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The repair of frontal encephaloceles in Papua New Guinea
Abstract
Frontal encephaloceles are common in Papua New Guinea (PNG). Seventeen cases collected over 7 years are reported. Eleven frontal (sincipital) encephaloceles were repaired successfully via an extracranial approach. One of these patients with a frontonasal encephalocele developed a recurrence following the extracranial approach, which was subsequently repaired intracranially. Three patients with small naso-ethmoidal encephaloceles were repaired intracranially via an extradural approach. The other three cases have not yet had surgical correction. Complex craniofacial surgery which corrects hypertelorism as well as the encephalocele is unavailable in the developing world. For the general surgeon in the developing world, the extracranial approach is recommended for the frontonasal encephaloceles, and the intracranial approach for the naso-ethmoidal and naso-orbital encephaloceles. Hydrocephalus and epilepsy have not developed in the patients.
... 2,11 Given the high complexity of the surgery as well as the nascent state of the health care systems and lack of advanced medical infrastructure, expedited treatment of the disease might unfortunately not always be feasible in many Asian countries, such as Burma, 11,16 Thailand, 2,3,10,26,38,39 Cambodia, 9,12,33,42 Malaysia, 1 Indonesia, 17,35 and Papua New Guinea. 18 However, it has been shown that treating affected patients under such circumstances can be made possible through the work of joint ventures between local surgeons and NGOs. To do this effectively, highly motivated local surgeons remain an indispensible resource. ...
OBJECTIVE
Although rare, frontoethmoidal meningoencephaloceles continue to pose a challenge to neurosurgeons and plastic reconstructive surgeons. Especially when faced with limited infrastructure and resources, establishing reliable and safe surgical techniques is of paramount importance. The authors present a case series in order to evaluate a previously proposed concise approach for meningoencephalocele repair, with a focus on sustainability of internationally driven surgical efforts.
METHODS
Between 2001 and 2016, a total of 246 patients with frontoethmoidal meningoencephaloceles were treated using a 1-stage extracranial approach by a single surgeon in the Department of Neurosurgery of the Yangon General Hospital in Yangon, Myanmar, initially assisted by European surgeons. Outcomes and complications were evaluated.
RESULTS
A total of 246 patients (138 male and 108 female) were treated. Their ages ranged from 75 days to 32 years (median 8 years). The duration of follow-up ranged between 4 weeks and 16 years (median 4 months). Eighteen patients (7.3%) showed signs of increased intracranial pressure postoperatively, and early CSF rhinorrhea was observed in 27 patients (11%), with 5 (2%) of them requiring operative dural repair. In 8 patients, a decompressive lumbar puncture was performed. There were 8 postoperative deaths (3.3%) due to meningitis. In 15 patients (6.1%), recurrent herniation of brain tissue was observed; this herniation led to blindness in 1 case. The remaining patients all showed good to very good aesthetic and functional results.
CONCLUSIONS
A minimally invasive, purely extracranial approach to frontoethmoidal meningoencephalocele repair may serve well, especially in middle- and low-income countries. This case series points out how the frequently critiqued lack of sustainability in the field of humanitarian surgical missions, as well as the often-cited missing aftercare and dependence on foreign supporters, can be circumvented by meticulous training of local surgeons.
... 22 Some claim that FMEC can be corrected only by an extracranial procedure. 4,24 We think this was done in developing countries in past years where surgical expertise was lacking and perioperative care was deficient. ...
Introduction:
Frontoethmoidal meningoencephalocele (FMEC) is a rare congenital anomaly characterized by herniation of brain tissue and meninges through a defect in the cranium and associated with facial dysmorphism. Treatment modalities include extra cranial, transcranial, or combined craniofacial approaches. The combined approach is considered the best treatment choice.
Methods:
Twelve patients with FMEC aging from 6 months to 4 years were treated by single-stage combined craniofacial approach between July 2011 and July 2015. They were followed up for evaluation of outcome and detection of complications.
