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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 20  |  Issue : 2  |  Page : 166-168

Aplasia cutis congenita of the scalp: The success of conservative approach in treatment of a large defect


Department of Pediatric Surgery, Children's Hospital Zagreb, Zagreb, Croatia

Date of Web Publication29-Mar-2019

Correspondence Address:
Dr. Marko Baskovic
Department of Pediatric Surgery, Children's Hospital Zagreb, Ulica Vjekoslava Klaica 16, 10000 Zagreb
Croatia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_93_18

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  Abstract 


Aplasia cutis congenita is a rare malformation that usually involves the scalp. It may occur as an isolated defect or be combined with congenital malformations. We present a case of a female infant at the age of 2 days with aplasia cutis congenita of the scalp. Although there is no consensus about the treatment, we have decided for a conservative approach because sagittal sinus, large veins, or brain were not exposed. Ten months since the onset of conservative treatment, the aplastic area was almost completely cured.

Keywords: Aplasia cutis congenita, conservative approach, newborn, scalp


How to cite this article:
Barcot Z, Baskovic M, Car A, Juric M. Aplasia cutis congenita of the scalp: The success of conservative approach in treatment of a large defect. Indian J Paediatr Dermatol 2019;20:166-8

How to cite this URL:
Barcot Z, Baskovic M, Car A, Juric M. Aplasia cutis congenita of the scalp: The success of conservative approach in treatment of a large defect. Indian J Paediatr Dermatol [serial online] 2019 [cited 2019 Sep 15];20:166-8. Available from: http://www.ijpd.in/text.asp?2019/20/2/166/255217




  Introduction Top


Aplasia cutis congenita (ACC) is a rare, heterogeneous group of congenital disorders characterized by the focal or widespread absence of the skin. ACC can occur anywhere on the body; however, the vast majority of cases occur on the scalp midline. At birth, the lesions may have already healed with scarring or may remain superficially eroded to deeply ulcerated while in approximately 15%–30% of cases, the scalp defect is associated with a defect in the underlying bone and dura mater, with exposure of the brain and sagittal sinus.[1],[2] Clinical subtypes are characterized by their location and pattern of lesions, associated malformations, and mode of inheritance. Consequently Frieden classified ACC into nine groups on the basis of location of the lesions and associated congenital anomalies, although new classifications are also present.[3],[4] There is no consensus for early management and treatment modalities for large ACC cases.


  Case Report Top


We present a case of a female infant at the age of 2 days (birth 40 + 3 weeks, vaginal, birth weight 3530 g, birth length 52 cm, and Apgar score 10/10) which who has been moved from a maternity hospital to our institution due to aplasia cutis congenita of the scalp. She was born of the first regular pregnancy of a 29-year-old mother who had epilepsy in her youth and was on valproate therapy. At birth, in the parietal scalp area above the wide-open fontanelle, a 6 cm × 8 cm defect was observed [Figure 1] without associated malformations on the rest of the body. Ultrasound of the brain and heart was normal. On craniogram, the parietal bone was found to be partially underdeveloped [Figure 2]. Neurological status of the newborn was normal. Magnetic resonance brain made using standard techniques and T1 HIRES, and Blackbone technique was neat with sustained continuity of dura without signs of cerebral herniation. The possibility of intracranial hemangioma was excluded from the study. Initially, Staphylococcus aureus was isolated, and Garamycin therapy with vaseline was initiated twice a day. The wound was followed up daily for changes. One month after beginning of therapy the eschar was gradually demarcated while the smaller nonepithelialized granulation zone treated merbromin with the baths. A protective helmet (Moor orthotics and prosthetics) was created for the child to temporarily protect the brain and the soft tissue [Figure 3]. Successive treatments saw the reduction of the eschar with the closure of the bony segments. After 6 months, on the scalp, a well-developed subcutaneous tissue with the presence of capillary bleeding was observed [Figure 4]. One month following that Suprasorb A + Ag® (calcium alginate fiber, 60% mannuronic acid, 40% alginic acid, silver alginate fibers, and 1.5% ionic silver) and Suprasorb H® (polyurethane film with a hydrocolloid layer) (Lohmann and Rauscher) wound dressings were introduced in the therapy. The iodine cream and the Mepitel® (a gentle two-sided wound contact layer with Safetac®– the original less-pain contact layer with silicone adhesion) (Mölnlycke Health Care) were introduced into the therapy by removing the Suprasorb® wound dressing. Ten months since the onset of conservative treatment, the aplastic area was almost completely cured [Figure 5].
Figure 1: Newborn at the age of 2 days with scalp defect (6 cm × 8 cm)

