Childhood autoimmune hepatitis in a paediatric unit of a tertiary care hospital

INTRODUCTION

Although childhood autoimmune hepatitis (AIH) has been extensively investigated in the West, data on AIH in the East is lacking. We aimed to investigate AIH’s clinical, biochemical and histological features, as well as its outcomes, in one of Singapore’s two major paediatric units.

METHODS

This was a retrospective study of children diagnosed with AIH in the paediatric unit of National University Hospital, Singapore, over the last 12 years. Children with de novo AIH after liver transplantation were excluded. The demographic and clinical features of the patients, and their laboratory, treatment and clinical outcomes were reviewed.

RESULTS

This study comprised ten patients (six females, four males), with a median age of 5.1 (range 2.1–13.8) years at diagnosis. Five patients had inflammatory bowel disease (IBD). Seven patients had type 1 AIH, and three had autoimmune sclerosing cholangitis (ASC) and IBD; none had type 2 AIH. The median level of aspartate aminotransferase at diagnosis was 183 (range 45–2,649) U/L. Prednisolone 1 mg/kg/day was prescribed at diagnosis for eight patients. Two patients were lost to follow-up and were treated symptomatically when they re-presented with end-stage liver disease. Azathioprine or mycophenolate mofetil was prescribed after 3–7 months of treatment. Normalisation of aminotransferase levels took an average of 5.3 (range 1–39) months.

CONCLUSION

AIH is a rare but important cause of liver pathology. Children in this region with elevated aminotransferases or unexplained hepatomegaly should be screened for AIH.     

Distribution of branchial anomalies in a paediatric Asian population

INTRODUCTION

The objective of the present study was to review the distribution and incidence of branchial anomalies in an Asian paediatric population and highlight the challenges involved in the diagnosis of branchial anomalies.

METHODS

This was a retrospective chart review of all paediatric patients who underwent surgery for branchial anomalies in a tertiary paediatric hospital from August 2007 to November 2012. The clinical notes were correlated with preoperative radiological investigations, intraoperative findings and histology results. Branchial anomalies were classified based on the results of the review.

RESULTS

A total of 28 children underwent surgery for 30 branchial anomalies during the review period. Two children had bilateral branchial anomalies requiring excision. Of the 30 branchial anomalies, 7 (23.3%) were first branchial anomalies, 5 (16.7%) were second branchial anomalies, 3 (10.0%) were third branchial anomalies, and 4 (13.3%) were fourth branchial anomalies (one of the four patients with fourth branchial anomalies had bilateral branchial anomalies). In addition, seven children had 8 (26.7%) branchial anomalies that were thought to originate from the pyriform sinus; however, we were unable to determine if these anomalies were from the third or fourth branchial arches. There was inadequate information on the remaining 3 (10.0%) branchial anomalies for classification.

CONCLUSION

The incidence of second branchial anomalies appears to be lower in our Asian paediatric population, while that of third and fourth branchial anomalies was higher. Knowledge of embryology and the related anatomy of the branchial apparatus is crucial in the identification of the type of branchial anomaly.     

Dislocation of the penis: a rare complication after traumatic pelvic injury

Traumatic injury to the male external genitalia is frequently encountered, but acute traumatic dislocation of the penile structure is extremely rare, with only a few reports found in the literature. We herein report the case of a 21-year-old man who sustained blunt trauma to the pelvis following a motor vehicle accident, and had features suspicious of penile dislocation. With the use of computed tomography and bedside ultrasonography, a diagnosis of penile dislocation was made, which was subsequently confirmed intraoperatively. Immediate surgical intervention via gentle manipulation of the penile tissue back to its native position was performed in order to restore normal anatomy. The exact mechanism of penile dislocation is not known. However, circumferential laceration around the foreskin causing degloving injury of the penis is suggested in our patient.     

Leptospirosis in pregnancy with pathological fetal cardiotocography changes

We report the case of a 33-year-old primigravida who presented at 37 weeks of gestation with symptoms suggestive of acute fatty liver of pregnancy, but was later diagnosed with leptospirosis (i.e. Weil''s disease or syndrome) on serological testing. Cardiotocography showed fetal distress, and an emergency Caesarean section was performed. A healthy neonate with no evidence of congenital leptospirosis was delivered. The patient was treated with intravenous ceftriaxone and discharged well 13 days after admission. Herein, we discuss the patient''s clinical presentation and the cardiotocography changes observed in leptospiral infection, and review the current literature.     

Orbital pseudotumour as a presentation of paediatric ulcerative colitis

A 2-year-old girl presented with a one-day history of acute-onset bilateral painful, swollen eyes and a two-month history of loose stools. Physical examination revealed a right eyelid swelling with proptosis. Magnetic resonance imaging revealed a right orbital pseudotumour. The patient responded well to treatment with intravenous antibiotics and nonsteroidal anti-inflammatory drugs. However, three weeks later, she was readmitted with a vasculitic lesion over her left upper chest, with mucous-bloody diarrhoea. Histopathology confirmed the diagnosis of ulcerative colitis. The patient was treated with intravenous pulse methylprednisolone and sulphasalazine. Two weeks after discharge, she was readmitted for cutaneous vasculitis and worsening diarrhoea. The patient’s bowel and extraintestinal diseases resolved upon addition of infliximab to her treatment regimen. Her inflammatory markers also normalised. Azathioprine was subsequently added. Infliximab was discontinued after four doses and prednisolone was tapered off. The patient remained well without any flare-up after 24 months of follow-up.     