Results:
Seven patients (58.3%) were males and 5 patients (41.7%) were females. Eight patients (66.7%) were less than 2 years while 4 patients (33.3%) were between 2 and 4 years. The main presentations were external mass, telecanthus and hypertelorbitism, radiologically, frontobasal bone defect and herniated dural sac with brain tissue were detected in all patients. Excision of the mass with dural repair, craniofacial reconstruction, and medial canthopexy were done for all patients. Orbital translocation was done for 8 patients (75%), nasal reconstruction for 7 patients (58.3%), while dacryocystorhinostomy in 3 patients (25%). Venticuloperitoneal shunt was done before correction of FMEC in one patient (8.3%). The follow-up period ranged from 6 to 48 months with mean 29.2 months. The esthetic results were satisfactory in 9 patients (75%). Ugly facial scars were recorded in 3 patients (25%).
Conclusion:
Early surgical management for FMEC is advisable to avoid deleterious effects on facial growth. Meticulous perioperative care is important for successful surgery. The authors recommend combined craniofacial approach to achieve good outcome and decrease the incidence of complications.
... The prognosis for the anterior encephalocele patient is generally good and is usually associated with normal intelligence and motor development. However, mental retardation, epilepsy and ocular problems have been described in this group [3]. Poor prognostic indicators include a large or posterior encephalocele and systemic anomalies. ...
Encephalocele is a rare and serious deformity that occurs by a protrusion of neural tissue (usually like a sac) through a bony defect in the skull, there are two types congenital (primary) and acquired (secondary) encephaloceles, congenital encephalocele belong to the neural tube defect (NTDs) along with spina bifida and anencephaly. The exact cause of congenital encephalocele is unknown, probably, it is multifactorial and there are roles for genetic and environmental factors, failure of closure of the neural tube during early pregnancy (third to fourth week) is thought the pathogenesis process of congenital encephalocele. According to the contents herniated sac, encephalocele is divided into many subtypes including meningocele, men-ingo-encephalocele, atretic encephalocele, and gliocele. The main classification of encephalocele includes four major types according to the location: occipital, Sincipital, basal, and convexity. The most common type of them depends on geographical region. Usually, encephalocele is present at birth as a visible mass but it may be occult like some cases of basal encephalocele. Ultrasound is the main diagnostic tool for prenatal diagnosis of encephalocele in early pregnancy during the first trimester. MRI plays a major role in diagnosis after birth as MRI demonstrates the contents of the herniated sac and aids in making the surgical decision. in most cases, surgery is the appropriate choice and it is an emergency if there is a risk of meningitis like CSF leakage. Hydrocephalus is the most common complication associated with encephalocele and it should be resolved before the surgery. Morbidity and mortality of encephaloceles have improved in recent years.
Giant Encephalocele is a rare entity in which the central nervous system structures in any combination protrude through a defect in the skull and the size of the head is smaller than that of the deformity. A few cases are reported globally. It could be existed alone or in combination with other central nervous system (CNS) anomalies. We report a case of 4 months old child with giant encephalocele associated with hand deformity. She underwent excision of the sac and the patient is doing well after 3 months follow up.
Giant occipital encephalocele is an uncommon condition. The sheer size of the lesion poses a challenge to the surgeon and the anesthetist. Here, we present a 4-month-old girl child presenting with a progressively increasing swelling in the occipital region since birth. The child after evaluation underwent repair of the encephalocele. The purpose of this report was to highlight the difficulties faced during surgery while operating on such a huge occipital encephalocele.
Encephaloceles are the result of a congenital cranial defect that allows intracranial contents to herniate. The herniated contents may consist of the meninges (meningocele), meninges, and brain (meningoencephalocele), or in severe cases meninges, brain, and part of the ventricular system (hydroencephalomeningocele). Encephaloceles are classified by their anatomic location. The vast majority (75 %) are located in the posterior cranial fossa. However, it is the anterior cranial fossa (sincipital) encephalocele that is the concern of the plastic surgeon, as it is considerably more deforming. These rare deformities are more common in Southeast Asia (1:6,000 births) and are most appropriately treated by a multidisciplinary team.
