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Repair of cicatricial ectropion in an infant with harlequin ichthyosis using engineered human skin
forces the notion that any patient with acquired ptosis should have a complete ophthalmic examination including, evaluation of extraocular motility.
weeks after birth from respiratory failure, feeding problems, fluid loss, or sepsis. Treatment is mainly supportive. We evaluated a male infant with harlequin ichthyosis. At birth the child had plaques of hyperkeratinized skin covering all surfaces. The interplaque skin was macerated. Severe bilateral upper eyelid cicatricial ectropion and eclabium were present. Our patient was treated at birth with tube feeding, oral isotretinoin, humidified incubator, topical aquaphor with occlusive dressings, and intravenous vancomycin for methicillin-resistant staphylococcus aureus sepsis. The eclabium resolved before our initial examination. Multiple large hyperkeratotic plaques remained on the scalp and forehead. Complete eversion of the upper eyelids occurred. Conjunctival prolapse limited visualization of the globes (Figure 1a). Treatment with intensive topical ointments resulted in little improvement. We proceeded with ectropion repair at age 40 days, hoping to improve the lagophthalmos and reduce the risk of deprivation amblyopia. Under general anesthesia, supracilliary incisions were carried across each upper eyelid. The thickened skin was separated from the orbicularis muscles and allowed to retract superiorly. Because the patient’s skin condition made the usual skin graft donor sites unavailable, we elected to augment the anterior lamella with an Apligraf (Organogenesis, Inc., Canton, Massachusetts, USA) “human skin equivalent.” The Apligraf was sutured over the orbicularis muscle (Figure 1b). Temporary suture tarsorrhaphies were placed. The grafts were covered with bolsters, which were removed 1 week after surgery. Progressive shrinkage of the grafts resulted in mild recurrent cicatricial ectropion. A second, similar procedure was performed at age 61 days. The eyelids subsequently remained well positioned, in part related to coexisting improvement in the cutaneous scaling (Figure 1c). Mild lagophthalmos persisted, necessitating the daily use of topical lubricating ointment. Both eyes appeared to have normal visual function until the child’s sudden death from apparent respiratory failure at age 6 months. Apligraf is a bilayered “human skin equivalent” composed of cultured dermal and epidermal layers. The material was originally used to repair defects after the removal of skin cancers.2 Its most promising application has been in nonhealing venous ulcers.3 Apligraf has also been used in infants with epidermolysis bullosa.4 Apligraf may promote healing by several mechanisms. It may act as a biological dressing facilitating healing by secondary intent. In some patients, partial take of the graft seems to occur with remodeling of the surrounding host tissue.2 Apligraf may also act as a “pharmacologic dressing” providing cytokines and cell mediators that stimulate healing by host cells. In our patient, it is unclear if the grafted tissue became vascularized and survived. The remarkable improvement in the ectropion of our patient may have primarily been related to the coexisting improvement in the facial hyperkeratotic plaques and relaxation of the facial skin. How-
REFERENCES
1. Ojemann RG. Management of cranial and spinal meningiomas. Clin Neurosurg 1993;40:321–383. 2. Cockerham KP, Kennerdell JS, Maroon JC. Tumors of the meninges and related tissues: meningiomas and sarcomas. In: Miller NR, Newman NJ, editors. Walsh and Hoyt’s clinical neuro-ophthalmology. 5th ed. Baltimore: Williams and Wilkins, 1998:2017–2082.
Repair of Cicatricial Ectropion in an Infant With Harlequin Ichthyosis Using Engineered Human Skin Susan M. Culican, MD, PhD, and Philip L. Custer, MD PURPOSE:
To report the use of an Apligraf (Organogenesis, Inc., Canton, Massachusetts, USA) human skin equivalent for repair of cicatricial ectropion in a patient with harlequin ichthyosis. DESIGN: Interventional case report. METHODS: A 6-week-old male child with harlequin ichthyosis and severe bilateral upper eyelid cicatricial ectropion underwent repair with Apligraf grafts. RESULTS: After the initial repair with Apligraf grafts, recurrent bilateral upper eyelid ectropion developed, requiring repeat Apligraf grafting at age 61 days. After the second graft, the eyelids remained well positioned until the child’s sudden death from respiratory failure at age 6 months. CONCLUSION: Apligraf human skin equivalent facilitated the repair of cicatricial ectropion in a child with harlequin ichthyosis. (Am J Ophthalmol 2002;134: 442– 443. © 2002 by Elsevier Science Inc. All rights reserved.)
