Tracheobronchial calcification associated with Keutel syndrome

Tracheobronchial cartilage calcification is an unusual radiologic finding in infants and children under 15 years old. Keutel syndrome is a rare, autosomal recessive disorder characterized by diffuse cartilage calcification, brachytelephalangia, pulmonary stenosis and midfacial hypoplasia. We report two children in whom abnormal tracheobronchial calcification was associated with Keutel syndrome. Keutel syndrome should be considered in the differential diagnosis of children with tracheobronchial calcification.     

Sibling composition and child immunization in India and Pakistan, 1990–2007

Background

This study aimed to assess trends in gender differentials in child immunization beyond the conventional male-female dichotomy, by considering gender, surviving siblings, birth order and different compositions of older siblings in tandem, during 1990–2007 in India and Pakistan.

Methods

Using different rounds of Demographic and Health Survey datasets, we adopted the World Health Organization guidelines for appraising full immunization among children. Sex composition of surviving older siblings was combined. Cochrane-Armitage and the Chisquare tests were used to test linear and nonlinear trends, respectively.

Results

Although child immunization has increased during the period of 1990–2007 in both India and Pakistan, results showed that more than 50% of the eligible children did not receive the recommended immunization. The results also showed that boys and girls with no older surviving siblings and those with only surviving siblings of the opposite sex appeared to have fully immunized proportionally compared with the children with other sibling compositions.

Conclusion

The findings confirmed that girls and boys were not always treated equally, and that there was a clear pattern of selective neglect in child immunization in both countries during the period of 1990–2007.     

MR imaging findings suggestive of posterior reversible encephalopathy syndrome in adolescents with systemic lupus erythematosus

Background

Endothelial damage, hypertension and cytotoxic medications may serve as risk factors for the posterior reversible encephalopathy syndrome (PRES) in systemic lupus erythematosus. There have been few case reports of these findings in pediatric lupus patients.

Objective

We describe clinical and neuroimaging findings in children and adolescents with lupus and a PRES diagnosis.

Materials and methods

We identified all clinically acquired brain MRIs of lupus patients at a tertiary care pediatric hospital (2002–2008). We reviewed clinical features, conventional MRI and diffusion-weighted imaging (DWI) findings of patients with gray- and white-matter changes suggestive of vasogenic edema and PRES.

Results

Six pediatric lupus patients presenting with seizures and altered mental status had MRI findings suggestive of PRES. In five children clinical and imaging changes were seen in conjunction with hypertension and active renal disease. MRI abnormalities were diffuse and involved frontal regions in five children. DWI changes reflected increased apparent diffusivity coefficient (unrestricted diffusion in all patients). Clinical and imaging changes significantly improved with antihypertensive and fluid management.

Conclusion

MRI changes suggestive of vasogenic edema and PRES may be seen in children with active lupus and hypertension. The differential diagnosis of seizures and altered mental status should include PRES in children, as it does in adults.     

Cervical spine anomalies in Menkes disease: a radiologic finding potentially confused with child abuse

Background

Menkes disease is an X-linked recessive disorder of copper transport caused by mutations in ATP7A, a copper-transporting ATPase. Certain radiologic findings reported in this condition overlap with those caused by child abuse. However, cervical spine defects simulating cervical spine fracture, a known result of nonaccidental pediatric trauma, have not been reported previously in this illness.

Objective

To assess the frequency of cervical spine anomalies in Menkes disease after discovery of an apparent C2 posterior arch defect in a child participating in a clinical trial.

Materials and methods

We examined cervical spine radiographs obtained in 35 children with Menkes disease enrolled in a clinical trial at the National Institutes of Health Clinical Center.

Results

Four of the 35 children with Menkes disease had apparent C2 posterior arch defects consistent with spondylolysis or incomplete/delayed ossification.

Conclusion

Defects in C2 were found in 11% of infants and young children with Menkes disease. Discovery of cervical spine defects expands the spectrum of radiologic findings associated with this condition. As with other skeletal abnormalities, this feature simulates nonaccidental trauma. In the context of Menkes disease, suspicions of child abuse should be considered cautiously and tempered by these findings to avoid unwarranted accusations.     

Pediatric Moyamoya Disease: Clinical Profile, Literature Review and Sixteen Year Experience from a Tertiary Care Teaching Institute

Objective

To assess the clinical spectrum and outcome of pediatric moyamoya disease (MMD) in Indian sub-continent.

Methods

The authors retrospectively analysed data of 23 patients, diagnosed with pediatric moyamoya disease from a single center during the period of 1996–2011.

