Abdullah, Khalid Y. Salah M.D.
Here, we report a case of a patient with multiple suture craniosynostosis of varying degrees with clear scaphocephaly and premature closure of metopic suture. Patient also has a minimal degree of bilateral coronal premature closure (plageocephaly) . Scaphoocephaly means a premature fusion and ossification of the sagittal suture. Trigonocephaly is the same effect on the metopic suture. The calvarial growth restriction causes decreased intracranial volume. Thus, a surgical intervention is indicated to restore the volume of the skull along with appearance. Our patient's skull deformity was corrected by Strip sagittal, coronal and metopic suturectomy .
Key words: Craniosynostosis, Scaphocephaly, Sagittal synostosis, Metopic synostosis, Trigonocephaly, Plageocephaly, Treatment.
Craniosynostosis is defined as a premature fusion of single or multiple cranial sutures. It affects approximately 1 in 2,500 live births.[1, 2] Sagittal synostosis is the most common type, accounting for 40-55% of non-syndromic craniosynostosis.[3,4] It occurs as a result of premature fusion of the sagittal suture, so arresting skull growth in the transverse direction with increased growth in the anteroposterior direction, resulting in an anteroposterior elongation with frontal bossing and occipital prominence giving the skull a characteristic “long boat” shape, so termed scaphocephaly (derived from skaphos: Greek term for skiff). Metopic synostosis occurs in between 1/700 and 1/15.000 newborns.[4, 5] This results in a skull with a triangular forehead and deficient lateral orbital rims and supraorbital retrusion and bitemporal indentations. The net results in decreased intracranial volume necessitating a surgical correction to restore the volume and normal appearance of the skull.
A 7-months-old patient, the first child to his mother. He is the product of normal uncomplicated vaginal delivery post full term unremarkable pregnancy. Ever since his birth his mother noticed an abnormally shaped skull (Figures 1a,b). By 1 month age they visited the OPD neurosurgery clinic by reference from a pediatrician. The baby initial examination revealed an average weight baby with elongated skull and about 1*1 cm anterior fontanel and closed posterior fontanel with good activity and well feeding power with no focal neurological deficit or other associated visible anomaly. They were instructed to come back 2 months later with a cranial CT scan with 3D reconstruction which showed a fused sagittal and metopic sutures (Figures 2a–d); diagnosed as scaphocephaly and he was arranged electively for corrective surgery under general anesthesia. On table a bi-coronal skin incision was made. The scalp was dissected preserving the adjacent neurovascular structures. The temporalis muscles were dissected. Two Burr holes were done on both sides 2cm posterior to the coronal sutures and 3cm lateral to the midline. Using the electrical saw; Bilateral coronal sutures were cut and sagittal, and metopic stripping suturectomy was performed. Scalp was then closed by layers after securing hemostasis and putting a drain. Postoperatively the patient was observed in the ward and discharged 3 days later with no peri-operative complications were encountered. Post surgery computerized cranial tomography showed no extra complications (Figure 3a-d). Six months and 1 year later, he was followed up with a control computerized cranial tomography (Figures 4a-c).
Craniosynostosis can be classified as either primary or secondary. Primary craniosynostosis occurs due to genetic, environmental, or a combination of factors specifically affecting cranial sutures with no or little impact on the rest of the human body. Secondary craniosynostosis develops as a result of mechanical impacts, metabolic disorders such as hyperthyroidism, hypocalcaemia, vitamin D deficiency etc. affecting cranial sutures non-specifically, or due to premature suture closure as a result of the impaired developmental program that regulates brain growth. Deformational plagiocephaly is a common and somewhat benign cause of skull deformity in infants that must be distinguished from the more serious craniosynostosis, which occurs alone or as a syndrome. Patients with cranosynostosis are diagnosed either at birth or within a few months thereafter and should treated as soon as possible, preferably within their first year of life. If the condition is left untreated, craniosynostosis can lead to further anatomical and psychological sequelae revealing itself as skull shape deformity and other complications such as raised intracranial pressure[10,11] and cranial growth restriction. The treatment varies according to the type and time of presentation and mostly involves either proper sleep positioning as in deformational plageocephaly or surgery varying from less invasive procedures in those patients diagnosed early [12-14] to single or repeated open calvarial reconstruction in the more complex cases.[15-16] The care of patients with cranosynostosis should be provided by a multidisciplinary team.
Surgical techniques for craniosynostosis repair have evolved over time, starting by strip sutural release first done by Lannelongue in 1890. Mehner puplished a paper in 1921 and introduced the concept of suture resection as opposed to a simple “release”. in 1967 Paul Tessier, a plastic surgeon had shaped the modern operation techniques with a different perspective and shaped the craniofacial surgery practiced today. Tessier proposed multiple techniques of osteotomy, bone grafting, and direct correction of deformities.[18,19] The newest technique of correcting craniostosis by s imple suturectomy via endoscope with barrel staving and postoperative head helmets use was developed. It is introduced by Jimenez and Barone in 1990. Operative times and hospital stays are short. In general, blood loss is less than that which occurs in the open procedure. Cosmetic results are comparable, and reoperation rates are similar to those of the open procedure.[20,21] A wide range of surgical techniques are used for metopic and sagittal synostosis. This case is treated with simple stripping of coronal and metopic sutures.
Good cosmetic result and lesser complications can be achieved as a result of early correction of craniosynostosis. Multiple suture craniosynostosis can be corrected surgically with multiple suturectomy. Evident scaphocephally and metopic synostosis with minimal plageocephaly can be well corrected by Strip sagittal, coronal and metopic suturectomy.
5 . Rice DP. Craniofacial sutures. Development, disease and treatment. Preface. Front Oral Biol. 2008;12:xi.