| Article Access Statistics | | | Viewed | 7456 | | | Printed | 217 | | | Emailed | 6 | | | PDF Downloaded | 219 | | | Comments | [Add] | | | Cited by others | 18 | | |
|
Click on image for details.
|
|
| BRIEF REPORT |
|
|
|
| Year : 2006 | Volume
: 54
| Issue : 3 | Page : 312-313 |
Aplasia cutis congenita of the scalp: Therapeutic modalities
SK Shivakumar, S Dwarakanath, Gopal Swaroop, NK Venkataramana
Department of Neurosurgery, Manipal Institute for Neurological Disorders, Bangalore - 560 017, Karnataka, India
| Date of Acceptance | 22-Feb-2006 |
Correspondence Address:
Gopal Swaroop
Manipal Institute for Neurological Disorders, Manipal Hospital, 98, Airport Road, Bangalore - 560 017
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.27165
Agenesis of scalp is an uncommon but well-recognized clinical entity. Congenital scalp and skull defects can be either obvious or occult; over 300 cases have been reported in literature. Aplasia cutis congenita (ACC) is recognized as a heterogeneous disorder, all characterized by focal absence of the epidermis, dermis and sometimes the calvarium and/or dura. We present a case of ACC in an infant whose mother was exposed to a teratogenic drug (Methimazole - an antithyroid drug) during pregnancy. This case report is presented to highlight the steps to successful management. Definitive full thickness scalp cover at the earliest avoids secondary infection, eschar formation and exsanguination.
Keywords: Aplasia cutis congenital, management, teratogenic
How to cite this article:
Shivakumar S K, Dwarakanath S, Swaroop G, Venkataramana N K. Aplasia cutis congenita of the scalp: Therapeutic modalities. Neurol India 2006;54:312-3 |
| » Introduction | |  |
Aplasia cutis congenita (ACC) is a heterogeneous disorder, all characterized by focal absence of the scalp and sometimes the calvarium and/or dura. We present a case of ACC whose mother was exposed to a teratogenic drug during pregnancy and review the relevant literature
| » Case Report | | |
A male child born at term was noticed at birth to have multiple scalp defects along the midline. He was born of a non-consanguineous marriage and there was no family history of congenital anomalies. The mother was on tablet Methimazole for thyrotoxicosis. Initially, regular dressings were performed. However, the scalp defects did not heal completely. The infant was referred to us when he was 3 months old. There were three lesions over midline scalp arranged in a linear fashion [Figure - 1]. The frontal lesion was the smallest, measuring about 1.2 cm in diameter. The second (posterior-frontal) was 3 x 2.5 cm in size, while the parietal was 4 x 3.5 cm in size [Figure - 1]. All the lesions were covered by a densely adherent thick black eschar. Neurologically, the child had no other obvious neurological deficits. He had short stubby toes in both the feet but no X-ray defects. The child was advised to continue dressing with Bacitracin ointment as review of literature revealed povidone iodine to be a desiccating agent and not recommended for usage in ACC. The anterior-most lesion healed completely over a period of 4 weeks. The posterior two lesions did not heal despite repeated dressings and there were several episodes of fresh bleeding from the lesions. The last episode of bleeding from the scalp defect was fairly severe and necessitated admission for definitive surgery.
A thorough saline-wash was performed and the eschars were gently separated. A full thickness pedicle rotation flap from the adjacent scalp was performed [Figure - 2]. The wound healed well over the following 2 weeks without residual defects.
| » Discussion | | |
ACC is a skin defect of multivariate etiology occurring at birth. Typically, the lesions appear as small ulcerations that usually heal spontaneously. Larger lesions may be associated with underlying bony lesions and can cause death secondary to infection or hemorrhage, especially if from the venous sinuses. In the past, birth trauma, congenital syphilis or skin avulsion by attached amniotic bands was considered a causative factor.[1],[2]
First described in 1826, Frieden classified ACC based on etiology and manifestation into nine groups.[3] Approximately 25% of the reported cases are familial, a vast majority (69%) showing an autosomal dominant inheritance. Our case fits into group 8, which is caused by viral infections and specific teratogens (e.g., Methimazole).[4],[5] Eighty-four percent cases of ACC involve the scalp, of which 75% are single. Most are situated in the midline. Extension through dermis, galea and bony calvarium occur in 35% of cases. When left untreated, these defects desiccate and form an eschar. As this dries, their edges retract, placing tension on the underlying dura. Frequent dressings tend to pull the eschar and cause frequent bleeds. If a tear occurs over the sagittal sinus, a life-threatening hemorrhage can result. The site often becomes secondarily infected. If there is a dural tear and the subarachnoid space is exposed, meningitis can result.