Results

There were 18 boys and 5 girls. Mean age at onset of symptoms was 3.8?±?2.2 y. All patients presented with cerebral ischemic events. Recurrent stroke was the presenting feature in 12(52.2 %) patients. Twenty one patients were definitive case of moyamoya disease and two were probable case of moyamoya disease. Posterior circulation was involved in 26.1 % patients. Three patients underwent indirect surgical revascularisation procedure and rest were managed conservatively. On follow up 66.6 % patients had residual neurological deficit.

Conclusions

Heightened awareness of this entity among pediatric neurologists is required for early diagnosis of pediatric moyamoya disease.     

Localized hypertrophic neuropathy of the sciatic nerve in children: MRI findings

Background

Localized hypertrophic neuropathy (LHN) of the sciatic nerve in children is a rare condition characterized by a painless neurological deficit in the sciatic nerve territory.

Objective

To demonstrate the role of MRI using a specific protocol and describe the primary findings in LHN.

Materials and methods

Imaging in four children (age 2?years to 12?years) is presented. All children presented with lower limb asymmetry. Three had a steppage gait. LHN was confirmed by electrophysiological studies and by MRI of the whole sciatic nerve with a dedicated protocol covering the lumbar spine and the lower limb.

Results

There were four direct MRI findings: (1) linear and focal hypertrophy with progressive enlargement of a peripheral nerve or plexus diameter, (2) abnormal hyperintensity of the nerve on T2-weighted images, (3) preserved fascicular configuration, and (4) variable enhancement after intravenous gadolinium administration. In addition there were atrophy and fatty infiltration of innervated muscles. MRI was helpful for determining the extent of lesions and in excluding peripheral nerve compression or tumour.

Conclusion

MRI of the whole sciatic nerve is the method of choice for diagnosing LHN of the sciatic nerve.     

Tracheobronchial calcifications in children

Background

Tracheobronchial calcifications are considered a rare radiologic finding in children. Our clinical experience indicates that this finding is not infrequently seen among children with prosthetic heart valves who have been treated with warfarin sodium.

Objective

We hypothesized that calcifications of the tracheobronchial tree are more common than previously reported in this patient population.

Materials and methods

We reviewed the medical records and imaging studies of children who underwent cardiac valve replacement at our institution to estimate the prevalence.

Results

Tracheobronchial calcifications were identified on chest radiographs in 6 out of 17 children (35%), indicating that this imaging finding might be frequently overlooked.

Conclusion

All children positive for tracheobronchial calcifications had been anticoagulated with warfarin sodium between the time of surgery and development of positive imaging findings. Our findings suggest that tracheobronchial calcifications are not uncommon in children treated with warfarin. Further investigation is necessary to determine wether there is a cause–effect relationship in these children.     

Efficacy of levamisole in children with frequently relapsing and steroid-dependent nephrotic syndrome

Objectives

To assess the efficacy of levamisole in frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome.

Study Design

Retrospective analysis of hospital case records.

Setting

Pediatric nephrology department of a tertiary referral pediatric hospital.

Participants

62 children with frequently relapsing nephrotic syndrome and 35 children with steroid-dependent nephrotic syndrome.

Methods

Case records of children who were diagnosed as steroid-dependant or frequently-relapsing nephrotic syndrome from June 2004 to June 2011, were reviewed. Levamisole was given daily (2 mg/kg/d) along with tapering doses of alternate day steroids after remission on daily steroids.

Results

Levamisole was effective in 77.3% children with a better (80.6%) efficacy in frequently relapsing nephrotic syndrome. A total of 34 children completed 1 year follow-up post levamisole therapy. The cumulative mean (SD) steroid dose 1-year before therapy was 4109(1154) mg/m² and 1-year post therapy was 661 (11) mg/m² (P<0.001). The relapses were also less during the period of post-levamisole therapy.

Conclusion

Levamisole is an effective alternative therapy in frequently relapsing and steroid-dependent nephrotic syndrome.     

Bare spot of the glenoid fossa in children: incidence and MRI features

Background

The bare spot of the glenoid fossa is a normal cartilage defect seen frequently in adults. It has been used on arthroscopy as a landmark for the center of the glenoid fossa. There are no reports of this variant in children, but we have noted it on some pediatric clinical shoulder MRI studies.

Objective

Our main purpose is to evaluate the incidence of the bare spot in children and define location and MRI features.

Materials and methods

Shoulder MRI studies (total 570) from 2004 to 2008 were reviewed. Children were divided into two age groups: group 1, 0–10 years (n?=?200), group 2, 11–20 years (n?=?370).

