The 20th Congress of RSCANP,

Băile Felix, 18-21.09.2019

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The 42st National Conference of Child and Adolescent Neurology and Psychiatry and Allied Professions with international participation


MODERN TREATMENT OF SCAPHOCEPHALY. EXPERIENCE BASED ON 120 CASES

Autor: D. Nica A. Mohan A. V. Ciurea H. A. Moisa
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Introduction
Craniosynostoses represent a group of birth defects that influence the structures of calvaria by the premature closure of one or of more of these structures so as to produce secondary craniofacial deformity. According to the classification proposed by Arseni, Horvath and Ciurea [1][2][3] craniosynostoses fall into three categories:

  • Simple – in which one or more sutures are characterized by premature synostosis;
  • Complex – the cranial dysmorphia is included in a plurimalformative complex;
  • Accompaniment – where the cranial dysmorphia is an epiphenomenon in other metabolic disorders .

Of the simple craniosynostosis conditions, scafocephaly has the highest incidence (40-60 % of cases) [4 ] i.e. when the premature synostosis of sagittal suture occurs. In these conditions, the skull acquires an elongated shape, with frontal and occipital bossing and biparietal narrow aspect.

Material and methods
The authors present a personal series of 120 cases of scaphocephaly diagnosed and operated between 1997-2012 (15 years). Cases were diagnosed in the authors’ hospitals of origin and treated in the Department of Paediatric Neurosurgery at the “Bagdasar – Arseni “ Emergency Hospital – Bucharest . Among the methods of surgical decompression of the skull and of removing the synostosed sagittal suture, the authors preferred to use classic extensive craniectomy of the Stein & Schut type. [5 ] All cases were monitored clinically , neurologically , ophthalmologically , neuropsychiatrically , by neuroimaging methods (mainly by CT scan with 3D reconstruction ) and, last but not least, from a neurosurgery perspective. Potential craniofacial changes, were assessed, too especially those of the palate and maxillofacial ones.

Results
In our statistics there was no case of postoperative mortality. Surgical outcomes assessed at 36 months after surgery were satisfactory in all cases, both neurologically, considering the psychomotor development, and aesthetically, considering the cosmetic appearance. Patients were followed for a period between 6 months and 5 years. In none of the cases there were changes of the palate or other maxillofacial modifications.

Introduction

Craniosynostosis conditions represent a heterogeneous group of neurosurgical malformations characterized by premature fusion of one or more cranial sutures, thus creating a complex craniofacial malformation whose adequate treatment involves neurosurgical intervention obligatorily. This phenomenon of premature closing results in a series of morphological alterations in the development of the craniofacial massive, the major consequence being the disproportion of the brain structures in relation to the skull .

R.Virchow [6] defines craniosyntoses as premature closures of sutures, followed by secondary deformities of the skull and subject to a law according to which “normal bone growth is inhibited in the direction perpendicular to the closed suture, while compensatory growth occurs parallel to the synostosed suture” . This postulate of Virchow partially retains its validity today, too. Virchow also [6] achieved a very correct framing of craniosyntoses that remained classic.

Very often the closing of a suture leads to a compensatory secondary deformity but this is not mandatory. On this consideration, premature synosthoses should not be defined according to the secondary deformation but only in relation to the suture or sutures affected initially. Craniosynostoses are common birth defects, occurring with a frequency of 1 in 2000 live births – according to Shilito & Matson [7] and Cohen [8]. Modern literature data show for scaphocephaly: an overall incidence of 0.4 / 1000 newborns, the gender ratio M / F = 3.5 / 1 and a rare presence of family cases.

Craniosynostoses are important for two reasons: they represent a significant health problem, seriously disrupting the child’s development in the absence of any treatment. The second reason is that, from a pathophysiological point of view, craniosynostoses are a very good model to study the contribution of genetic and/or environmental factors in producing malformations.

