A Pituitary Abscess Caused by Morganella morganii: A Case Report.

BACKGROUND Pituitary abscess (PA) is a rare pituitary lesion accounting for less than 1% of all pituitary diseases and is associated with high mortality rates. The non-specific clinical symptoms and radiological features preclude accurate diagnosis of the disease. Hence, surgical intervention is still considered the criterion standard method for PA diagnosis. Most PAs occur as a primary disease or due to complications such as surgery, sepsis, and adjacent inflamed lesions. CASE REPORT A 53-year-old man presented to the Emergency Department with a headache associated with nausea, vomiting, and constipation. The patient had no visual disturbances, polyuria, polydipsia, nocturia, or abnormal discharges. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a large suprasellar mass. The patient underwent left-side trans-nasal transsphenoidal microscopic surgery for mass removal. An abscess with discharge was noticed and sent for microbiological evaluation, revealing an infection with a commensal enteric bacterium that rarely causes nosocomial infection, known as Morganella morganii. The patient was discharged after completing the intravenous antibiotic course and was given oral antibiotics. CONCLUSIONS Pituitary abscess is a rare pituitary lesion. However, taking the history of recent meningitis, sinusitis, and/or surgery may help to reach an early diagnosis, prompt surgical intervention, and vital therapeutic steps to reduce mortality rates and improve patient's outcomes. PA should always be considered when patients demonstrate signs of pituitary dysfunction associated with signs of infection. Furthermore, antibiotics should be administered immediately once the PA case is confirmed and should last for at least 6 weeks postoperatively.

Epidemiology, prenatal management, and prevention of neural tube defects.

This review article discusses the epidemiology, risk factors, prenatal screening, diagnosis, prevention potentials, and epidemiologic impact of neural tube defects (NTDs). The average incidence of NTDs is 1/1000 births, with a marked geographic variation. In the developed countries, the incidence of NTDs has fallen over recent decades. However, it still remains high in the less-developed countries in Latin America, Africa, the Middle East, Asia, and the Far East (>1 to 11/1000 births). Recognized NTDs risks include maternal diabetes, obesity, lower socioeconomic status, hyperthermia, and exposure to certain teratogens during the periconceptional period. Periconceptional folic acid supplementation decreased the prevalence of NTDs by 50-70%, and an obligatory folic acid fortification of food was adopted in several countries to reach women with unplanned pregnancies and those facing social deprivation. Prevention of NTDs can be accelerated if more, especially low income countries, adopted fortification of the staple food in their communities.

Split cord malformation associated with spinal open neural tube defect.

OBJECTIVE: To illustrate the clinical and radiological findings of split cord malformation (SCM) in patients with spinal open neural tube defect (SONTD), and report the outcome of their treatment. METHODS: A retrospective study of the clinical and radiological findings of 11 patients diagnosed with SCM, identified among 83 patients with SONTD at King Khalid University Hospital, in Riyadh, Saudi Arabia between 1995 and 2010. RESULTS: There were 6 girls and 5 boys; their age ranged from less than a year to 9 years (mean 4.2 years). Six patients had type I SCM, and 5 patients type II SCM. The CT and MRI imaging showed characteristic bony, cartilaginous, or fibrous septum, and other SONTD-associated anomalies. Seven patients were graded A & B according to the Frankel grading score, and none of them required surgery, while worsening neurology led to surgical intervention in 3 patients, with clinical improvement after surgery, and one patient that underwent cord untethering remained stable. CONCLUSION: Split cord malformation is not uncommon among patients with SONTD. It tends to involve mainly the lumbar spine, and female predominance is more remarkable in type I. Neurological manifestations of SCM may be superimposed with SONTD. Surgery is effective for symptomatic patients, and not indicated in the severely disabled.

Classification, clinical features, and genetics of neural tube defects.

