CLINICAL STUDY
Novel Surgical Approach to Acalvaria
Diego J. Caycedo, MD,yz§ Juan P. Castillo-Huila, MD,y
James A. Zapata-Copete, MD, MSc,y and Luis Fernando Santacruz, MDz§
Abstract: Acalvaria, also known as acrania, is a rare congenital
anomaly which is characterized by a complete or partial defect of
the calvarium. Due to the low number of patients reported, there
is no a standard for the treatment of this condition; thereby, the
objective is to present a new surviving patient with acalvaria and
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a novel surgical approach to treat it, giving craniofacial surgeons
and neurosurgeons more tools to face it. In this article, the
authors present a reproducible surgical approach in patients with
partial acalvaria, which consist in use the present bone as a graft
to cover the defect, giving a temporal but stable coverture. No
other calvaria graft has been published along the available
literature, since only around 4 patients are acalvaria survivors
worldwide. However, the authors need to make a longer follow-
up to establish the usefulness of the technique in long term. Other
limitation is the future procedures needed to a better function and
aesthetic.
Key Words: Acalvaria, bone graft, craniofacial
(J Craniofac Surg 2021;32: 210–211)
A calvaria, also known as acrania, is a rare congenital anomaly
which is characterized by a complete or partial defects of the
calvarium,1 it is, absence of flat bones of skull, dura mater and
associated muscles, but normal cranial contents and facial bones.2 It
is postulated as a postneurulation defect;3 however, its cause have
not been elucidated and many theories have been proposed.2– 5
Previously, it was thought to be incompatible with life; however,
since the first known surviving patient was presented in 1996,1 other
patients have been reported with some suggestions, surgical and
nonsurgical approaches.2,3,5 –7
Due to the low number of patients reported, there is no a standard
for the treatment of this condition, thereby, our objective is to
present a new surviving a patient with acalvaria and a novel surgical
approach to treat it, giving craniofacial surgeons and neurosurgeons
more tools to face it.
From the Plastic Surgery Section, Surgery Department; yPLASTICUV
Research Group, Universidad del Valle; zImbanaco Medical Center; and
§Club Noel Children Hospital, Cali, Colombia.
Received March 2, 2020.
Accepted for publication May 24, 2020.
Address correspondence and reprint requests to Diego J. Caycedo, MD,
Universidad del Valle, Cali 4B#36-00, Colombia;
E-mail: diegojosecaycedo@gmail.com
The authors report no conflicts of interest.
Copyright # 2020 by Mutaz B. Habal, MD
ISSN: 1049-2275
DOI: 10.1097/SCS.0000000000006802
210 The Journal of Craniofacial Surgery  Volume 32, Number 1, January/February 2021
Copyright © 2020 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Clinical Report
JR is the first male newborn, from twin pregnancy of an
estimated 35 weeks 1 day of gestational age, was delivered from
a 35-year-old primigravida mother. This pregnancy was product of
in vitro fertilization and the delivery was performed by cesarean
section due to hypertensive crisis and podalic presentation.
During pregnancy, mother assisted to 9 prenatal controls and
presented 1 urinary tract infection. There were no other infectious
diseases. The neonate’s weight was 2.2 kg (percentile 3rd–10th),
length was 43 cm (percentile 10–50th) and cephalic diameter 30 cm
(percentile <10th). Apgar scores were 7 and 9 at 1 and 5 minutes,
respectively. During the clinical examination cranial asymmetry
and large areas of parietal and temporal bones were missing, with
oblique right craniofacial cleft. A rudimentary eye globe was
present in the left orbit accompanied by an eyelid hypoplasia. In
the right orbit, a mobile palpable was present (right eye) but no
palpebral fissure was formed. Airway was normal and it was
confirmed endoscopically. No alterations in lip or palate was
present (Fig. 1A-B).
Tomography confirmed partial cranial agenesis (Fig. 1C-D),
presence of both eyes was seen, with fronto-orbital tumor depend-
ing from eye globe.
Surgical Procedure
In supine position, antisepsis was made, and a careful design of
scalp flaps was made to avoid vascular territories lesions and to
allow final closure. Dissection was made until bone exposition in a
supraperiostic plain, with craniectomy contralateral parietal bone
graft bars are designed and taken in subperiostic plain (Fig. 2A-B).
Encephalocele was covered with dura mater and duraplasty with a
biosynthetic membrane (Fig. 2C). Autologous bone grafts are fixed
on the defect with absorbable plates and screws (Fig. 2D). Local
flaps were used to final closure, and no distant flaps were needed.
Tomography was taken for immediate control with adequate and
expected positioning (Fig. 3A).
Postoperative Follow-Up
Patient was transferred to neonatal intensive care unit to ensure
adequate control. Last control, previous to write this article, was at
22 months with no complication (Fig. 3B-C), and tomography
shows adequate integration of bone grafts (Fig. 3A).
DISCUSSION
Acalvaria is a very rare condition but no clear incidence have been
reported, and how was previously exposed a few patients are
available in literature, thus a standard for treatment is not available.
In this article, we present a reproducible surgical approach in
patients with partial acalvaria, which consist in use the present
bone as a graft to cover the defect, giving a temporal but stable
coverture. Other surgical approaches have been published, Hawasli
et al3 presented a patient which dermal generation template was
used; however, in our patient skin was no lacking and a primary
closure was allowed, furthermore they did not use anything to bone
defect correction.
The Journal of Craniofacial Surgery  Volume 32, Number 1, January/February 2021
FIGURE 1. (A-B) Presurgical patient phenotype. (C-D) Presurgical tomography.
FIGURE 2. (A-B) Calvaria graft donor area. (C) Meningeal coverage with
synthetic membrane. (D) Graft fixation with absorbable material.
Although nonsurgical management have been reported with
adequate patient evolution,2,5 we preferred the exposed approach
trying to contain the intracranial structures and at the same time
provide a better shape to patient head. We think the shape and
appearance of the head in patients with acalvaria is not something
purely aesthetic. A better appearance will allow a better social
interaction, leading to a better social skills and quality of life, not
only for the children but for the parents. However, this procedure is
temporal to mass contention, and future procedures have to be
performed for better aesthetic and stable results.
This study is a pioneer in this technique and approach, no other
calvaria graft have been published along the available literature,
# 2020 Mutaz B. Habal, MD
Copyright © 2020 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Acalvaria
FIGURE 3. (A) Left: immediate postsurgical tomography; right: postsurgical
tomography at 22 months. (B-C) Patient at 22 months.
since only around 4 patients are acalvaria survivors worldwide.
However, we need to make a longer follow-up to establish the
usefulness of the technique in long term. Other limitation is the
future procedures needed to a better function and aesthetic.
CONCLUSION
Acalvaria is a craniofacial malformation with bad prognosis. The
contralateral cranial vault graft is a feasible and reproducible
method for reconstruction in patients with partial acalvaria. We
recommend individualizing each patient taking into consideration
the available tissues and the expected outcomes. This is the 4th
survivor patient in the available literature.
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