Craniosynostosis is the premature fusion of cranial vault sutures. It represents a complex situation involving not only the cranial vault but often the whole cranial bony sutures. Craniosynostosis are named according to the resulting head shape. It may be associated with other malformations as part of complex syndromes. The overall incidence is 3-5/10000 live births.
The treatment is usually surgical. In most instances, the indication for surgery is for cosmetic reasons. However with multiple Craniosynostosis, brain growth may be impeded by the unyielding skull. ICP may be pathologically elevated. Most cases of single suture involvement can be treated with linear excision of suture. Involvement of multiple sutures or skull has usually required combined efforts of a Neurosurgeon and craniofacial surgeons.
All patients were admitted in the department of neurosurgery, Liaquat University Hospital, Jamshoro, Sind , Pakistan on the basis of visible skull deformity. Patients were examined for signs of raised ICP and other congenital deformity. The records of patients were maintained till follow up.
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27 children were included in this study from 2002-2009. Age range was 1 year to 6 years. Male were 18(66.6%), female 9(33.3%). The common suture affected was coronal 12 (44.4%). 2 Children with craniostenosis were belonged to same family and all were presented with suture involvement. 03 (11.1%) death occurred due to hypothermia.
Early diagnosis, expert surgical techniques and per and post operative care for bleeding and temperature regulation prevent mortality and morbidity.
Craniosynostosis is a premature fusion of one or more cranial sutures. It can be present at birth but can be missed if mild. It usually manifests as an observable deformity within the first few months of life. The prevalence of Craniosynostosis has been estimated as 3-5 per 10000 births.1,2 It is the birth defect usually of unknown cause. Several potential risk factors have been identified in epidemiologic studies. Male sex, advanced maternal age, smoking, white race, nitro-stable drugs and certain occupations. Genetic association has been identified.3,4,5 Many teratogenic agents plays role in the development of craniosynostosis like diphenylhydantoin. Any agent that can cause ossification defects in the fetus including methotrexate and retinoic acid may cause craniosynostosis. Extrinsic forces in utero and abnormal position or early descent of fetal head in pelvis can result in craniosynostosis.6,7
Craniocynostosis can happen as an isolated defect (involvement of single suture) or as a part of syndrome. Saggittal suture affected in 40 to 60% of cases, coronal suture in 20-30% of cases, metopic in less than 10% of cases.8 Lambdoid synostosis is very rare.9 Craniosynostosis is also seen in the context of various syndromes . The most common syndromes encountered in clinical practice are Crouzon, Apert, Saethre-Chotzen, and Pfeiffer.
Abnormal head shapes produce because of restricted growth occur perpendicular to the fused suture and compensatory growth under non fused sutures. Growth of the calvarias is not the result of simple tissue growth at the level of suture rather bones of skull grow secondary to the brain growth.10,11
Premature fusions of suture results in cosmetic deformity and compromise of skull growth. Raised intracranial pressure has been reported in 50% of patients with multiple suture involvement and 10% in cases with single future involvement. Visual disturbances can occur in children with Craniosynostosis secondary to derangement of facial and skull base bony structures. In case of multiple suture involvement blindness can occur because of both raise ICP and facial skeletal deformity.12 Visual outcome is poor in patients who developed optic atrophy.13
No medical treatment exists for craniosynostosis. Indications for surgical treatment in the form of cranial vault reconstruction in the early months of life include progressive facial and cranial deformity, intracranial hypertension, and progressive exophthalmos threatening the eyes.
Over the three decades, various techniques for craniosynostosis have been tried. Otto recognized the first premature closure of sutures as a discrete clinical entity in 1830 and coined the term craniosynostosis.14 The first reported procedure for correction of craniosynostosis was performed in 1890 by Lannelongue.15 Later, Lane described the first strip craniectomy.16 There have been many new developments such as distraction osteogenesis, biodegradable miniplate fixation, and the development of minimally invasive endoscopic techniques.
Though the techniques are standardized, all principles are universally accepted. The individual surgeon's preferences, training and experience continue to modify the surgical correction of synostoses.
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Surgical technique start from strip carniectomy to the complete calvarial remodeling.17,18 Goal of surgery should be correction of form and functions with minimization of morbidity and mortality.
We have reviewed our experience in 27 patients who underwent surgery and discussed their results.
Material and Methods:
All case of craniosynostosis/craniostenosis admitted between 2002-2009 were included in this study. Patients were admitted either from OPD, as a referral or direct in the department of neurosurgery, Liaquat University Hospital, Jamshoro. Patients were diagnosed on examination of suture involvement and investigated by single x-ray and CT scan. Preoperative preparation with routine investigations and blood arrangement is routine in the ward. Following variable were analyzed: Age, Sex, Type of Suture involvement, and signs of raised ICP. Per-operative complications like blood loss, hypothermia and post-operative complications were recorded. SPSS method was used for data analysis. Patients were reviewed at followup period of 6 months to 1 year.
27 children included in this study. Age range was 1-6 years. Children were divided into three groups according to their age. Most of the children 13 (48.1%) came under 01 to 03 years age group.(Table-1) Male were 18 (66.6%) and Female 9 (33.3%). Most common suture involved was coronal 12 (44.4%) followed by saggittal 10 (37.03). Strip craniectomies were performed in all cases. A bicoronal flap was made and Scalp flap turned to supraorbital region. One patient died on operating table because of hypothermia and two chidren were expired on second day of surgery because of blood loss. No other significant morbidity found during post operative period except CSF leakage. Excellent results found in 02 patients with craniostenosis.
Craniosynostosis can be either primary from a problem with the involved suture or it can be secondary to position that children spend most of their time. Not all the children need surgery especially those with mild deformity while children with obvious deformity benefit from early surgery. Our catchments area is the rural site of Pakistan and people due to illiteracy goes to quake and initially mishandled. People in rural area believed that children who borne with small heads are Godâ€™s special creatures and source of pray, and compelled to live at shrines for their ill means. It is awful of the meditation of the people they make them their earning channels for their livelihood. Many children became mishandled because of lack of knowledge about these skull problems in community and less number of neurologist and neurosurgeons in rural areas of Pakistan. Cultural and socio-economic reason of mishandling are also involved.
Surgical technique can be suture release, strip Craniectomy, Craniofacial remodeling and orthognathic surgery in adolescence.19 In Our series, linear strip craniectomies used by author because there is no maxillofacial surgery team in our institute. Strip craniectomy releases pressure from brain and eyes and when needed, craniofacial reconstruction should be used. The timing of surgery for isolated non-syndromic craniosynostosis is controversial. We tried to avoid surgery in younger children less than 6 months age because of severe per-operative complications like blood loss and hypothermia.
In our study, maximum number of patients were under 3 years of age which is compatible with other international studies.2We tried to avoid surgery under 6 months of age because of severe complications like blood loss and hypothermia. Male sex was predominant in our study compatible to other international studies.19,20,21 Common suture involved was coronal followed by saggittal in our series., whish is contrary to the literature.19,22,23
We did suturectomies in all cases with extension into temporal and supraorbital regions.
During surgery, dura found attached to the skull bone and tear during suturectomy in many cases. This shows underlying raise ICP. Most significant post-operative complications found except CSF leakage from wound in two patients that were managed by repeated lumbar puncture.
Death occurred in 03 patients, 02 were male and 01 was female child. Female child was expired at operating table because of hypothermia. Patient was managed aggressively and every efforts taken but child could not revised. Same mortality also observed in literature.19
Early diagnosis, expert surgical techniques and per and post operative
care for bleeding and temperature regulation prevent mortality and morbidity.