This is the first of the two parts of a paper concerning a novel method on the surgical treatment of orbital dysmorphisms, especially telecanthus (TO and hypertelorism (HT). The normal orbital values of a given ethnic group is an essential factor in determining the degree of correction intended in that patient population. We did not find any data related to the normal orbital values in Khmer-Cambodians in whom we performed the corrective surgeries. Thus, the aims of this article are to evaluate the orbital morphometric data procured in Khmer-Cambodians and to analyze the contradictory definitions of TC and HT found in the literature. We measured the inner canthal distance, outer canthal distance, and interpupillary distance in 688 Khmer-Cambodians. The measured normal values are presented and compared with other Asian populations. We discovered that the Khmer-Cambodian orbital morphometry did not resemble the general conception of an Asian appearance, but rather showed a similarity to values found in Indians. Telecanthus and hypertelorism are frequent orbital dysmorphisms that, however, find conflicting definitions in the literature. By means of a short literature review, we have attempted to reorganize the multiplicity of definitions in orbital measurements, as well as clarify the confusing terminology used in TC and HT.
Background:
Papua New Guinea (PNG) is a developing Pacific Nation of 7.3 million people. Although neurosurgery training was introduced to PNG in the year 2000, it was in 2003 that a neurosurgery service was established. Prior to this time, neurosurgery in PNG was performed by general surgeons, with some assistance from visiting Australian neurosurgeons. Neurosurgical training was introduced to PNG in 2000. The model involved a further 3 years of training for a surgeon who had already completed 4 years of general surgical training. We aim to review the output, outcomes and impact achieved by training the first national neurosurgeon.
Methods:
The data on activity (output) and outcomes were collected prospectively from 2003-2012. Ongoing mentoring and continuing professional development were provided through annual neurosurgical visits from Australia. There were serious limitations in the provision of equipment, with a lack of computerized tomographic or MR imaging, and adjuvant oncological services.
Results:
There were 1618 neurosurgery admissions, 1020 neurosurgical procedures with a 5.74 % overall mortality. Seventy percent of cases presented as emergencies. There were improved outcomes, particularly for head injuries, whilst hydrocephalus was managed with an acceptable morbidity and revision rate.
Conclusions:
The training of a neurosurgeon resulted in PNG patients receiving a better range of surgical services, with a lower mortality. The outcomes able to be delivered were limited by late presentations of patients and lack of resources including imaging. These themes are familiar to all low- and middle-income countries (LMICs) and this may serve as a model for other LMIC neurosurgical services to adopt as they consider whether to establish and develop neurosurgical and other sub-specialist surgical services.
Introduction:
Encephalocele is a rare congenital malformation of the central nervous system with protrusion of cranial content (meninges, brain, and ventricles in different combinations) beyond the normal confines of the skull. Anterior encephaloceles occur with a high frequency in Southeast Asia, while in the Western countries occipital encephaloceles prevail. The treatment of an anterior (naso-ethmoidal) encephalocele involves a neurosurgeon or a multidisciplinary team (neurosurgeon, maxillofacial surgeon, plastic surgeon, and ENT surgeon) dealing with craniofacial surgery. Goals of surgery include removal or repositioning of nonfunctional cerebral tissue, closure of the dura, and reconstruction of skeletal and cutaneous structures. The prognosis depends from the anatomical site, volume of neural contents, and the presence of coexisting malformations.
Case report:
We report the case of an Italian child suffering from a naso-ethmoidal encephalocele with bilateral orbital extension. The surgical treatment was performed in two steps.
Conclusion:
Sincipital encephalocele is a complex pathology without a unique standardized surgical treatment. Its low incidence in Western countries can make its management particularly tricky.
Frontoethmoidal encephaloceles are herniations of the intracranial contents through a defect in the skull at the junction of the frontal and ethmoidal bones. They are generally classified as nasofrontal, nasoethmoidal, and naso-orbital, although there may be some overlap or multiplicity. The records of 35 patients treated for frontoethmoidal encephaloceles were examined. Of these, 12 cases with complete and accurate medical records were evaluated in detail. The successful correction of frontoethmoidal encephaloceles was shown to depend on the following: a detailed understanding of the pathological anatomy (such as interorbital hypertelorism rather than true orbital hypertelorism and the presence of secondary trigonocephaly), careful planning of the bone movements to correct these deformities, and attention to detail regarding the placement of scars, positioning of the medial canthi, and the nasal reconstruction. Avoiding the “long-nose” deformity often seen after repair should be a priority. In general, the authors recommend a one-stage repair with both a transcranial and external approach.