The molecular bases of most types of craniosynostosis are today known and the genetic tests allow an accurate diagnosis. The identification of genetic lesions has for the moment no direct impact on the treatment of such suffering, but allows for an accurate prenatal diagnostic. [9][10]

Craniosynostoses are divided, according to the scale proposed by Arseni, Horvath and Ciurea [3], into three groups:

 

  • Simple: one or more synostosed sutures.
  • Complex: the cranial dysmorphia is included into a multi-malformation complex.
  • Accompaniment: the cranial dysmorphia is minor, being an epiphenomenon within other diseases – metabolic or hematologic ones.

 

In all craniosynostoses, neurosurgery intervention has major indication, aiming first of all to decompress the brain structures and to remodel the calvaria so as to obtain a correct result both functionally and aesthetically. Sometimes it takes multiple surgeries to correct an abnormal blood flow and abnormal CSF circulation.

This type of surgery is relatively simple and is possible today due to the development of CT scan neuroimaging with three-dimensional reconstruction. For proper assessment, the patient is monitored both pre- and post-surgery. [1] [2] [3] [4]

Scaphocephaly is the most common form of simple of craniosynostosis – that is the premature closure of the sagittal suture. Due to the need for urgent surgical treatment the authors present this pathology, facilitating its understanding by any doctor.

Fig. 1 (a, b) Patient with Scaphocephaly. From the personal collection of Prof. A.V. Ciurea

Material and methods

The authors present 120 neurosurgical cases treated for scaphocephaly. These cases were diagnosed the authors’ hospitals of origin, were treated surgically and monitored in the “Bagdasar-Arseniy” University Emergency Hospital from Bucharest, in the paediatric neurosurgery department. To exclude a plurimalformative complex, in all patients, an oro-maxillo-facial consultation has been required. The study group included 94 boys and 26 girls aged between 6 and 30 weeks.

The cases were treated over a 15-year study period (1997-2012) Total number of craniosynostoses during this period was 231 cases, with scaphocephaly prevailing in 51.9% of cases (Figure 2 and 3).

Fig. 2 – Diagram of the types of scaphocephaly during the time interval 1997-2012

Figure 3. Types of craniosynostoses [7]

Among the commonly used laboratory investigations we mention the following:

  • cephalograms (graphs of skull circumference growth curve)
  • plain radiographs
  • CT scan
  • CT scan with 3D reconstruction
  • Nuclear Magnetic Resonance (NMR)
  • Prenatal Ultrasonography

Skull circumference growth curve is a very important element in assessing changes of the skull (see Figures 4, 5 and 6). Besides inspection, which is mandatory to be made as a routine, the standard deviations of the skull growth will attract immediate attention on some changes of the calvaria, and consequently to the possibility of the presence of scaphocephaly. The main cephalic indices that are usually modified are the transverse and the longitudinal diameters. Their ratio is often subunitary. In the cases studied, the recorded growth curve (Fig. 4,5 and 6) clearly illustrates cranial volume changes in the studied patients.

Figure 4: Skull circumference growth curve (top) and that of body weight growth (bottom) in male children between 0 and 24 months. (After the Center for Disease Control, USA).

Figure 5: Skull circumference growth curve (top) and that of body weight growth (bottom) in female children between 0 and 24 months. (After the Center for Disease Control, USA).

Figure 6. Skull circumference values in the studied cases.

Values are for 94 boys (top) and 6 females (bottom) and are correlated with age.

 

Computed tomography with 3D reconstruction is the most used, providing the physician with maximum information on deformities of the skull and of the synostosed suture. More importantly, it offers the possibility to plan the interventions of osteotomy and cranial remodelling. (Fig 7,8)

Fig. 7 Aspect of CT with 3D reconstruction – frontal view. From the collection of Prof. A.V. Ciurea

Fig. 8. Aspect of CT with 3D reconstruction – lateral view. From the collection of Prof. A.V. Ciurea

The simple computed tomography reveals to the physician the classic “boat skull” appearance (Figure 9)
Another advantage of native CT scan is the ability to assess the size of the ventricular system.

Figure 9: Native Computed Tomography: pathognomonic appearance – “boat skull.” From the personal collection of Prof. A.V. Ciurea

Regarding the value of MRI imaging in scaphocephaly , it allows assessment of the size and integrity of compressed neural and of ventricular structures. Current Gold Standard, the NMR and its derivatives evaluate all intracranial structures with high resolution, but any NMR in infants involves certain risks due to the required sedation. Precisely for this reason, in practice, it is preferred to use a CT scan with 3D reconstruction as a first option.