T
HE ICHTHYOSES ARE A COLLECTION OF SCALING SKIN
diseases giving the appearance of “fish skin.” Harlequin ichthyosis describes a genetically heterogeneous group of disorders with autosomal recessive inheritance.1 Until recently, children with this condition usually died within
Accepted for publication April 16, 2002. From the Department of Ophthalmology, Washington University School of Medicine, St. Louis, Missouri. Inquiries to Philip L. Custer, MD, Department of Ophthalmology, Washington University School of Medicine, Box 8096, 660 S Euclid Ave, St. Louis, MO 63110; fax: (314) 362-3725; e-mail: custer@vision. wustl.edu
442
AMERICAN JOURNAL
OF
OPHTHALMOLOGY
SEPTEMBER 2002
FIGURE 1. (a) Preoperative appearance of a 40-day-old male infant with harlequin ichthyosis with plaques of hyperkeratinized skin and bilateral upper eyelid ectropion. (b) Intraoperative photographs show engineered human skin grafts in both upper eyelids. (c) Postoperative appearance (age: 116 days) after repeat grafting and improvement of the ichthyosis.
ever, the Apligraf enabled initial repositioning of the eyelid margins, facilitating corneal protection until improvement in the dermatopathy occurred. REFERENCES
1. Scwayder T, Ott F. All about ichthyosis. Ped Clin N Am 1991;38:835– 857. 2. Eaglstein WH, Iriondo M, Laszlo K. A composite skin substitute (Graftskin) for surgical wounds. Dermatol Surg 1995;21: 839 – 843. 3. Falanga V, Sabolinski M. A bilayered living skin construct (APLIGRAF) accelerates complete closure of hard-to-heal venous ulcers. Wound Repair Regen 1999;7:201–207. 4. Fallabella AF, Schachner LA, Valencia IC, Eaglstein WH. The use of tissue-engineered skin (Apligraf) to treat a newborn with epidermolysis bullosa. Arch Derm 1999;135:1219 – 1222.
Bilateral Panuveitis in a Child With Hypohidrotic Ectodermal Dysplasia Natalia Rodriguez, MD, Dean Eliott, MD, Enrique Garcia-Valenzuela, MD, PhD, and John Baker, MD VOL. 134, NO. 3
PURPOSE:
To report a case of bilateral panuveitis in a patient with hypohidrotic ectodermal dysplasia. DESIGN: Interventional case report. METHODS: A 6-year-old African-American boy with hypohidrotic ectodermal dysplasia presented with pain, photophobia, and decreased vision in both eyes. RESULTS: Findings included severe bilateral panuveitis with optic disk edema, macular epiretinal membrane, peripheral retinal vasculitis, and retinitis. All other known causes of panuveitis were explored and ruled out. CONCLUSIONS: The abnormal development of tissues of ectodermal origin evident in hypohidrotic ectodermal dysplasia may include a predisposition to panuveitis. (Am J Ophthalmol 2002;134:443– 445. © 2002 by Elsevier Science Inc. All rights reserved.)