Frontoethmoidal mengingoencephaloceles (fMECs) are frequently observed in Cambodia, especially in poor families. The authors describe issues related to the surgical treatment of fMECs in Cambodia at the end of a humanitarian program that provided surgery free of charge to patients and their families.
The authors reviewed 257 cases of fMEC involving patients who presented to their institution, the Children's Surgical Center in Phnom Penh, between 2004 and 2009. They treated 200 of these patients surgically (108 males, 92 females; 89% younger than 18 years) using a "low-cost" management plan with no routine pre- or postoperative investigations. Initially, surgery was performed by visiting foreign surgeons who taught the procedures to resident surgeons. Patients were not charged for consultations or treatment and received at least 1 follow-up examination 6 months postoperatively.
The nasoethmoidal type was the most frequent fMEC encountered (69%). Many patients had associated ophthalmological issues (46% of cases). Only 1 familial case was detected. Combined neurosurgical and facial procedures were successfully standardized and learned by surgeons initially unfamiliar with fMEC management. A neurosurgical approach avoided the need for a facial incision in 42 cases, improving cosmetic results. The most common postoperative issues were a temporary CSF leak (24 cases [12%]) and/or infection (28 cases [14%]). There were 3 deaths directly related to the operations. Cosmetic results were good in 145 cases, average in 27, poor in 7, and worse than preoperative appearance in 6 patients. Fifteen patients were lost to follow-up. The parents of 87% of the children were rice farmers. Questionnaire results confirmed that fMEC has important social and educational consequences for the affected children and that these consequences can be partially improved by fMEC correction.
This experience in fMEC management demonstrates that local surgeons can treat these malformations with limited surgical materials and in a nonspecialized infrastructure after principles of treatment have been learned and if they are carefully respected. Surgery for fMEC can thus be more accessible to a larger number of patients in developing countries. Moreover, local treatment facilitates better postoperative and follow-up care.
An audit of neurosurgery in Papua New Guinea (PNG) based on the experience of a visiting neurosurgeon is presented. The objectives of the study were to determine the type and frequency of neurosurgical conditions in PNG, whether major neurosurgery can be performed successfully in PNG, and to develop a strategy for the development of neurosurgical services in PNG.
The audit was carried out over two periods of 2 weeks duration in 1992 and 1993 in Port Moresby and Goroka. Instrumentation and equipment were limited and no additional equipment was used. Myelography and angiography were available in Port Moresby.
There were 82 patients in total, 55 (67.1%) were consultations, 23 (28%) had elective surgery, four (4.8%) had emergency surgery and 16 (19.5%) await surgery. Cases were subdivided into nine major groupings: neurotrauma 18 (seven severe); spine 18; congenital 13; hydrancephaly four; scalp, skull and orbit six; vascular three; peripheral nerve three; and neurology five. Seventy-two (87.8%) patients required CT/MRI which were unavailable. Four ventriculograms were performed in lieu of CT/MRI. Fifteen (18.3%) cases could not be treated in PNG.
Neurosurgical problems in PNG can often be adequately managed with limited resources. Complex procedures were performed with gratifying results and acceptable morbidity. There is sufficient pathology in a country of four million people to justify training a small number of neurosurgeons by the end of the decade. CT scanning will be a necessary adjunct.
Papua New Guinea (PNG) is a country of 4.5 million people with an annual health budget of only 96 million Kina (1K = US$0.35). There are 19 hospitals in the country and national surgeons are now staffing most of these hospitals. This review aims to describe the surgical pathology in the year 2000 and the capability of PNG surgeons to manage it.
A review of publications, reports and surgical audit data on surgery in PNG was conducted. Surgical audit has been computerized for over 5 years. The review also draws on personal experience and data from MMed theses submitted to the University of Papua New Guinea.