Bilateral discoid medial menisci: a rare phenomenon

Discoid medial meniscus is a relatively rare pathology of the knee joint, with bilateral cases even rarer. Herein, we report the case of a 25-year-old man diagnosed with discoid medial meniscus in the right knee with a horizontal tear. Increased cupping of the medial condyle of the tibia, widening of the medial joint space and the presence of discoid meniscus in the right knee prompted investigation of the asymptomatic left knee with magnetic resonance imaging. The contralateral asymptomatic knee also showed evidence of discoid medial meniscus. The symptomatic knee was successfully treated by arthroscopic partial meniscectomy, with excellent functional outcome.     

Infant lumbar and thoracic epidurals for abdominal surgeries: cases in a paediatric tertiary institution

INTRODUCTION

There is strong evidence that epidural analgesia provides good postoperative pain relief in adults, but its use in infants is less established. In this retrospective study, we present our experience with managing infant epidural analgesia for abdominal surgeries in a tertiary paediatric institution.

METHODS

The records of 54 infants who had received a thoracic or lumbar epidural as perioperative analgesia for abdominal surgeries were included. The mean age of the infants was 6.1 (standard deviation [SD] 3.8) months and their mean weight was 6.8 kg (SD 1.8). Most (63%) had an ASA (American Society of Anesthesiologists) status of 2 and all underwent elective gastrointestinal, urogenital, hepatobiliary or retroperitoneal surgeries. 20 catheters (37.0%) were inserted in the thoracic region and 33 (61.1%) in the lumbar region.

RESULTS

A total of 52 (96.3%) catheters provided adequate intraoperative analgesia and 36 (66.7%) provided effective analgesia for the postoperative period. Active management of epidural analgesia, such as through epidural top-ups and infusion rate adjustment, was necessary to optimise analgesia in 22 (44%) of the 50 patients postoperatively. Reasons for premature catheter removal were mainly technical issues such as catheter disconnection, leakage and blockage.

CONCLUSION

Our data suggests that in experienced hands, specialised settings and active management, the success rate of epidural analgesia in infants undergoing major abdominal surgeries is high and without major incident.     

Clinical profile and visual outcome of traumatic paediatric cataract in suburban Malaysia: a ten-year experience

INTRODUCTION

Available data on traumatic cataract in Asian children is primarily confined to South Asian countries. We aimed to describe the demographics, nature of injury and visual outcomes of Malaysian children with traumatic cataract from a suburban area, and discuss the literature on Asian children with this condition.

METHODS

We conducted a retrospective study of 29 children below 17 years of age who were diagnosed with traumatic paediatric cataract and who attended Hospital Universiti Sains Malaysia, Kelantan, Malaysia, between January 2000 and December 2010. Follow-up periods ranged from 12 to 120 months. Demographic data, clinical features, mechanism and extent of injury, and final visual outcome were recorded.

RESULTS

The study population was predominantly male. The right eye was injured in 62.07% of patients. A majority of patients had penetrating injuries, with the most common cause being injury by an organic foreign body (24.14%). Presenting visual acuity worse than 6/60 was observed in 68.97% of patients. Only 34.48% of patients had a final corrected visual acuity of 6/12 and better. 55.18% of patients were operated on within less than one month of their injuries. A majority of children sustained concurrent injuries to the anterior segment structures. Corneal opacity and amblyopia were the most common causes of poor final visual acuity.

CONCLUSION

Health education and awareness are essential tools that can prevent avoidable blindness due to traumatic cataract in the paediatric population. The importance of rehabilitation programmes for these patients should be emphasised.     

Dural metastasis of nasopharyngeal carcinoma: rare,but worth considering

Metastasis of nasopharyngeal carcinoma (NPC) to the dura, an extremely rare condition, can be symptomatically silent and mistaken for a benign entity radiographically. Missed diagnosis can lead to serious consequences or prove immediately fatal. We report a woman with dural metastasis of NPC that mimicked a meningioma on radiography. Craniectomy with tumour resection was performed due to rapid progression from the onset of symptoms to disability. The patient was still alive two years after surgery. This case emphasises the need to keep in mind the possibility of dural metastasis of NPC in patients with abnormal imaging features. This would not only avoid wrong and optimistic diagnosis, but also allow for appropriate treatment in a timely manner. To our knowledge, this is the first report of metastasis of NPC to the dura. We provide detailed information on the neoplastic lesion, which masqueraded as a benign entity and caused potentially fatal consequences.     