Results

A total of 12 bare spots (2.1%) were identified; all were seen in group 2. Eight (67%) were central and four were eccentric in the glenoid fossa. All showed a well-marginated focal cartilage defect containing hyperintense joint fluid or contrast agent. Three also had air.

Conclusion

The bare spot is seen in children. The absence in children younger than 10 years and the low incidence in the second decade support the proposed acquired nature. Familiarity with this finding is important so as not to misinterpret it as a pathologic condition.     

MRI evaluation of the knee in children with infantile Blount disease: tibial and extra-tibial findings

Background

Infantile Blount disease is a developmental condition characterized by disorganized endochondral ossification in the medial aspect of the proximal tibial physis.

Objective

To describe the MR imaging abnormalities in the proximal tibia, distal femur, menisci and ligaments of children with infantile Blount disease.

Materials and methods

We retrospectively evaluated 11 children (18 total knee MR examinations) with infantile Blount disease and compared them with an age-matched control group with normal MR examinations. Morphological and morphometric measurements were performed.

Results

The medial menisci were enlarged with increased T2 signal intensity in all MR examinations. The medial femoral epiphyseal cartilage showed abnormal foci of increased signal intensity in nine (50%). The mid-coronal thickness of the medial tibial epiphyseal cartilage was decreased with concomitant increase in the mid-coronal joint space distance. Angular measurements of the proximal tibia demonstrated posteromedial down-sloping configuration.

Conclusions

Most severe abnormalities of infantile Blount disease occur in the medial compartment of the knee, especially at the medial tibial physis and epiphysis. However, other important structures of the knee and the lateral compartment are often affected. MR imaging helps to delineate the extent of multiple tibial and extra-tibial abnormalities, including meniscal abnormalities, perichondrial membrane changes and premature physeal closure.     

Congenital arterioportal fistulas: radiological treatment and color Doppler US follow-up

Background

Congenital intrahepatic arterioportal fistulas (APFs) are a rare cause of portal hypertension in children. Doppler US is a useful diagnostic imaging modality. Transarterial embolization is a minimally invasive and effective therapy allowing occlusion of the fistula and restoration of liver hemodynamics.

Objective

To describe the clinical and radiologic findings, percutaneous treatment and role of D-US in the postembolization follow-up of children with APF.

Materials and methods

Between 2002 and 2011, four children with APF were treated. Initial diagnosis and follow-up was performed with D-US and confirmed by arteriography, followed by endovascular embolization in all patients.

Results

D-US demonstrated abnormal arterioportal communications in all patients. Six endovascular procedures were performed in these four children. In two children, no residual fistula was seen on D-US after the first procedure and symptoms resolved. In the other two children, D-US demonstrated residual flow through the fistula, with resolution of pathological D-US findings and symptoms after the second endovascular procedure. All four children were successfully treated and asymptomatic at the end of follow-up. The mean follow-up was 24?months.

Conclusion

Interventional radiology has a key role in the treatment of congenital APF. D-US is a noninvasive and effective tool for the diagnosis and follow-up of these patients.     

Agenesis of the internal carotid artery: associated malformations including a high rate of aortic and cardiac malformations

Background

Agenesis of the internal carotid artery (ICA) is a rare congenital anomaly occurring in less than 0.01% of the population, often incidentally discovered in pediatric populations. We recognized a high incidence of additional congenital malformations in children with ICA agenesis.

Objective

Our study reports nine cases of ICA agenesis and co-existent malformations and discusses implications of the association.

Materials and methods

We conducted a retrospective chart review of nine children evaluated at our institution with imaging findings of ICA agenesis.

Results

Seven children (78%) had congenital aortic or cardiac anomalies including coarctation (4), hypoplastic left heart (1), tetralogy of Fallot (1), and muscular ventricular septal defect (VSD) (1). Four children were diagnosed with an inherited disorder: Alagille syndrome (1), PHACE syndrome (1), VACTERL association (1), and methylenetetrahydrofolate reductase (MTHFR) gene variant (1). Additional congenital anomalies are also described.

Conclusion

In the setting of ICA agenesis, we report a robust association with congenital aortic and cardiac anomalies, as well as a broad spectrum of additional anatomical abnormalities that can occur in the setting of known genetic syndromes or as isolated findings. Knowledge of the natural history of ICA agenesis and associated anomalies will guide optimal care for these children.     

Association of initial CT findings with quality-of-life outcomes for traumatic brain injury in children

Background

Traumatic brain injury (TBI) is a leading cause of acquired disability in children and adolescents.

Objective

To demonstrate the association between specific findings on initial noncontrast head CT and long-term outcomes in children who have suffered TBI.