Surgical pathology Surgical practice in PNG remains very general. Late presentation and advanced disease are common. Trauma, infection, malignancy and congenital anomalies dominate the surgical scene. The pattern of disease is different from what is found in the West. Western diseases are emerging with the incidence of appendicectomy rising from 5/100,000 to 75/100,000 in the past 30 years. The incidence of diabetes and gallstones has also risen. Osteoporosis, Colles' and neck of femur fractures are rare. Surgical capability The standard of surgical care is acceptable with a low wound infection rate for clean and clean-contaminated abdominal surgery of 0.9% and an anastomotic leak rate of 1.6%. Transurethral prostatectomy is also being performed to a satisfactory standard for head injuries admitted with a Glasgow Coma Score of 6-8 and a good outcome is achieved in over 70% of cases. Hospital mortality for surgical admissions is 3.7%. Subspecialties in orthopaedics, urology and head and neck surgery have been established. Neurosurgery, paediatric and cardiac surgery are being developed. Priorities for the next decade Papua New Guinea needs to continue to develop surgical subspecialties, particularly paediatric and neurosurgery, while maintaining a broad competence in general surgery. Services for burns, spinal injuries, rehabilitation and oncology need to be improved. Surgeons need to be more involved in rural health and teaching basic skills to primary health-care workers. Acquisition, maintenance and repair of surgical equipment needs to be improved so that PNG's well-trained surgeons can have the right tools for their trade.
Papua New Guinea offers a wide range of surgical pathology. The standard of surgery in PNG is reasonable but there are many areas that need development during the period of the next national health plan, 2001-2010. Australasian surgery has many opportunities to assist surgeons in PNG to achieve their objectives.
Anterior encephalocele is a rare condition, and only a few large series have been published in the literature. Surprisingly, the incidence is much higher in Southeast Asian countries, including some parts of India. While the reported incidence in the West is between 1:35,000 and 1:40,000 live births, it is as high as 1:5,000 live births in Thailand. We present a series comprising 92 cases of anterior encephaloceles treated over a 30-year period (1971-2000). Frontoethmoidal encephaloceles are the commonest type, followed by the nasopharyngeal and orbital type. Among the frontoethmoidal encephaloceles, nasoethmoid is the commonest type, and these patients present with swelling over the bridge of the nose with significant hypertelorism and orbital deformities. The nasopharyngeal type remains occult and presents with nasal obstruction or CSF rhinorrhea. Rarely, the patient may present with meningitis. Since 1978, computed tomography (CT) scans have regularly been performed in our patients. CT scans delineate the skull defect and associated brain anomalies. There was associated hydrocephalus present in 12 patients and agenesis of the corpus callosum in 5 patients. In all patients, one-stage repair of the encephalocele and correction of bony anomalies by appropriate osteotomy was undertaken. Since 1988, in cases of frontoethmoidal encephalocele with significant hypertelorism, medial advancement of the medial half of the orbits on either sides was carried out, instead of a classical Tessier's operation. Postoperative morbidity included CSF leak in 20 patients, wound infection in 2 and chest infection in 3. There were 3 deaths in our study. The overall cosmetic outcome was good.
A 3-month-old male infant was found to have a protruding mass with irregular contour at the root of his nose since birth. The mass protruded more when he cried. It was diagnosed and treated as a hemangioma by laser surgery in another hospital. However, the lesion recurred 1 week later. Reviewing the previous computed tomographic study of the brain, it revealed that the protruding mass communicated with the brain. Sincipital encephalocele was diagnosed, and craniotomy for excising the encephalocele and repair of the dura were undertaken successfully. We report this patient with sincipital encephalocele that was misdiagnosed and treated as a vascular malformation on the face. Fortunately, there was no complication. The authors stress the importance of accurate diagnosis of a lesion near the craniofacial junction to avoid unnecessary surgery and possible catastrophes.