Neural tube defects (NTDs) constitute a major health burden (0.5-2/1000 pregnancies worldwide), and remain a preventable cause of still birth, neonatal, and infant death, or significant lifelong handicaps. The malformations result from failure of the neural folds to fuse in the midline, and form the neural tube between the third and the fourth week of embryonic development. This review article discusses their classification, clinical features, and genetics. Most NTDs are sporadic and both genetic, and non-genetic environmental factors are involved in its etiology. Consanguinity was suggested to contribute to the high incidence of NTDs in several countries, including Saudi Arabia. Syndromes, often associated with chromosomal anomalies, account for <10% of all NTDs; but a higher proportion (20%) has been documented in Saudi Arabia. Genetic predisposition constitutes the major underlying risk factor, with a strong implication of genes that regulate folate one-carbon metabolism and planar cell polarity.

Agenesis of the corpus callosum associated with spinal open neural tube defect.

OBJECTIVE: To ascertain the incidence and clinical implications of agenesis of the corpus callosum (ACC) in spinal open neural tube defects (SONTD). METHODS: All cases of SONTD registered at the Spina Bifida Clinic in King Khalid University Hospital, Riyadh, Saudi Arabia between 1995 and 2010 were retrospectively reviewed, and mid-sagittal MRI of the corpus callosum (CC) area was analyzed in each case. Neurodevelopmental outcome was classified as poor in children with seizures, severe neurodevelopmental impairment, or death. RESULTS: Thirty-eight patients (45.8%) with ACC were identified among 83 cases with SONTD. Patients' age ranged between one and 16 years. Total ACC was found in 10 patients, partial ACC in 25, and in 3 patients, the CC was hypoplastic. Active hydrocephalus was an associated finding in 9 out of 10 patients with total ACC, 22 out of 25 with partial ACC, and in all patients with hypoplasia of the CC. Thirteen patients (34.2%) had normal intellectual function, whereas 24 patients presented with learning disability, epilepsy, or poor intellectual function; and one patient died of respiratory failure. CONCLUSION: Agenesis of the corpus callosum is found in a significant portion of patients with SONTD. When associated with hydrocephalus, its presence affects neuro-developmental outcome.

Risk factors for neural tube defects in Riyadh City, Saudi Arabia: Case-control study.

Both genetic and non-genetic environmental factors are involved in the etiology of neural tube defects (NTD) which affect 0.5-2/1000 pregnancies worldwide. This study aimed to explore the risk factors for the development of NTD in Saudi population, and highlight identifiable and preventable causes. Similar studies are scarce in similar populations ofthe Arabian Peninsula and North Africa. This is an unmatched concurrent case-control study including NTD cases born at King Khalid University Hospital, Riyadh during a 4-year period (2002-2006). The case-control study included 25 cases and 125 controls (case: control ratio of 1:5). Years of formal education, employment, household environment (including availability of air conditioning) and rate of parental consanguinity did not differ between mothers of cases and controls. Significantly higher proportion of mothers of cases had history of stillbirth compared to control mothers (16% vs 4.1%, P=0.02). Also family history of hydrocephalus and congenital anomalies were more prevalent in cases than controls (P values=0.0000 and 0.003, respectively). There was significant protective effect of periconceptional folic acid consumption both prior to conception (OR 0.02, 95% CI 0.00-0.07) and during the first 6 weeks of conception (OR 0.13, 95% CI 0.04-0.39). Further research, including a larger cohort, is required to enable ascertainment of gene-nutrient and gene environment interactions associated with NTD in Saudi Arabia.

Endoscopic third ventriculostomy for hydrocephalus in children younger than 1 year of age.