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Figure 2: Craniogram with poorly developed parietal bone

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Figure 3: Specially designed protective helmet

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Figure 4: Well-developed subcutaneous tissue with the presence of capillary bleeding

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Figure 5: Ten months since the onset of conservative treatment, the aplastic area was almost completely cured

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  Discussion Top


ACC is an uncommon disorder. The true incidence is unknown but has been estimated around 1–3 in 10,000 live births.[5] ACC is likely to be underreported, as milder cases often go undetected. Some studies report a slightly higher female predominance.[6],[7] Approximately 85% of ACC cases present as isolated scalp lesions which was also recorded in our patient.[8] Most cases of ACC occur sporadically, although familial cases have been reported. Although the mother used valproate when she was a child which has also been mentioned as a teratogenic factorin ACC but she did not use the same in pregnancy,[9] the mother did not use any medication during pregnancy. The estimated mortality rate among patients with large ACC ranges from 20% to over 50%.[1] Although in our case the parietal bone was not completely missing but large (>1 cm) and irregular defects are often associated with defects of the underlying bone, dura, and intracranial vasculature. Sometimes, enlarged transcranial veins and arteriovenous malformation can be present.[10] Newborns with large bone defects have a high risk. Major complications that can occur in large defects of ACC involving the bone and dura include hemorrhage from sagittal sinus or enlarged veins, sagittal sinus thrombosis, local infection, meningitis, and leakage of cerebrospinal fluid. Brain herniation and infarction have also been reported. These complications are associated with a high mortality rate, ranging from 10 to 50 percent of cases.[1] In our case, complications were not recorded. Given the high variability in the clinical presentation and severity of ACC, the approach to the treatment must be individualized based on the size and depth of the defect, presence of associated abnormalities, risk of complications, and clinical experience. Treatment options range from nonintervention, conservative treatments, surgical treatment, or a combination of surgical and nonsurgical interventions.[1],[8] Large defects of the scalp or scalp defects that involve the underlying skull or dura, with exposure of sagittal sinus, large veins, or brain, may require surgical intervention due to the high risk of infection, hemorrhage, or thrombosis. However, due to the high morbidity and mortality risk associated with surgery itself and general anesthesia, estimated to be approximately 14%,[11] there is no agreement among experts on whether surgery should be performed immediately or delayed after initial conservative management. Considering that there was no exposure of sagittal sinus, large veins, or brain, we decided for a conservative approach. A conservative approach has been traditionally used for small scalp defects (≤1 cm in diameter with intact dura, no sagittal sinus involvement, and no large vascular malformation). However, there are several reports of successful conservative management of large scalp defects with or without bone involvement.[12],[13],[14],[15],[16],[17],[18] In lesions involving the bone, if the dura mater is intact, conservative treatment may promote the osteogenic potential of the bone surrounding the defect, which we noticed in our patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given consent for images and other clinical information to be reported in the journal. The patient's parents understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Acknowledgment

We are thankful to the family of our patient for their support and cooperation.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Bharti G, Groves L, David LR, Sanger C, Argenta LC. Aplasia cutis congenita: Clinical management of a rare congenital anomaly. J Craniofac Surg 2011;22:159-65.  Back to cited text no. 1
    
2.
Humphrey SR, Hu X, Adamson K, Schaus A, Jensen JN, Drolet B, et al. A practical approach to the evaluation and treatment of an infant with aplasia cutis congenita. J Perinatol 2018;38:110-7.  Back to cited text no. 2
    