Accepted for publication April 18, 2002. From the Kresge Eye Institute (N.R., D.E., E.G.-V.) and the Children’s Hospital of Michigan (D.E., J.B.), Wayne State University School of Medicine, Detroit, Michigan. Inquiries to Dean Eliott, MD, Kresge Eye Institute, Wayne State University School of Medicine, 4717 St. Antoine, Detroit, MI 48201; fax: (313) 577-2905; e-mail: deliott @ med.wayne.edu
BRIEF REPORTS
443
weeks after birth from respiratory failure, feeding problems, fluid loss, or sepsis. Treatment is mainly supportive. We evaluated a male infant with harlequin ichthyosis. At birth the child had plaques of hyperkeratinized skin covering all surfaces. The interplaque skin was macerated. Severe bilateral upper eyelid cicatricial ectropion and eclabium were present. Our patient was treated at birth with tube feeding, oral isotretinoin, humidified incubator, topical aquaphor with occlusive dressings, and intravenous vancomycin for methicillin-resistant staphylococcus aureus sepsis. The eclabium resolved before our initial examination. Multiple large hyperkeratotic plaques remained on the scalp and forehead. Complete eversion of the upper eyelids occurred. Conjunctival prolapse limited visualization of the globes (Figure 1a). Treatment with intensive topical ointments resulted in little improvement. We proceeded with ectropion repair at age 40 days, hoping to improve the lagophthalmos and reduce the risk of deprivation amblyopia. Under general anesthesia, supracilliary incisions were carried across each upper eyelid. The thickened skin was separated from the orbicularis muscles and allowed to retract superiorly. Because the patient’s skin condition made the usual skin graft donor sites unavailable, we elected to augment the anterior lamella with an Apligraf (Organogenesis, Inc., Canton, Massachusetts, USA) “human skin equivalent.” The Apligraf was sutured over the orbicularis muscle (Figure 1b). Temporary suture tarsorrhaphies were placed. The grafts were covered with bolsters, which were removed 1 week after surgery. Progressive shrinkage of the grafts resulted in mild recurrent cicatricial ectropion. A second, similar procedure was performed at age 61 days. The eyelids subsequently remained well positioned, in part related to coexisting improvement in the cutaneous scaling (Figure 1c). Mild lagophthalmos persisted, necessitating the daily use of topical lubricating ointment. Both eyes appeared to have normal visual function until the child’s sudden death from apparent respiratory failure at age 6 months. Apligraf is a bilayered “human skin equivalent” composed of cultured dermal and epidermal layers. The material was originally used to repair defects after the removal of skin cancers.2 Its most promising application has been in nonhealing venous ulcers.3 Apligraf has also been used in infants with epidermolysis bullosa.4 Apligraf may promote healing by several mechanisms. It may act as a biological dressing facilitating healing by secondary intent. In some patients, partial take of the graft seems to occur with remodeling of the surrounding host tissue.2 Apligraf may also act as a “pharmacologic dressing” providing cytokines and cell mediators that stimulate healing by host cells. In our patient, it is unclear if the grafted tissue became vascularized and survived. The remarkable improvement in the ectropion of our patient may have primarily been related to the coexisting improvement in the facial hyperkeratotic plaques and relaxation of the facial skin. How-
REFERENCES
1. Ojemann RG. Management of cranial and spinal meningiomas. Clin Neurosurg 1993;40:321–383. 2. Cockerham KP, Kennerdell JS, Maroon JC. Tumors of the meninges and related tissues: meningiomas and sarcomas. In: Miller NR, Newman NJ, editors. Walsh and Hoyt’s clinical neuro-ophthalmology. 5th ed. Baltimore: Williams and Wilkins, 1998:2017–2082.
Repair of Cicatricial Ectropion in an Infant With Harlequin Ichthyosis Using Engineered Human Skin Susan M. Culican, MD, PhD, and Philip L. Custer, MD PURPOSE:
To report the use of an Apligraf (Organogenesis, Inc., Canton, Massachusetts, USA) human skin equivalent for repair of cicatricial ectropion in a patient with harlequin ichthyosis. DESIGN: Interventional case report. METHODS: A 6-week-old male child with harlequin ichthyosis and severe bilateral upper eyelid cicatricial ectropion underwent repair with Apligraf grafts. RESULTS: After the initial repair with Apligraf grafts, recurrent bilateral upper eyelid ectropion developed, requiring repeat Apligraf grafting at age 61 days. After the second graft, the eyelids remained well positioned until the child’s sudden death from respiratory failure at age 6 months. CONCLUSION: Apligraf human skin equivalent facilitated the repair of cicatricial ectropion in a child with harlequin ichthyosis. (Am J Ophthalmol 2002;134: 442– 443. © 2002 by Elsevier Science Inc. All rights reserved.)
