Pediatric Traumatology, Orthopaedics and Reconstructive SurgeryPediatric Traumatology, Orthopaedics and Reconstructive SurgeryОртопедия, травматология и восстановительная хирургия детского возрастаPediatric Traumatology, Orthopaedics and Reconstructive Surgery2309-39942410-8731Eco-Vector6081510.17816/PTORS60815Clinical casesКлинические случаиClinical casesResearch ArticleA complex scalp resurfacing utilizing Integra® as temporary dressing in aplasia cutis congenitaКомплексная регенерация кожи головы при врожденной аплазии с использованием заменителя дермы Integrahttps://orcid.org/0000-0002-2228-3473YapPaulineYapPauline
Malaysia

MD

paulineyap@live.comhttps://orcid.org/0000-0001-6731-9962Mohamad ShahNurul SyazanaMohamad ShahNurul Syazana
Malaysia

PhD

д-р медицины

syazanashah@usm.myhttps://orcid.org/0000-0002-4003-6783Mat SaadArman ZaharilMat SaadArman Zaharil
Malaysia

MS, Professor

магистр хирургии

armanzaharil@gmail.comhttps://orcid.org/0000-0002-0600-9765Wan SulaimanWan AzmanWan SulaimanWan Azman
Malaysia

MS, Professor

магистр хирургии

wsazman@yahoo.comhttps://orcid.org/0000-0003-4120-4918Mat JoharSiti Fatimah NoorMat JoharSiti Fatimah Noor
Malaysia

MS

магистр хирургии

fatimahmj@usm.myUniversiti Malaysia SabahUniversiti Malaysia SabahUniversiti Sains MalaysiaUniversiti Sains MalaysiaManagement and Science UniversityManagement and Science UniversityUniversiti Sains Malaysia0606202129062021922292341402202104062021Copyright ©; 2021, Yap P., Mohamad Shah N., Mat Saad A.Z., Wan Sulaiman W., Mat Johar S.N.Copyright ©; 2021, Yap P., Mohamad Shah N., Mat Saad A., Wan Sulaiman W., Mat Johar S.Copyright ©; 2021, Yap P., Mohamad Shah N., Mat Saad A., Wan Sulaiman W., Mat Johar S.2021Yap P., Mohamad Shah N., Mat Saad A.Z., Wan Sulaiman W., Mat Johar S.N.Yap P., Mohamad Shah N., Mat Saad A., Wan Sulaiman W., Mat Johar S.Yap P., Mohamad Shah N., Mat Saad A., Wan Sulaiman W., Mat Johar S.http://creativecommons.org/licenses/by/4.0https://journals.eco-vector.com/turner/article/view/60815

BACKGROUND: Aplasia cutis congenita is a rare newborn malformation characterized by focal absence of skin. It possesses difficulty in reconstruction surgery for neurosurgeons and plastic surgeons. We report a challenging case of aplasia cutis congenita who received treatment in our center.

CLINICAL CASE: A 2-year-old boy, presented to Plastic and Reconstructive Surgery, Hospital USM, with bilateral vertex defect with encephalocele who received a series of surgical interventions since 1 month old. Unfortunately, he returned after 2 years with a chronic nonhealing scalp wound associated with dura defect and cerebral spinal fluid leakage. The wound was debrided and the swab culture result showed no organism growth. Part of the wound bed with dura defect was repaired using a small piece of transplanted fascia lata and Integra® was applied.

DISCUSSION: There is scarcity in the medical literature on the reconstructive technique of aplasia cutis congenita. In the case we described here, we successfully managed the wound with multiple application of dermal substitute (Integra®) dressing with negative pressure wound therapy and split-thickness skin graft.

CONCLUSIONS: Management of aplasia cutis congenita with skull defect remains a controversy. Its management varies depends on its pattern and underlying condition. We successfully develop a new simple method in treating scalp accutilizing Integra®.

Обоснование. Врожденная аплазия кожи — редкий порок развития новорожденных, характеризуемый очаговым отсутствием кожи. Подобные дефекты относятся к сфере деятельности нейрохирургов и пластических хирургов. В статье приведен сложный случай врожденной аплазии кожи у новорожденного, получавшего лечение в нашем центре.

Клиническое наблюдение. Мальчик, 2 года, поступил в отделение пластической и реконструктивной хирургии больницы при Университете Сайнс Малайзия с двусторонним дефектом теменной зоны и грыжей головного мозга, перенес ряд хирургических вмешательств с одномесячного возраста. Через 2 года ребенок снова доставлен в лечебное учреждение с хронической незаживающей раной кожи головы, осложненной дефектом твердой мозговой оболочки и утечкой спинномозговой жидкости. Выполнена хирургическая обработка раны, результаты посева мазка на микрофлору отрицательные. Часть раневого ложа с дефектом твердой мозговой оболочки восстановлена с помощью трансплантированных тканей широкой фасции небольшого размера, наложен заменитель дермы Integra.

Обсуждение. В медицинской литературе представлено недостаточно информации о технике реконструкции врожденной аплазии кожи. В описанном нами случае рану успешно лечили путем многократного наложения заменителя дермы Integra, применяли метод терапии раны отрицательным давлением, производили пересадку расщепленного кожного трансплантата.