OBJECTIVE: The purpose of this study is to assess the role of endoscopic third ventriculostomy (ETV) in the treatment of hydrocephalus in children under 1 year of age. The authors analyzed data of ETV in their institution. METHODS: Between January 1995 and December 2008, 52 ETV procedures were performed for the treatment of hydrocephalus in 49 infants (32 male and 17 female). Their age ranged from 6 days to just under 12 months (mean age 6.2 months). The cause of hydrocephalus was occlusive in 43 patients (aqueduct stenosis in 31, Chiari II malformation in eight, Dandy-Walker cyst in two, quadrigeminal lipoma in one, and cerebellopontine angle arachnoid cyst in one patient). Communicating hydrocephalus was caused by intraventricular hemorrhage, meningitis, and/or ventriculitis in six patients. RESULTS: The overall success rate was 69.4% with mean follow-up period of 68.2 months. Patients with aqueduct stenosis had a higher success rate of ETV which was 77.4%. Seven infants were born preterm, six of them required a permanent ventriculoperitoneal shunts (VPS; P = 0.003). Malfunctioned VPS was removed in two patients following ETV. There was one death from intracranial hemorrhage, two cerebrospinal fluid leaks, and one meningitis. CONCLUSION: Endoscopic third ventriculostomy can be considered a possible treatment procedure alternative to VPS for the treatment of occlusive hydrocephalus in infants. ETV was effective in full-term infants while the results in low birth weight, preterm infants were poor. Success of ETV is not only age dependent but also etiology dependent. Infants with occlusive hydrocephalus treated with VPS, who present with shunt failure, could be treated by ETV and removal of the shunt device.

Hematologic risk factors for stroke in Saudi children.

OBJECTIVE: To explore the hematologic risk factors for stroke in a cohort of Saudi children. METHODS: We evaluated children at the Division of Pediatric Neurology at King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, during the periods July 1992 to February 2001 (retrospective study) and February 2001 to March 2003 (prospective study). Investigations for suspected cases included neuroimaging, transcranial Doppler (TCD) for cases of sickle cell disease (SCD), and Duplex scan. Hemostatic assays included coagulation screening tests, tests of thrombin generation and fibrinolysis, coagulation inhibitors, and activated protein C resistance. RESULTS: During the study period, 104 Saudi children (aged one month to 12 years) with stroke were seen. The mean age of the cohort was 27.1 months (SD = 39.3 months) and median was 6 months. Ischemic strokes accounted for the majority of cases (76%). A major risk factor was identified in 93 of 104 cases of stroke (89.4%). Hematologic disorders were the most common (46.2%), followed by prothrombic disorders (31.7%); microcytic hypochromic anemia (26%); sickle cell disease (SCD), or SCbeta(0)-thalassemia, (11.5%), and factor IX deficiency (2.9%). Raised anticardiolipin antibodies (13/49, 26.5%) was the most frequent abnormality. Deficiencies of the natural anticoagulants (protein S, protein C and antithrombin III) were as follows: protein S (15/70, 21.4%); protein C (15/70, 21.4%) and combined deficiency of 2 or more inhibitors (9/70, 12.9%). Activated protein C resistance has not been detected. Contrary to the findings of previous studies from Saudi Arabia, SCD is a common risk factor and is severe, as it resulted in multiple strokes. Moyamoya syndrome was diagnosed in 2 patients with SCD, one of whom had revascularization surgery (encephaloduroarteriosynangiosis). Assessment of children with SCD at risk of stroke was helped by the introduction of TCD followed by neuroimaging, using MRI and magnetic resonance angiography. CONCLUSIONS: The study strongly highlights the importance of prothrombotic disorders and the severe phenotype of SCD as risk factors for stroke in Saudi children.

Congenital and genetic cerebrovascular anomalies as risk factors for stroke in Saudi children.