: Learning Objectives: After studying this article, the participant should be able to: 1. Understand the cause and pathogenesis of facial clefting and encephaloceles. 2. Recognize and classify facial clefts and encephaloceles. 3. Understand the different treatment plans for reconstruction of facial clefts and encephaloceles. : The wide variety of craniofacial malformations makes classification difficult. A simple classification system allows an overview of the current understanding of the causes, assessments, and treatments of the most frequently encountered craniofacial anomalies. Facial clefts and encephaloceles are reviewed with respect to their diverse causes, pathogenesis, anatomical features, and treatments. Approaches to the surgical treatment of these conditions are reviewed. (Plast. Reconstr. Surg. 112: 606, 2003.) (C)2003American Society of Plastic Surgeons
Anterior encephalocele is a rarely reported CNS malformation with a geographical predilection for South-East Asia. The paucity of literature prompted us to analyse our results with hemiorbital advancement and classical Tessier's operation in 103 cases over 32 years (1971-2002). In our series, the frontoethmoidal subtype was the most common (80%), followed by the orbital (8%), transethmoidal (8%), transsellar (3%) and interfrontal types (1%). All patients with classical frontoethmoidal encephalocele had swelling over the nose or inner canthus since birth, with varying degrees of hypertelorism; and those with the nasopharyngeal subtype commonly presented with nasal obstruction and cerebrospinal fluid (CSF) rhinorrhoea. Neurofibromatosis was present in seven patients, all of whom had an orbital encephalocele. The diagnostic workup included a computerised tomography scan of the head in 96 patients and magnetic resonance imaging of the brain in 16 patients. The associated findings on imaging were hydrocephalus (15%), corpus callosum agenesis (7%), arachnoid cyst (3%), porencephalic cyst (3%) and single ventricle (3%). A classical Tessier's operation was performed in the initial 30 cases. However, since 1988, we have been performing a single stage hemiorbital advancement and repair of the encephalocele. There were three deaths, one due to pneumonia and two due to fulminant meningitis. Twenty-two patients (22%) had postoperative CSF leak, of which five required lumboperitoneal shunt placement. We believe that hemiorbital advancement offers satisfactory results with less morbidity than the Tessier's operation.
This is the first of the two parts of a paper concerning a novel method on the surgical treatment of orbital dysmorphisms, especially telecanthus (TC) and hypertelorism (HT). The normal orbital values of a given ethnic group is an essential factor in determining the degree of correction intended in that patient population. We did not find any data related to the normal orbital values in Khmer-Cambodians in whom we performed the corrective surgeries. Thus, the aims of this article are to evaluate the orbital morphometric data procured in Khmer-Cambodians and to analyze the contradictory definitions of TC and HT found in the literature. We measured the inner canthal distance, outer canthal distance, and interpupillary distance in 688 Khmer-Cambodians. The measured normal values are presented and compared with other Asian populations. We discovered that the Khmer-Cambodian orbital morphometry did not resemble the general conception of an Asian appearance, but rather showed a similarity to values found in Indians. Telecanthus and hypertelorism are frequent orbital dysmorphisms that, however, find conflicting definitions in the literature. By means of a short literature review, we have attempted to reorganize the multiplicity of definitions in orbital measurements, as well as clarify the confusing terminology used in TC and HT.
Frontoethmoidal meningoencephaloceles (MECs) are a relatively common abnormality in southeast Asia, with disastrous consequences for the sufferer. In Cambodia, a lack of skilled neuro-and craniofacial surgeons, and the cost of surgery limit the possibilities for appropriate treatment of patients with these lesions. The authors developed a low-cost humanitarian program with the goals of treating frontoethmoidal MECs, ensuring careful postoperative follow-up, and teaching Khmer surgeons how to treat these malformations.
This program was facilitated by two nongovernmental organizations: Rose Charities Cambodia provided the facilities, patients, and local staff, and "Médecins du Monde" provided visiting surgeons and anesthesiologists. All operations were free of charge for all patients. A strict follow-up program was organized to evaluate the surgical results, the social impact of the surgery, and the satisfaction levels of the children and their parents.
Forty-five children and seven young adults with MECs were treated using a rather simple surgical technique. Of the three types of MECs encountered, the most frequent was the nasoethmoidal type (43 cases). The most common postoperative issue was a temporary CSF leak (in 16 patients). Cosmetic results were considered excellent or good in 40 patients, average in nine, and poor in one; two patients were lost to follow-up. The overall cost of each operation was estimated to be $380 (US dollars), far less than a standard MEC operation would cost in a more developed country. At the end of this humanitarian program, Khmer surgeons were able to treat standard cases of frontoethmoidal MECs without the help of foreigners.