T
HE ICHTHYOSES ARE A COLLECTION OF SCALING SKIN
diseases giving the appearance of “fish skin.” Harlequin ichthyosis describes a genetically heterogeneous group of disorders with autosomal recessive inheritance.1 Until recently, children with this condition usually died within
Accepted for publication April 16, 2002. From the Department of Ophthalmology, Washington University School of Medicine, St. Louis, Missouri. Inquiries to Philip L. Custer, MD, Department of Ophthalmology, Washington University School of Medicine, Box 8096, 660 S Euclid Ave, St. Louis, MO 63110; fax: (314) 362-3725; e-mail: custer@vision. wustl.edu
442
AMERICAN JOURNAL
OF
OPHTHALMOLOGY
SEPTEMBER 2002
FIGURE 1. (a) Preoperative appearance of a 40-day-old male infant with harlequin ichthyosis with plaques of hyperkeratinized skin and bilateral upper eyelid ectropion. (b) Intraoperative photographs show engineered human skin grafts in both upper eyelids. (c) Postoperative appearance (age: 116 days) after repeat grafting and improvement of the ichthyosis.
ever, the Apligraf enabled initial repositioning of the eyelid margins, facilitating corneal protection until improvement in the dermatopathy occurred. REFERENCES
1. Scwayder T, Ott F. All about ichthyosis. Ped Clin N Am 1991;38:835– 857. 2. Eaglstein WH, Iriondo M, Laszlo K. A composite skin substitute (Graftskin) for surgical wounds. Dermatol Surg 1995;21: 839 – 843. 3. Falanga V, Sabolinski M. A bilayered living skin construct (APLIGRAF) accelerates complete closure of hard-to-heal venous ulcers. Wound Repair Regen 1999;7:201–207. 4. Fallabella AF, Schachner LA, Valencia IC, Eaglstein WH. The use of tissue-engineered skin (Apligraf) to treat a newborn with epidermolysis bullosa. Arch Derm 1999;135:1219 – 1222.
Bilateral Panuveitis in a Child With Hypohidrotic Ectodermal Dysplasia Natalia Rodriguez, MD, Dean Eliott, MD, Enrique Garcia-Valenzuela, MD, PhD, and John Baker, MD VOL. 134, NO. 3
PURPOSE:
To report a case of bilateral panuveitis in a patient with hypohidrotic ectodermal dysplasia. DESIGN: Interventional case report. METHODS: A 6-year-old African-American boy with hypohidrotic ectodermal dysplasia presented with pain, photophobia, and decreased vision in both eyes. RESULTS: Findings included severe bilateral panuveitis with optic disk edema, macular epiretinal membrane, peripheral retinal vasculitis, and retinitis. All other known causes of panuveitis were explored and ruled out. CONCLUSIONS: The abnormal development of tissues of ectodermal origin evident in hypohidrotic ectodermal dysplasia may include a predisposition to panuveitis. (Am J Ophthalmol 2002;134:443– 445. © 2002 by Elsevier Science Inc. All rights reserved.)
Accepted for publication April 18, 2002. From the Kresge Eye Institute (N.R., D.E., E.G.-V.) and the Children’s Hospital of Michigan (D.E., J.B.), Wayne State University School of Medicine, Detroit, Michigan. Inquiries to Dean Eliott, MD, Kresge Eye Institute, Wayne State University School of Medicine, 4717 St. Antoine, Detroit, MI 48201; fax: (313) 577-2905; e-mail: deliott @ med.wayne.edu
BRIEF REPORTS
443