 

 

The Clinic

Surgery indications in scaphocephaly are:

 

  • intracranial hypertension syndrome
  • neurological deficiency syndrome (cranial nerve palsy)
  • Psychiatric disorders/ mental retardation
  • Visual Deficits
  • Aesthetics / cranial deformities

 

The patient’s positioning can be in dorsal decubitus with hyperextension of the cervical spine facilitating access to the front or occipital areas. Another possibility of surgical positioning is prone position that facilitates access to two-thirds of the posterior sagittal suture. The most commonly used was the prone positioning with broad access to the anterior and posterior portion of the sagittal suture. (Figure 10)

Fig. 10. Intraoperator positioning of the patient. From the personal collection of Prof. A. V . Ciurea

Among the surgical procedures known in scaphocephaly, we mention:

 

  1. simple linear craniectomy (2cm),
  2. Extensive craniectomies (6- 8cm),
  3. Craniectomies and reconstructive techniques.

 

We used as standard method the extensive craniectomy – after Stein & Schut [5]. In this procedure, an incision is made biparietally and the scalp is taken off. Subsequently we proceed with osteotomy of the synostosed sagittal suture with the execution of two secondary side osteotomies (anterior and posterior) (Figure 11) . This is done very carefully (preferably with a craniotome with water flow) and so as to avoid the damage of the superior sagittal sinus and prevent life-threatening bleeding . Of course, with the development of craniotomes and of high speed drilling procedures, the techniques of craniectomy followed by reconstruction became popular.

To assess the effectiveness of craniotomy, computed tomography with 3D reconstruction is performed both pre – and post – operatively (see Figures 12 and 13). Intervention ends with suturing the operative wound in two anatomical plans with external drainage.

Figure 11 (left) and b (right) : Removal of sagittal suture with two lateral osteotomies to expand the brain. From the personal collection of Prof. A.V. Ciurea

Figure 12. Aspect of CT scan with 3D reconstruction: Pre-operatively

From the personal collection of Prof. A.V. Ciurea

Figure 13. Aspect of tomography with 3D reconstruction. Post-operatively

From the personal collection of Prof. A.V. Ciurea

Surgery complications were found in 31 cases (25.8 %)

The main complication Number of cases Percent (%)
Seizures 13 10,8
Transient neurological deficit 2 1.6
Anaemic syndrome 26 21.6
Wound infection 2 1.6
Hemorrhagic Shock 1 1.6
CSF fistula 1 1.6

 

There was no postoperative death. Postoperatively, patients were transferred for 24h in the paediatric intensive care unit (PICU) with monitoring for at least 24 hours.

Surgical outcomes assessed at 18 months after surgery were satisfactory in all cases, both neurologically as psychomotor development, and aesthetically, as cosmetic appearance. Accurate assessment is based on CT scan with 3D reconstruction (Fig. 14 a, b ) In our series, there was no need to reopen the re-synostosed sagittal suture.

Fig.14 3D CT scan in case of scaphocephaly a (left) – Preoperatively and b (right) – postoperatively. From the personal collection of Prof. AV. Ciurea

 

 

Discussion

 

Scaphocephaly is the most frequent simple craniosynostosis, where an early fusion of the sagittal suture occurs but without association with other synostoses. The compensatory development induces a uniform and longitudinal elongation of the skull, with frontal and occipital bossing, thus inducing a secondary deformity. Literature data show an overall incidence of 0.4 cases per 1000 newborns and a predominance of male patients. The ratio between male and female patients is 3.5 / 1. Familial cases are rare. [9] [10]

Clinical examination revealed the pathognomonic sign of growing prominence in the mid- sagittal line of the skull. This deformity is observed at birth. Clinical diagnosis is put in the first days after birth.

Often, a prominent forehead is noticed and a disproportion between the front of the skull, which is increased, and the temporal region, which is narrowed. The overall appearance of the skull is in “boat shape” with a long anterior -posterior axis and a short latero –lateral one. Development of the facial massif is relatively normal.

