Surgical Management of the Highly Edematous Conjunctiva Due to Ectropion in a 3-Year-Old Collodion Baby
Collodion baby is an extremely rare congenital condition characterized by the presence of parchment-like or cellophane-like membranes that encompass the entire body at birth. These membranes typically peel off after several weeks, revealing one of several types of ichthyosis skin. The condition is often associated with complications such as ectropion, eclabium, and restricted joint movements due to the tight and inelastic skin. While systemic and conservative treatments for ectropion in collodion babies have been occasionally reported, surgical management of the highly edematous conjunctiva due to ectropion is rarely documented. This article presents a detailed case of a 3-year-old collodion baby with bilateral ectropion and the surgical approach employed to manage the condition.
The patient, a 3-year-old boy, was referred to the ophthalmology department for the management of bilateral upper conjunctival bullae that had persisted for one month. The boy had been diagnosed with ectropion at birth, a condition where the eyelids turn outward, exposing the inner conjunctiva. On physical examination, it was observed that the patient had highly edematous upper palpebral conjunctiva with ectropion affecting both the upper and lower eyelids. The lower eyelids lacked eyelashes, and the boy also had no eyebrows or head hair. Despite these abnormalities, there was no evidence of corneal involvement. The skin across the entire body, including the eyelids, was rigid and covered with a cellophane-like membrane. Sparse skin scales and cracks were also observed, and the movements of the extremities were restricted.
The boy was born prematurely at 36 weeks via cesarean section due to premature rupture of the amniotic membrane. At birth, he was diagnosed as a collodion baby based on his appearance, and this diagnosis was later confirmed by a skin biopsy, which revealed overgrowth and thickening of the stratum corneum. He was subsequently diagnosed with congenital lamellar ichthyosis and received systemic treatment in the pediatrics and dermatology departments. When referred for the management of ectropion, the patient showed no signs of compromised vital functions.
Given the severity of the conjunctival edema and the functional impairment caused by the ectropion, surgical intervention was deemed necessary. The surgical procedure chosen was permanent tarsorrhaphy, a technique where the eyelids are partially sutured together to correct the ectropion and alleviate the conjunctival edema. The surgery was performed under general anesthesia. The gray lines of both the upper and lower eyelids were incised, and the eyelid margins were debrided. An incision was made in the upper conjunctiva to release the sub-conjunctival fluid. The upper and lower eyelids were then sutured together using 2 to 3 intermittent stitches. Silicone tubes were placed at the suture site to protect the skin. Importantly, the central portion of the eyelids covering the cornea was left unsutured to ensure that the patient retained some degree of vision post-surgery.
Three months after the procedure, the stitches were removed, and a significant reduction in the conjunctival edema was observed. Although the ectropion was not fully corrected due to the stiffness of the eyelid skin, the patient’s condition improved markedly, and the conjunctival bullae had diminished. The surgical intervention not only addressed the striking appearance of the ectropion but also improved the patient’s ability to see.
Lamellar ichthyosis is one of the most common underlying causes of collodion baby. In this case, the ectropion had been present since birth but became more pronounced over time, likely due to the increasing tension between the developing eyeball and the inelastic eyelids. While conservative methods are typically employed for the treatment of ectropion in collodion babies, surgical intervention was necessary in this case due to the severity of the conjunctival edema and the functional impairment it caused. Permanent tarsorrhaphy proved to be an effective surgical option for alleviating the conjunctival edema caused by ectropion. However, care must be taken to avoid suturing the central portion of the eyelids to prevent potential amblyopia, a condition where vision does not develop properly due to the lack of visual stimulation during critical periods of development.
This case highlights the challenges associated with managing ectropion in collodion babies and underscores the importance of a tailored surgical approach. While permanent tarsorrhaphy can provide significant relief from conjunctival edema, the procedure must be carefully planned to preserve the patient’s vision and address the unique anatomical challenges posed by the condition. The successful outcome in this case demonstrates the potential benefits of surgical intervention in carefully selected patients with severe ectropion and conjunctival edema.
In conclusion, the surgical management of highly edematous conjunctiva due to ectropion in a collodion baby requires a thorough understanding of the underlying condition and a meticulous surgical approach. Permanent tarsorrhaphy can be an effective treatment option, but it must be performed with care to avoid compromising the patient’s vision. This case serves as a valuable example of the challenges and considerations involved in the surgical management of ectropion in collodion babies and provides insights into the potential benefits of such interventions.
doi.org/10.1097/CM9.0000000000001060
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