Patients in developing nations who have limited access to standard neurosurgical care can be treated for frontoethmoidal MECs with few complications and a satisfactory cost-to-benefit ratio.
One hundred and thirty nine patients suffering from encephalomeningocoele and myelomeningocoele who had attended the neurosurgical outpatients department of the Rangoon People's Hospital were interviewed. A predominance of frontal encephalomeningocoele was found; a low proportion of these patients had been conceived in the cold season and an increased interval separated the index patients from the previous full term pregnancy of their mothers; no first degree relatives were affected. It is suggested that an environmental factor is implicated in the developmental aetiology.
: The effectiveness of chitosan as an intraoperative topical hemostatic agent for brain tissue was tested. Cerebral cortical lesions were made in 10 cats. Chitosan was used to obtain hemostasis. The elapsed time from the application of the hemostatic agent to hemostasis was determined. The animals were killed and the lesions were examined grossly and microscopically to evaluate the biocompatibility of chitosan. Chitosan was easy to apply and required minimal subsequent manipulation. Histopathological examination revealed that chitosan is biocompatible with cerebral cortex. (Neurosurgery 15:9-13. 1984) Copyright (C) by the Congress of Neurological Surgeons
Results are described of 86 intracranial operations for the repair of nasal encephalocoele children at the Lahore Neurosurgical Centre in the last 13 years. These midline lesions at the base of the anterior cranial fossa have been considered in the past to be inoperable and even when operable, unpleasantly difficult. The intracranial approach has made it possible to treat this condition satisfactorily with comparatively few complications. Attempts at extra-cranial repair have met little success as they do not take into account the basic embryology and anatomy of the condition and fail to tackle the sac at the patent foramen caecum, the neck of the sac. This paper describes the embryology, anatomy, basic pathology and clinical features of the condition with characteristic X-rays showing the patent foramen caecum and outlines the operative repair.
Three nasofrontal encephalocoeles managed at the University of South Alabama Medical Center are presented. Each consisted of abnormal brain originating from one frontal lobe, although the amount of cerebral tissue and the accompanying skin and meningeal layers varied. The encephalocoeles protruded through a defect in the anterior skull base near the cribiform plate. Repair in the neonatal period was required in two of the infants because of the size of the lesion and obstruction of the nasal airway. The operative approach utilized a bifrontal craniotomy with resection of the encephalocoele intradurally, repair of the anterior cranial fossa dura and osteoplastic repair of the foramen cecum defect. The closure of the facial defect depended upon the nature of the skin covering the herniation; either absence or excess of skin occurred. The preoperative evaluation disclosed associated congenital deformities in 2 of 3 patients in this series. Computerized tomographic scanning was of importance in preoperative planning. The operative technique can be modified to allow for each child's unique anatomy. Repair of nasofrontal encephalocoeles in the neonatal period may simplify the required operative procedures.
✓ Cranial defects and cerebral abnormalities as revealed by postmortem dissection in 12 patients with sincipital encephalomeningocele are reported. The various methods of classifying this lesion are discussed. A classification based on the location of the defect in the cranium is outlined. The clinical application of such a classification and its usefulness in the surgical management are emphasized.