Management strategies are based on the size and presence of an underlying skull defect. Smaller eschars with intact calvarium can be allowed to heal spontaneously with routine wound care. Large lesions with an underlying skull defect require surgical closure to prevent massive hemorrhage. When primary closure is not possible, a full thickness vascularized pedicle graft can be utilized. Genetic counseling is useful when ACC is inherited. Systemic examination is necessary to rule out any other defects.[1],[5]
| » References | | |
| 1. | Robinson JC, Kelly Jr. Congenital defects of the scalp and skull. In : Textbook of Neurosurgery by Wilkins RH, Rengachary SS (Editor) 2nd ed. Mc Graw Hill: 1996. p. 3565-7.  |
| 2. | Bajpai M, Pal K. Aplasia cutis cerebri with partial acrania - Total reconstruction in a severe case and review of the literature. J Pediatr Surg 2003;38:e4. [PUBMED] [FULLTEXT] |
| 3. | Frieden IJ. Aplasia cutis congenita: A clinical review and proposal for classification. J Am Acad Dermatol 1986;14:646-60. [PUBMED] [FULLTEXT] |
| 4. | Karg E, Bereg E, Gaspar L, Katona M, Turi S. Aplasia cutis congenita after methimazole exposure in utero: Pediatr Dermatol 2004;21:491-4. [PUBMED] [FULLTEXT] |
| 5. | Kim CS, Tatum SA, Rodziewicz G . Scalp aplasia cutis with sagittal sinus hemorrhage . Arch Otolaryngol Head Neck Surg 2001;127:71-4. |
Figures
[Figure - 1], [Figure - 2]
| This article has been cited by | | 1 |
Aplasie cutanée congénitale et antithyroïdiens de synthèse au cours de la grossesse : série de cas et revue de la littérature |
|
| C. Sachs,M. Tebacher-Alt,M. Mark,B. Cribier,D. Lipsker | | Annales de Dermatologie et de Vénéréologie. 2016; 143(6-7): 423 | | [Pubmed] | [DOI] | | | 2 |
Treatment of multiple wounds of aplasia cutis congenita on the lower limb: a case report |
|
| M. Cherubino,F. Maggiulli,R. Dibartolo,L. Valdatta | | Journal of Wound Care. 2016; 25(12): 760 | | [Pubmed] | [DOI] | | | 3 |
Surgical management of aplasia cutis congenita |
|
| J.E. Betancourth-Alvarenga,F. Vázquez-Rueda,V. Vargas-Cruz,R.M. Paredes-Esteban,J. Ayala-Montoro | | Anales de Pediatría (English Edition). 2015; 83(5): 341 | | [Pubmed] | [DOI] | | | 4 |
Manejo quirúrgico de la aplasia cutis congénita |
|
| J.E. Betancourth-Alvarenga,F. Vázquez-Rueda,V. Vargas-Cruz,R.M. Paredes-Esteban,J. Ayala-Montoro | | Anales de Pediatría. 2015; 83(5): 341 | | [Pubmed] | [DOI] | | | 5 |
Aplasia Cutis Congenita |
|
| Eldad Silberstein,Vasileios A. Pagkalos,Daniella Landau,Alexander Bogdanov Berezovsky,Yuval Krieger,Yaron Shoham,Avraham Levy,Lior Rosenberg,Tali Silberstein | | Plastic and Reconstructive Surgery. 2014; 134(5): 766e | | [Pubmed] | [DOI] | | | 6 |
Consenso brasileiro para o diagnóstico e tratamento do hipertireoidismo: recomendações do Departamento de Tireoide da Sociedade Brasileira de Endocrinologia e Metabologia |
|
| Ana Luiza Maia,Rafael S. Scheffel,Erika Laurini Souza Meyer,Glaucia M. F. S. Mazeto,Gisah Amaral de Carvalho,Hans Graf,Mario Vaisman,Lea M. Z. Maciel,Helton E. Ramos,Alfio José Tincani,Nathalia Carvalho de Andrada,Laura S. Ward | | Arquivos Brasileiros de Endocrinologia & Metabologia. 2013; 57(3): 205 | | [Pubmed] | [DOI] | | | 7 |
The Brazilian consensus for the diagnosis and treatment of hyperthyroidism: Recommendations by the thyroid department of the Brazilian Society of Endocrinology and Metabolism [Consenso brasileiro para o diagnóstico e tratamento do hipertireoidismo: Recomendações do departamento de tireoide da Sociedade Brasileira de Endocrinologia e Metabologia] |