3.
Frieden IJ. Aplasia cutis congenita: A clinical review and proposal for classification. J Am Acad Dermatol 1986;14:646-60.  Back to cited text no. 3
    
4.
Silberstein E, Pagkalos VA, Landau D, Berezovsky AB, Krieger Y, Shoham Y, et al. Aplasia cutis congenita: Clinical management and a new classification system. Plast Reconstr Surg 2014;134:766e-74e.  Back to cited text no. 4
    
5.
Martinez-Regueira S, Vazquez-Lopez ME, Somoza-Rubio C, Morales-Redondo R, Gonzalez-Gay MA. Aplasia cutis congenita in a defined population from Northwest Spain. Pediatr Dermatol 2006;23:528-32.  Back to cited text no. 5
    
6.
Demmel U. Clinical aspects of congenital skin defects. I. Congenital skin defects on the head of the newborn. Eur J Pediatr 1975;121:21-50.  Back to cited text no. 6
    
7.
Sagi EF, Linder N, Shouval D. Papular acrodermatitis of childhood associated with hepatitis A virus infection. Pediatr Dermatol 1985;3:31-3.  Back to cited text no. 7
    
8.
Sánchez-Pedreño Guillén P, Rodriguez Pichardo A, Camacho Martínez F. Aplasia cutis congenita. J Am Acad Dermatol 1985;13:429-33.  Back to cited text no. 8
    
9.
Hubert A, Bonneau D, Couet D, Berthier M, Oriot D, Larrègue M, et al. Aplasia cutis congenita of the scalp in an infant exposed to valproic acid in utero. Acta Paediatr 1994;83:789-90.  Back to cited text no. 9
    
10.
Baselga E, Torrelo A, Drolet BA, Zambrano A, Alomar A, Esterly NB, et al. Familial nonmembranous aplasia cutis of the scalp. Pediatr Dermatol 2005;22:213-7.  Back to cited text no. 10
    
11.
Santos de Oliveira R, Barros Jucá CE, Lopes Lins-Neto A, Aparecida do Carmo Rego M, Farina J, Machado HR, et al. Aplasia cutis congenita of the scalp: Is there a better treatment strategy? Childs Nerv Syst 2006;22:1072-9.  Back to cited text no. 11
    
12.
Harvey G, Solanki NS, Anderson PJ, Carney B, Snell BJ. Management of aplasia cutis congenita of the scalp. J Craniofac Surg 2012;23:1662-4.  Back to cited text no. 12
    
13.
Alexandros B, Dimitrios G, Elias A, Evangelos D, Andreas M, Sotirios P, et al. Aplasia cutis congenita: Two case reports and discussion of the literature. Surg Neurol Int 2017;8:273.  Back to cited text no. 13
    
14.
Fröjd V, Maltese G, Kölby L, Tarnow P. Conservative healing of an 11 × 9-cm aplasia cutis congenita of the scalp with bone defect. J Neurol Surg Rep 2014;75:e220-3.  Back to cited text no. 14
    
15.
Orgun D, Horiguchi M, Hayashi A, Shimoji K, Arai H, Mizuno H, et al. Conservative treatment of large aplasia cutis congenita of the scalp with bone defect with basic fibroblast growth factor application. J Craniofac Surg 2017;28:e154-8.  Back to cited text no. 15
    
16.
Goncalves JF, Silva TM, Macedo I. Aplasia cutis congenita of the scalp with sagittal venous sinus exposure. Arch Dis Child Fetal Neonatal Ed 2016;101:F283.  Back to cited text no. 16
    
17.
Rhee ST, Colville C, Buchman SR, Muraszko K. Complete osseous regeneration of a large skull defect in a patient with cutis aplasia: A conservative approach. J Craniofac Surg 2002;13:497-500.  Back to cited text no. 17
    
18.
Bang RL, Ghoneim IE, Gang RK, Al Najjadah I. Treatment dilemma: Conservative versus surgery in cutis aplasia congenita. Eur J Pediatr Surg 2003;13:125-9.  Back to cited text no. 18
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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