OBJECTIVE: To explore the role of and report on congenital and genetic cerebrovascular anomalies as risk factors for stroke in a prospective and retrospective cohort of Saudi children. METHODS: Children with stroke were evaluated at the Division of Pediatric Neurology (DPN), or were seen as inpatients in the Pediatric Wards at King Khalid University Hospital (KKUH), Riyadh, Kingdom of Saudi Arabia during the periods July 1992 to February 2001 (retrospective study) and February 2001 to March 2003 (prospective study). Stroke work-up for each suspected case included hemostatic assays, serological, biochemical and neurophysiological tests. Neuroimaging modalities included routine skull x-rays, CT, MRI, magnetic resonance angiography (MRA) and conventional cerebral angiography. RESULTS: Of 104 children with stroke, congenital and genetic cerebrovascular anomalies were the underlying risk factor in 7 (6.7%). The patients were evaluated at the DPN at a mean age of 66 months (range = 8 months to 11 years, median = 6 years); and they had stroke at a mean age of 48 months (range = 2 months to 10 years, median = 8 months). Four patients had stroke in association with neurocutaneous syndromes. Two had Sturge-Weber syndrome (SWS), one had Klippel-Trenaunay syndrome associated with SWS, and the fourth had neurofibromatosis type 1. Two patients had intracranial hemorrhage secondary to ruptured aneurysm. A girl (aged 9 years and 4 months) had left posterior cerebral artery aneurysm. She was diagnosed to have autosomal dominant polycystic kidney disease following renal ultrasonography. She died 5 months later despite surgical intervention (clipping of aneurysm). The second child was an 8-month-old boy who presented with subarachnoid and intraventricular hemorrhage (IVH) following ruptured anterior communicating artery aneurysm. He recovered with no residual symptoms following successful clipping of the aneurysm. Arteriovenous malformation (AVM) caused IVH in a 7-year-old boy who reported to hospital 5 hours after onset of headache, vomiting, drowsiness, and dizziness. Following drainage of the IVH and stabilization of the patient, the AVM was successfully embolized 6 weeks later. CONCLUSIONS: As a group, congenital and genetic cerebrovascular anomalies constitute a significant risk factor for stroke in Saudi children. Recognition of these diseases is important since some are treatable and because other family members may be at risk.

Moyamoya syndrome as a risk factor for stroke in Saudi children. Novel and usual associations.

OBJECTIVE: To report on moyamoya syndrome (MMS) as a risk factor for stroke in a prospective and retrospective cohort of Saudi children. The usual and novel associations of MMS in this cohort will also be described. METHODS: Children with stroke were evaluated at the Division of Pediatric Neurology at King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia during the periods July 1992 to February 2001 (retrospective study) and February 2001 to March 2003 (prospective study). Investigations for suspected cases included hemostatic assays, biochemical, and serological tests. Neuroimaging included CT, MRI, magnetic resonance angiography (MRA), single photon computerized tomography (SPECT) brain scan and conventional cerebral angiography. RESULTS: Moyamoya syndrome was the underlying risk factor for stroke in 6 (5.8%) of the 104 children (aged one month to 12 years). They were 4 females and 2 males. Their first cerebral ischemic event occurred at a mean age of 45 months (median = 44 months, range 17-66 months). In all 6 cases, MMS was associated with an underlying hematologic abnormality or other diseases. Protein C deficiency was identified in one girl and protein S deficiency in another. Two patients had respectively, sickle cell disease (SCD) and sickle cell-beta-thalassemia (S beta-thalassemia), which had been associated in the latter with membranous ventricular septal defect. Adams-Oliver syndrome (AOS, OMIM 100300) was associated with MMS in an 18-month-old girl. A 4-year-old boy had wrinkly skin syndrome (WSS, OMIM 278250) phenotype. The association of MMS and protein C deficiency was first reported in this cohort of patients, whereas the association of the syndrome with WWS and AOS has not, hitherto, been described. The 3 patients who had MMS associated with protein C deficiency, SCD, and AOS underwent successful revascularization surgery in the form of encephaloduroarteriosynangiosis. CONCLUSIONS: Moyamoya syndrome constitutes an important risk factor of stroke in Saudi children. Comprehensive clinical evaluation and investigations, including screening for thrombophilia and neuroimaging studies, are required for the primary diagnosis of the disease and for unraveling other diseases associated with MMS. This will help in managing these patients and in guiding genetic counseling for their families.

Aneurysmal subarachnoid hemorrhage in the first year of life: case report and review of the literature.

INTRODUCTION: Primary subarachnoid hemorrhage is rare in infancy. A bleeding arterial aneurysm as its cause is even less frequent. A review of the literature turned up 85 cases of cerebral aneurysm that occurred in the 1st year of life, 63 of them presenting with subarachnoid hemorrhage (SAH). CASE REPORT: The authors report a case of an 8-month-old boy who presented with seizures due to ruptured anterior communicating (ACom) artery aneurysm with subsequent subarachnoid and intraventricular hemorrhage. The infant was operated successfully, without complications. DISCUSSION: In this report the authors highlight certain clinical and diagnostic features, surgical considerations, and outcomes of aneurysmal subarachnoid hemorrhage in the 1st year of life.