HE term "encephalocele" generally denotes a cephalic hernia through a congenital defect in the skull (cranium bifidum). The protrusions contain meninges and cerebrospinal fluid (cranial meningocele) and may also contain some portion of the brain (meningoencephalocele or encephalomeningocele). They may be partially or completely epithelialized. The great majority of encephaloceles are located in the occipital area. Anterior encephaloceles are uncommon and have been classified into sincipital varieties which visibly involve the nasofrontal region or the extremely rare basal ones which are hidden in the nasopharynx or orbit? The incidence of this malformation has been estimated at one in every 3000 to 10,000 live births. 11,~2 The clinical manifestations and surgical management of encephaloceles have been described in reports dealing for the most part with the larger related problems of spina bifida and myelomeningocele. ~,7,1~ There are no comprehensive surveys on the morbidity and the quality of survival in individuals born with different types of encephaloceles. I~rber, 11 however, has recently reviewed the prognosis of occipital encephaloceles in a series of infants. There is no comparison of the long-term outlook with this condition before and after ventriculoatrial shunt operations were widely adopted for controlling the hydrocephalus associated with many encephaloceles. This report analyzes the morbidity of encephaloceles and the results of treatment over a 20-year span through 1967 at the Indiana University Medical Center. The study interval includes the decade immediately before and that just after the first ventriculoatrial shunting operations were done at this in
An exceptionally high incidence of frontoethmoidal encephalomeningocele is found in Southeast Asia. Diagnosis, with details of the bones and intracranial abnormalities, must be studied for treatment. Bone defects in 120 patients were assessed on plain radiograms. CT in 110 patients was found to be useful for confirming the diagnosis, classifying the lesions and demonstrating more details of facial abnormalities. The content of the sac and intracranial abnormalities can be assessed by CT, but MRI is more useful in demonstrating the details in multiple planes. MRI and MRA are non-invasive studies of the blood vessels at the base of the brain. These also help in the planning and selection of an operative approach.
Summary A case report of fronto-ethmoidal meningoencephalocele is described.
A series of 74 cephaloceles (17 cranial meningoceles and 57 meningoencephaloceles) is reported. Infants born with large meningoencephaloceles containing recognizable cerebral tissue usually did badly despite endeavors to conserve brain function by expanding the cranial capacity (5 cases) or decompressing hydrocephalic ventricles (9 cases). Infants with cranial meningoceles almost all did well, even when there was associated hydrocephalus. The etiological diversity of cephaloceles is emphasized. Frontoethmoidal meningoencephaloceles, which occur with noteworthy frequency in South and Southeast Asia, require separate consideration in both genetic counseling and treatment; the associated facial deformities (hypertelorism and orbital dystopia) can be corrected with a one-stage craniofacial reconstruction. Antenatal diagnosis by ultrasound is now often possible and was achieved in 4 cases; we suggest that neurosurgeons should participate in such antenatal evaluations.
Twenty-five cases of fronto-ethmoidal meningoencephaloceles have been studied. The relationship to other sincipital meningoencephaloceles is explored. In all cases the exit holes from the anterior cranial fossa are at the site of the foramen caecum. The facial component of the defect determines the sub-classification: naso-frontal, naso-ethmoidal and naso-orbital. The cranio-facial deformity may consist of hypertelorism, orbital dystopia, elongation of the face and dental malocclusion. These reflect the distorting influence of the extruded intracranial contents on facial growth. Early removal of the meningoencephalocele by the cranio-facial route is recommended to allow normal growth forces to be re-established. In older patients with established deformities translocation of the orbits may be necessary.
This presentation deals with our experience of surgery on 8 patients with fronto-ethmoidal meningocoele. 7 of these patients had an intracranial repair of the meningocoele, coupled with or followed by an orbital osteotomy, to correct the associated hypertelorism. We feel that an orbital osteotomy done at the time of meningocoele repair improves the results of surgery, without adding to its risk. The osteotomy practised by the authors includes resection of a central wedge of bone and a medial shift of the roof and medial wall of the orbit.
Eighteen cases of anterior encephalocele treated at the University of Malaya Hospital between 1970 and 1980 are discussed, and the literature concerning this defect is reviewed. A detailed analysis of the microscopic abnormalities present in the surgical specimens is included, along with the relevant radiographic and demographic data. Anterior encephalocele is more common in Southeast Asia than elsewhere. The possible ethnographic and geographic implications are presented, as well as a discussion of the relevant embryology, in attempting to define possible etiologies for this malformation. The author's surgical approach to the repair of this defect and reasons for preferring a transcranial, intradural approach are described. Potential complications are enumerated.
Anterior encephalocele in Harare
- Kalangu K
- Levy LF
- Makarawo S
- Nkrumah FK
Birth defects in Papua New Guinea
- Dryden R
- Vince J.
Fronto-ethmoidal encephalocele
- Suwanela C
- Hogsaprabhas C.
Treatment by transcranial operation
- Naim-ur-Rahman
Frontal encephalocele
- Klerk DJJ
Anterior encephalocele in Harare
- Kalangu K