|
| Maia, A.L. and Scheffel, R.S. and Souza Meyer, E.L. and Mazeto, G.M.F.S. and de Carvalho, G.A. and Graf, H. and Vaisman, M. and Maciel, L.M.Z. and Ramos, H.E. and Tincani, A.J. and de Andrada, N.C. and Ward, L.S. | | Arquivos Brasileiros de Endocrinologia e Metabologia. 2013; 57(3): 205-232 | | [Pubmed] | | | 8 |
Hyperthyroidism and other causes of thyrotoxicosis: Management guidelines of the american thyroid association and American association of clinical endocrinoloigists |
|
| Bahn, R.S. and Burch, H.B. and Cooper, D.S. and Garber, J.R. and Carol Greenlee, M. and Klein, I. and Laurberg, P. and Ross McDougall, I. and Montori, V.M. and Rivkees, S.A. and Ross, D.S. and Sosa, J.A. and Stan, M.N. | | Endocrine Practice. 2011; 17(3): 456-520 | | [Pubmed] | | | 9 |
Hyperthyroidism and other Causes of Thyrotoxicosis: Management Guidelines of the American Thyroid Association and American Association of Clinical Endocrinoloigists |
|
| Rebecca S. Bahn,Henry B. Burch,David S. Cooper,Jeffrey R. Garber,M. Carol Greenlee,Irwin Klein,Peter Laurberg,I. Ross McDougall,Victor M. Montori,Scott A. Rivkees,Douglas S. Ross,Julie Ann Sosa,Marius N. Stan | | Endocrine Practice. 2011; 17(3): 456 | | [Pubmed] | [DOI] | | | 10 |
Aplasia Cutis Congenita : Clinical Management of a Rare Congenital Anomaly |
|
| Gaurav Bharti, Leslie Groves, Lisa R. David, Claire Sanger, Louis C. Argenta | | Journal of Craniofacial Surgery. 2011; 22(1): 159 | | [VIEW] | [DOI] | | | 11 |
Aplasia Cutis Congenita and Other Anomalies Associated with Methimazole Exposure During Pregnancy |
|
| CRISTINA RODRÍGUEZ-GARCÍA, SORAHAYA GONZÁLEZ-HERNÁNDEZ, ANGELA HERNÁNDEZ-MARTÍN, NURIA PÉREZ-ROBAYNA, ROSALBA SÁNCHEZ, ANTONIO TORRELO | | Pediatric Dermatology. 2011; : no | | [VIEW] | [DOI] | | | 12 |
Hyperthyroidism and Other Causes of Thyrotoxicosis: Management Guidelines of the American Thyroid Association and American Association of Clinical Endocrinologists |
|
| | | Thyroid. 2011; 21(6): 593 | | [VIEW] | [DOI] | | | 13 |
Aplasia cutis congenita |
|
| Iljin, A., Kruk-Jeromin, J. | | Advances in Clinical and Experimental Medicine. 2010; 19(1): 121-125 | | [Pubmed] | | | 14 |
Vertex ulcer and irregularity in the inferior extremities of a neonate | [Úlcera en vértex y anomalías en las extremidades inferiores de un neonato] |
|
| Pérez Valdez, C.M., Hernández Rousselin, M.A. | | Piel. 2009; 24(2): 99-100 | | [Pubmed] | | | 15 |
Úlcera en vértex y anomalías en las extremidades inferiores de un neonato |
|
| Carlos Manuel Pérez Valdez,Miriam Azucena Hernández Rousselin | | Piel. 2009; 24(2): 99 | | [Pubmed] | [DOI] | | | 16 |
Surgical treatment of aplasia cutis congenita of the scalp | [Chirurgiczne leczenie wrodzonego ubytku skóry owłosionej głowy] |
|
| Kruk-Jeromin, J., Iljin, A. | | Postepy Dermatologii i Alergologii. 2008; 25(3): 95-99 | | [Pubmed] | | | 17 |
Aplasia cutis congenita associated with cutis marmorata telangiectatica congenita, atrial septal defect, and epilepsy: A newly recognized syndrome? - A case report |
|
| Chen, J.-F., Chen, S.-C., Chiang, C.-P. | | Dermatologica Sinica. 2008; 26(3): 157-164 | | [Pubmed] | | | 18 |
Aplasia cutis congenita with skull defect in a monozygotic twin after exposure to methimazole in utero |
|
| Hideyuki Iwayama, Haruki Hosono, Hikaru Yamamoto, Makoto Oshiro, Norishi Ueda | | Birth Defects Research Part A Clinical and Molecular Teratology. 2007; 79(10): 680-684 | | [Pubmed] | [DOI] | |
|
|
|
|
|
|
|
|