Materials and methods

This was an IRB-approved prospective study of children ages 2–17?years treated in emergency departments for TBI and who underwent a head CT as part of the initial work-up (n?=?347). The change in quality of life at 12?months after injury was measured by the PedsQL scale.

Results

Children with TBI who had intracranial injuries identified on the initial head CT had a significantly lower quality-of-life scores compared to children with TBI whose initial head CTs were normal. In multivariate analysis, children whose initial head CT scans demonstrated intraventricular hemorrhage, parenchymal injury, midline shift ≥5?mm, hemorrhagic shear injury, abnormal cisterns or subdural hematomas ≥3?mm had lower quality of life scores 1?year after injury than children whose initial CTs did not have these same injuries.

Conclusion

Associations exist between findings from the initial noncontrast head CT and quality of life score 12?months after injury in children with TBI.     

A retrospective review of pituitary MRI findings in children on growth hormone therapy

Background

Patients with congenital hypopituitarism might have the classic triad of pituitary stalk interruption syndrome, which consists of: (1) an interrupted or thin pituitary stalk, (2) an absent or ectopic posterior pituitary (EPP), and (3) anterior pituitary hypoplasia or aplasia.

Objective

To examine the relationship between pituitary anatomy and the degree of hormonal dysfunction.

Materials and methods

This study involved a retrospective review of MRI findings in all children diagnosed with congenital growth hormone deficiency from 1988 to 2010 at a tertiary-level pediatric hospital.

Results

Of the 52 MRIs reviewed in 52 children, 26 children had normal pituitary anatomy and 26 had one or more elements of the classic triad. Fourteen of fifteen children with multiple pituitary hormone deficiencies had structural anomalies on MRI. Twelve of 37 children with isolated growth hormone deficiency had an abnormal MRI.

Conclusion

Children with multiple pituitary hormone deficiencies were more likely to have the classic triad than children with isolated growth hormone deficiency. A normal MRI was the most common finding in children with isolated growth hormone deficiency.     

Clinical profile and outcome of chronic pancreatitis in children

Objective

To evaluate the etiology, presentation, complications and management of chronic pancreatitis in children.

Design

Retrospective chart review.

Setting

Gastroenterology department at Christian Medical College and Hospital, Vellore, India between January 2005 and December 2010.

Participants

99 Children (<18 yrs) diagnosed with chronic pancreatitis based on clinical and imaging features.

Main outcome measures

Etiology, clinical presentation, complications and management of chronic pancreatitis in children.

Results

Of 3887 children who attended the Gastroenterology department, 99(2.5%) had chronic pancreatitis, of which 60 (60.6%) were males. In 95(95.9%) patients no definite cause was detected and they were labeled as Idiopathic chronic pancreatitis. All patients had abdominal pain, while 9(9.1%) had diabetes mellitus. Of the 22 children tested for stool fat, 10(45.5%) had steatorrhea. Pancreatic calcification was seen in 69 (69.7%). 68 (71.6%) patients with idiopathic chronic pancreatitis had calcification. Calcific idiopathic chronic pancreatitis was more frequent in males (67.6% vs. 48.1%, P=0.07), and was more commonly associated with diabetes mellitus (13.2% vs. none, P=0.047) and steatorrhea (61.5% vs. 16.7%, P=0.069). Pseudocyst (17.1%) and ascites (9.1%) were the most common complications. All children were treated with pancreatic enzyme supplements for pain relief. 57 patients were followed up. With enzyme supplementation, pain relief was present in 32 (56.1%) patients. Of those who did not improve, 10 underwent endotherapy and 15 underwent surgery. Follow up of 8 patients who underwent endotherapy, showed that 5 (62.5%) had relief. Follow up of 11 patients who underwent surgery showed that only 3 (27 %) had pain relief. There was no death.

Conclusions

Idiopathic chronic pancreatitis is the predominant form of chronic pancreatitis in children and adolescents. It can present with or without calcification. The calcific variety is an aggressive disease characterized by early morphological and functional damage to the pancreas.     

Clinical and MRI features of neurological complications after influenza A (H1N1) infection in critically ill children

Background

Influenza A (H1N1) can cause severe neurological complications.

Objective

The purpose of this study was to analyze clinical and MRI features of neurological complications after H1N1 infection in critically ill children.

Materials and methods

We retrospectively analyzed clinical and neuroimaging findings in 17 children who were hospitalized in an intensive care unit with severe neurological complications after H1N1 infection in South China between September 2009 and December 2011. All children underwent pre- and post-contrast-enhanced brain MRI. Postmortem studies were performed in two children.