Заключение. На сегодняшний день методы лечения врожденной аплазии кожи с дефектом костей черепа не определены. Выбор терапии зависит от клинической картины и причин заболевания. Мы предлагаем новый простой в использовании метод лечения врожденной аплазии кожи волосистой части головы с помощью заменителя дермы Integra.

skin substitutecongenital defect of skull and scalpscalp defectcongenitalaplasia cutis of the scalpзаменитель коживрожденный дефект волосистой части кожи головы и костей черепадефект кожи головыврожденныйаплазия кожи головыBrzezinski P, Pinteala T, Chiriac AE, et al. Aplasia cutis congenita of the scalp – what are the steps to be followed? Case report and review of the literature. An Bras Dermatol. 2015;90(1):100–103. DOI: 10.1590/abd1806-4841.20153078Brzezinski P., Pinteala T., Chiriac A.E. et al. Aplasia cutis congenita of the scalp – what are the steps to be followed? Case report and review of the literature // An. Bras. Dermatol. 2015. Vol. 90. No. 1. P. 100–103. DOI: 10.1590/abd1806-4841.20153078Moros Peña M, Labay Matías M, Valle Sánchez F, et al. Aplasia cutis congenita in a newborn: etiopathogenic review and diagnostic approach. An Esp Pediatr. 2000;52(2):453–456. (In Spain)Moros Peña M., Labay Matías M., Valle Sánchez F. et al. Aplasia cutis congenita in a newborn: etiopathogenic review and diagnostic approach // An. Esp. Pediatr. 2000. Vol. 52. No. 2. P. 453–456. (In Spain)Smartt J, Kim E, Tobias A, et al. Aplasia cutis congenita with calvarial defects: a simplified management strategy using acellular dermal matrix. Plast Reconstr Surg. 2008;121(4):1224–1229. DOI: 10.1097/01.prs.0000302588.95409.feSmartt J., Kim E., Tobias A. et al. Aplasia cutis congenita with calvarial defects: a simplified management strategy using acellular dermal matrix // Plast. Reconstr. Surg. 2008. Vol. 121. No. 4. P. 1224–1229. DOI: 10.1097/01.prs.0000302588.95409.feDemmel U. Clinical aspects of congenital skin defects. I Congenital skin defects on the head of the newborn. Eur J Pediatr. 1975;121(1):21–50.Demmel U. Clinical aspects of congenital skin defects. I Congenital skin defects on the head of the newborn // Eur. J. Pediatr. 1975. Vol. 121. No. 1. P. 21–50.Johnson R, Offiah A, Cohen MC. Fatal superior sagittal sinus hemorrhage as a complication of aplasia cutis congenita: A case report and literature review. Forensic Sci Med Pathol. 2015;11(2):243–248. DOI: 10.1007/s12024-014-9645-5Johnson R., Offiah A., Cohen M.C. Fatal superior sagittal sinus hemorrhage as a complication of aplasia cutis congenita: A case report and literature review // Forensic. Sci. Med. Pathol. 2015. Vol. 11. No. 2. P. 243–248. DOI: 10.1007/s12024-014-9645-5Maillet-Declerck M, Vinchon M, Guerreschi P, et al. Aplasia cutis congenita: Review of 29 cases and proposal of a therapeutic strategy. Aplasia cutis congenita: Review of 29 cases and proposal of a therapeutic strategy. Eur J Pediatr Surg. 2013;23(2):89–93. DOI: 10.1055/s-0032-1322539Maillet-Declerck M., Vinchon M., Guerreschi P. et al. Aplasia cutis congenita: Review of 29 cases and proposal of a therapeutic strategy. Aplasia cutis congenita: Review of 29 cases and proposal of a therapeutic strategy // Eur. J. Pediatr. Surg. 2013. Vol. 23. No. 2. P. 89–93. DOI: 10.1055/s-0032-1322539Tavasoli A, Ashrafi M, Mohammadi M, et al Aplasia Cutis Congenita (ACC) and Seizure in a Premature Neonate: Could It Be a New Neurocutaneous Syndrome? Iranian Journal of Neonatology. 2013;4(4):54–57. DOI: 10.22038/IJN.2013.2014Tavasoli A., Ashrafi M., Mohammadi M. et al. Aplasia Cutis Congenita (ACC) and Seizure in a Premature Neonate: Could It Be a New Neurocutaneous Syndrome? // Iranian Journal of Neonatology. 2013. Vol. 4. No. 4. P. 54–57. DOI: 10.22038/IJN.2013.2014Bharti G, Groves L, David LR, et al. Aplasia cutis congenita: clinical management of a rare congenital anomaly. J Craniofac Surg. 2011;22(1):159–165. DOI: 10.1097/SCS.0b013e3181f73937Bharti G., Groves L., David L.R. et al. Aplasia cutis congenita: clinical management of a rare congenital anomaly // J. Craniofac. Surg. 2011. Vol. 22. No. 1. P. 159–165. DOI: 10.1097/SCS.0b013e3181f73937Kim CS, Tatum SA, Rodziewics G. Scalp aplasia cutis congenita presenting with sagittal sinus hemorrhage. Arch Otolaryngol Head Neck Surg. 2001;127(1):71–74. DOI: 10.1001/archotol.127.1.71Kim C.S., Tatum S.A., Rodziewics G. Scalp aplasia cutis congenita presenting with sagittal sinus hemorrhage // Arch. Otolaryngol. Head Neck Surg. 2001. Vol. 127. No. 1. P. 71–74. DOI: 10.1001/archotol.127.1.71de Oliveira RS, Barros Juca CE, LinsNeto AL, et al. Aplasia cutis congenita of the scalp: is there a better treatment strategy? Childs Nerv Syst. 2006;(9):1072–1079. DOI: 10.1007/s00381-006-0074-yde Oliveira R.S., Barros Juca C.E., LinsNeto A.L. et al. Aplasia cutis congenita of the scalp: is there a better treatment strategy? // Childs Nerv Syst. 2006. Vol. 22. No. 9. P. 1072–1079. DOI: 10.1007/s00381-006-0074-y