Of the clinical signs, headache and emesis are rare. Intracranial hypertension syndrome occurs only in infants and is rare, too. For children over 3 years old, we find frequent seizures and visual dysfunction. There are remarkable neurological deficits, papilloedema or optic atrophy.

It is to be mentioned that scaphocephaly requires a multidisciplinary approach of the patient. A correctly composed team contains a neuro- radiologist, a craniofacial surgeon, a neurosurgeon , a paediatric anaesthesiologist. The orthodontist is not involved in the treatment of this condition, since there are no changes of implant dentistry.

Summing up the neurosurgical interventions in scaphocephaly, they are:

 

  • Simple linear craniectomy (2cm),
  • Extensive craniectomy (6- 8cm),
  • Other techniques of craniectomy and reconstruction.

 

It has to be noted that the simple linear craniectomy was first used to treat scaphocephaly[11]. It was followed by (strip) bilateral craniectomies [12], after which extensive (6- 8cm) craniectomies started to be used that allowed for the brain swelling, too. Among these techniques, we mention Raimondi technique [13] (1998 ) Venes & Sayers technique[14] (1976), Stein & Schut [5] (1977) , Albright [15] ( 1985). Stein & Schut technique consists of a large osteotomy of the sagittal suture with anterior and posterior side openings (Fig. 15)

Fig. 15 Stein & Schut surgery technique, 1977

 

 

The combined use craniectomy and reconstruction techniques are used in all scaphocephalies at present[16]. Thus, calvaria gets an appearance closer to the normal aspect. Of course, surgery performed as early as possible, in the first 6 months of life, allow effective cerebral decompression. Moreover, the interventions made in the first 3 months of life dramatically decrease the gap between patients and healthy children.

Besides the classical methods of surgical treatment, literature shows also minimally invasive surgical techniques. Of these, we mention endoscopic technique presented by Jimenez et. al. [17] in 2004. The endoscope was used in conducting an osteotomy of the sagittal suture in scaphocephaly at 4-6 weeks of life. Osteotomy was achieved by opening the anterior and posterior sagittal suture. The aim was to perform subcutaneous osteotomy assisted by the endoscope. The procedure is minimally invasive but can lead to the damage of the superior longitudinal sinus. The method addresses infants in the first 4-6 weeks of life and, postoperatively, the child has to wear a special helmet that will guide cranial bone regeneration.

Making a comparison between the two methods – the classical one (open surgery) versus the minimally invasive one, it is noted that the minimally invasive approach is accompanied by a lower intra-operative blood loss, less risk of infection, a shorter length of stay in hospital and a lower cost [18], items that overlap a better quality of the child’s life[19].

We should mention that scaphocephaly can be detected in prenatal ultrasound testing, which helps to solve surgery in the first three months of life (Fig. 16 and 17).

Figure 16 Patient with scaphocephaly – Pre-operative aspect (From the personal collection of Prof. A.V. Ciurea.)

Figure 17 Patient with scaphocephaly – Post-operative aspect (From the personal collection of Prof. A.V. Ciurea).

 

 

Conclusions

 

Scaphocephaly is the most common of craniosynostoses and consists in premature closure of the sagittal suture. Clinical and imaging diagnosis in these cases is extremely easy. Currently, we believe that genetic screening should be performed in all patients with craniosynostosis to evaluate exactly the transmission of cranial dysmorphias. [20]

Neurosurgical decompression is required in the first 3-6 months of life, the surgical window, the interventions at a later period become difficult due to intraoperative anaemic syndrome. The correct treatment of such patients requires the existence of a multidisciplinary team and a properly equipped neurosurgical centre. Modern techniques of cranial remodelling offer a very good therapeutic solution, with a low risk (compared to conventional techniques) .

Today, endoscopic treatment methods of scaphocephaly allow long operating duration at low risk of infection, decrease the need for repeated blood transfusions, have a lower cost than the traditional methods and decrease the duration of hospitalization of children, increasing the quality of life.

Early recognition of the impairment is the first step towards effective and perfect overall results that can be achieved in 95 % of cases.

 

Bibliography

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