Results

Six children died, five because of acute necrotizing encephalopathy (ANE) and one because of intracranial fungal infection. Eleven recovered; their manifestations of H1N1 were meningitis (3), encephalitis (1) and influenza encephalopathy (7). MRI features of ANE included multiple symmetrical brain lesions demonstrating prolonged T1 and T2 signal in the thalami, internal capsule, lenticular nucleus and pontine tegmentum. Postmortem MRI in two children with acute necrotizing encephalopathy showed diffuse prolonged T1 and T2 signal in the bilateral thalami, brainstem deformation and tonsillar herniation.

Conclusion

Fatal neurological complications in children after H1N1 infection include ANE and opportunistic fungal infection. MRI is essential for identification of neurological complications and for clinical evaluation.     

Impfungen zum Schutz vor Atemwegsinfektionen bei Risikogruppen

Background

S. pneumoniae and influenza are the most common pathogens in the airways that are preventable by vaccination.

Objectives

The German Committee for Vaccinations (STIKO) also recommends vaccinations against pneumoccocci and influenza virus for subjects who are at risk. Using a literature search, we assessed which vaccines may be considered for which conditions.

Results

The STIKO gives an almost general recommendation for both pneumococcus and influenza vaccination. Children and adolescents with any condition considered a health risk may benefit from these immunisations. Namely, but not exclusively, patients with immunodeficiencies or airway diseases should be considered for vaccination. Pneumococcal conjugate vaccines appear more beneficial than the pneumococcal polysaccharide vaccine, also beyond the age of 5 years. A live attenuated influenza vaccine is more efficient than the trivalent inactivated influenza vaccine in children.

Conclusion

Vaccinations against pneumococci and influenza would greatly reduce morbidity caused by these pathogens in children and adolescents with chronic conditions.     

The trochlear pre-ossification center: a normal developmental stage and potential pitfall on MR images

Background

The hypertrophic changes that occur in the cartilage of an epiphysis prior to the onset of ossification are known as the pre-ossification center. Awareness of the appearance of the pre-ossification center on MR images is important to avoid confusing normal developmental changes with pathology.

Objective

The purpose of this study was to determine the characteristics of the trochlear pre-ossification center on MR imaging and examine age and gender differences.

Materials and methods

We retrospectively analyzed MR images from 61 children. The trochleas were categorized into three types on the basis of signal intensity (SI). Trochlear types were compared to age and gender.

Results

There was no significant difference between the ages of boys and girls. Type 1 trochleas showed homogeneous SI on all pulse sequences. Type 2 trochleas demonstrated a focus of high SI in the epiphyseal cartilage on fat-suppressed water-sensitive sequences, with high or intermediate SI on gradient-echo images (pre-ossification center). Type 3 trochleas showed low SI on fat-suppressed water-sensitive sequences and gradient-echo images. Thirty-seven trochleas were described as type 1, sixteen as type 2 and eight as type 3. ANOVAs confirmed a statistically significant difference in the age of children with type 3 trochleas and those with types 1 and 2 (P?<?0.001). Spearman rank correlations determined a positive relationship between trochlear type and age of the children (r?=?0.53).

Conclusion

Development-related changes in the trochlea follow a predictable pattern. The signal characteristics of the pre-ossification center likely reflect normal chondrocyte hypertrophy and an increase in free water in the matrix.     

Comparison of Self-Esteem and Maternal Attitude Between Children with Learning Disability and Unaffected Siblings

Objectives

To compare self-esteem and maternal attitude between children with learning disability and their unaffected siblings.

Methods

This cross sectional study was conducted at a tertiary care hospital in an urban setting. It comprised of 31 pairs of children with a learning disability, their unaffected siblings and input from their mothers. All children were assessed with Rosenberg self-esteem scale. Mothers were asked to fill Index of parental attitude (IPA) and semi structured proforma with demographic data and questionnaire about her children with a learning disability and his/her unaffected sibling.

Results

Self-esteem was found to be lower in children with learning disability. They felt they do not have much to be proud of and have a fewer number of good qualities. They are also inclined to consider themselves as failures. In factors affecting self-esteem, index of parental attitude was found to be unfavorable towards children with learning disability. Mothers felt child was interfering with their activities and was getting on their nerves. In addition, they also felt that they do not understand their child, feel like they do not love their child and wished that child was more like others they know off. More academic failures, academic difficulties and negative school report were also perceived by mother as lowering child’s self-esteem.

Conclusions

Self-esteem was lower in children with learning disability. In factors affecting self-esteem maternal attitude, academic difficulties, academic failure and negative school reports was found to be unfavorable.