheek rotation flap was done at 2-1/2 years. Occuloplasty of the right eye and scar revision was done at 3 years of age. All these surgeries were carried out elsewhere. Yet, the compromised esthetics, infection and epiphora persisted and hence the consultation.
|Figure 1: (a-c) Right lower lid tarsal ectropion with severely infected, hypertrophied conjunctiva and continuous epiphora. ABS involving the orbit and eye with facial cleft number nine associated with atypical cleft number two. Scar evident in the right temporal area, suggestive of repair of facial cleft|
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On examination, the upper and the lower eyelids of the right eye together with the lower palpebral conjunctiva and globe were involved in the scarring, causing proptosis, corneal opacity and prolapse of the lower palpebral conjunctiva with loss of vision in the right eye. The vision in the left eye was intact, although mild traction of the lower eyelid caused increased scleral exposure of the left eye. No other abnormalities were seen. A scar was evident in the right temporal area, suggestive of repair of facial cleft.
A computed tomography scan of the brain and facial bones revealed that the right orbit was smaller in volume than the left. A diagnosis of right cicatricial ectropion due to ABS and subsequent corrective surgeries was made. The treatment aimed to correct the ectropion with retro-auricular full-thickness skin graft under general anesthesia and ensuring the patent ductal system to prevent epiphora.
Under general anaesthesia, nasotracheal intubation was done. Through a subciliary incision, the right orbital region was reached with dissection done in layers. Scar tissues from previous surgeries were released. Standard lateral canthotomy was performed. The lateral tarsal strip was adapted, grasped and placed in position along the lateral orbital rim, and the level of fixation and the length of the strip used was in relation to the laxity of the eyelid and degree of prolapse. Drill holes were placed in the lateral orbital rim at the level of Whitnall's tubercle [Figure 2]. The strip was then anchored to the lateral wall at this level with 3.0 prolene. This arrangement ensured that the lacrimal ductal system was patent and functioning.
|Figure 2: (a) Standard lateral canthotomy performed. The lateral tarsal strip was adapted, grasped and placed in position along the lateral orbital rim. (b) The level of fixation and the length of the strip used was in relation to the laxity of the eyelid and degree of prolapse. (c) Drill holes placed in the lateral orbital rim at the level of whilnall's tubercle. (d) The strip was anchored to the lateral wall|
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|Figure 3: (a) Graft harvested from postauricular region. (b) Auricle was reflected by two elliptical incisions and the fat layer teased and full thickness skin graft harvested. (c) The full thickness skin graft along with sterile mesh was placed and secured with firm sutures|
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|Figure 4: (a-c) Satisfactory cosmetic appearance and function of the eyelid|
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Later, the lower eyelid skin defect was measured. Local anaesthesia was administered in the retro-auricular region for a clear field. Auricle was reflected by two elliptical incisions, fat layer teased and a full-thickness skin graft was harvested. This graft was placed and secured with firm sutures [Figure 2]. The graft was to lessen the chance of haematoma formation and to facilitate better healing. Bolsters were left in place. Appropriate antibiotics and painkillers were prescribed. Topical antibiotic ointment was applied twice daily for 7 days. The bolster and sutures were removed in 10 days. The child was followed frequently. At follow-up of 2 years, the cosmetic appearance of the eyelid and function were very satisfactory [Figure 2].
| Discussion|| |
The facial clefts in ABS have been reported and often striking in the presentation.,, In the present case too, the child's past history reflected the existence of Tessier Cleft type-9 and type-2. They have been adequately corrected. In the process of staged correction, the rehabilitation and reconstruction of eyelids is a challenge. Though primarily esthetic in nature, the functional need cannot be understated. Though such anomalies are known to cause congenital blindness in severe cases, we did not face such an issue in the present case. The need for reconstruction stems to control epiphora and for esthetic reasons. The unequal pull exerted by the scars and malformed tissues exerts continuous pressure on the lacrimal ductal system. This in turn produces continuous epiphora., The reconstruction of the lateral canthus, scar revision and compensation of lost tissue in the lower eyelid necessitated the extensive surgery required in the present case.
There are several mechanisms by which ABS could mediate facial clefts initiation and formation.,, Despite the mechanism and manifestation, the treatment could be altered. In the present case, the effect of the past treatment, scar tissues, its contraction were corrected along with the deficient lower border and eyelid. The entropion correction was done using time tested lateral canthopexy, with the author's modification with reliable results. The use of versatile local graft from the retroauricular region suited the local color and texture. The presence of bolster and proper epithelialisation ensured proper uptake of the graft. This correction ensured proper esthetics and removed the pressure on the lacrimal ductal system.
The diagnosis of ABS in being increasingly reported. This is because of early diagnosis by widespread awareness, early pregnancy monitoring and awareness of ABS-ADAMS among parents and medical fraternity., Consequently, better treatments and outcomes are being achieved. In this case, the need for this revision surgery highlights the need for appropriate timing of surgery and the need for constant supervision for a better outcome. The post-surgery healing, growth, and physiological remodeling of hard/soft tissues in a young, growing child are dynamic. Constant surveillance would prevent or help to address unfavorable outcomes in such cases. Expertise and experience of the surgical team also play a vital role in the outcome.
| Conclusion|| |
A successful case of revision of previously treated Tessier Cleft type-9 and type-2 in a young ABS patient is presented. The possible outcomes in primary surgery as well as the need for dynamic follow-up is emphasised.
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
Conflicts of interest
There are no conflicts of interest.
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Dr. S M Balaji
Director and Consultant Oral and Maxillofacial Surgeon, Balaji Dental and Craniofacial Hospital, 30, KB Dasan Road, Teynampet, Chennai - 600 018, Tamil Nadu
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4]