Hepatic tuberculosis: a rare cause of fluorodeoxyglucose hepatic superscan with background suppression on positron emission tomography

Fluorodeoxyglucose (FDG) hepatic superscan refers to the diffuse intense uptake of 18F-FDG in the liver on positron emission tomography (PET), with reduced physiological activity in the brain and heart. The common causes include lymphoma and metastasis. In this case report, we describe the imaging features of tuberculosis as a rare cause of FDG hepatic superscan. PET imaging may be the only clue to a diagnosis of hepatic tuberculosis, as other imaging modalities may demonstrate only nonspecific hepatomegaly. It is important to consider this entity in the differential diagnosis of patients presenting with FDG hepatic superscan and proceed with liver biopsy for a definitive diagnosis.     

Isolated and bilateral simultaneous facial palsy disclosing early human immunodeficiency virus infection

Bilateral lower motor neuron type facial palsy is an unusual neurological disorder. There are few reports that associate it with the human immunodeficiency virus (HIV) infection on initial presentation. A 51-year-old married woman, who was previously healthy and had no risk of HIV infection, presented solely with bilateral simultaneous facial palsy. A positive HIV serology test was confirmed by an enzyme-linked immunosorbent assay test. Following a short course of oral prednisolone, the patient recovered completely from facial palsy in three months, even though an antiretroviral treatment was suspended. Exclusion of HIV infection in patients with bilateral facial palsy is essential for early diagnosis and management of HIV.     

Challenges in the management of a rare case of extensive retroperitoneal haemangioma in a pregnant woman

Haemangioma of the retroperitoneal space is a rare benign capillary malformation, which can grow significantly in pregnancy due to the multiple associated cardiovascular changes. We herein describe the case of a pregnant woman with an extensive right retroperitoneal haemangioma extending from the level of the renal hilum, across the lateral anterior abdominal wall and into the thigh. We also highlight the challenges faced in the management of the patient’s delivery process. To the best of our knowledge, this is the first case of such nature and severity described in the English literature.     

Struma ovarii: management and follow-up of a rare ovarian tumour

INTRODUCTION

Struma ovarii represents about 1.0% of all ovarian tumours. While management involves surgery, there is a paucity of data regarding the extent and approach of surgery, and postoperative management. This study aimed to delineate the management of struma ovarii, its associated complications, and postoperative follow-up and investigations.

METHODS

We retrospectively reviewed cases of benign struma ovarii treated at KK Women’s and Children’s Hospital, Singapore, between January 2000 and May 2011.

RESULTS

A total of 68 patients underwent surgical removal of ovarian cyst or mass (24 cystectomy, 20 salpingo-oopherectomy and 24 total hysterectomy and bilateral salpingo-oopherectomy). Of the 68 surgeries, 39 (57.4%) included intraoperative frozen section sampling or procedures for staging of ovarian malignancy. The majority (73.5%) of surgeries were laparotomies. Histology revealed benign struma ovarii in all (98.5%) but one patient. Only 7 (10.3%) patients had postoperative complications – 3 wound-related, 2 thyroid-related, 1 incisional hernia and 1 nonspecific. The mean length of hospital stay was 4.2 days. During follow-up, 45 (66.2%) patients required no additional investigations. The most common investigation done was ultrasonography (n = 18, 26.5%). While no recurrences were diagnosed histologically, two patients were subsequently found to have complex/dermoid ovarian cysts on the ipsilateral side of the previous struma ovarii on ultrasonography.

CONCLUSION

Simple surgery is recommended for patients with struma ovarii, especially if they have fertility potential. Laparoscopic surgery is the recommended approach due to its shorter recovery time and lower morbidity. Most patients do not require extended periods of follow-up or postoperative investigations.     

Burden of blood transmitted infections in substance users admitted for inpatient treatment in Singapore and the associated factors

INTRODUCTION

There is a paucity of local data on the prevalence of blood transmitted infections (BTIs), such as hepatitis B, hepatitis C and human immunodeficiency virus (HIV) infections, among illicit drug users. This study aimed to examine the prevalence of BTIs among substance-dependent inpatients and identify the factors associated with BTIs.

METHODS

We conducted a retrospective case note analysis of 170 inpatients who had a history of substance dependence and were seen at the National Addictions Management Service, Singapore, between 1 June 2009 and 31 May 2010.

RESULTS

The majority of the 170 inpatients were male (88.2%) and Chinese (58.2%). The mean age of the patients was 43.1 years, and the main drug of abuse was opioids (86.5%). BTIs were found in 70 (41.2%) inpatients; the prevalence of hepatitis B, hepatitis C and HIV infections was 3.7%, 39.6% and 0%, respectively. Lifetime intravenous drug use, but not needle-sharing, was more common among inpatients who were positive for BTIs (p < 0.01). Logistic regression analysis showed that lifetime intravenous drug use (OR 4.3, 95% CI 1.7–10.8, p < 0.01) was the only significant predictor of BTI.

CONCLUSION

41.2% of the substance users seeking help were positive for at least one BTI. Lifetime intravenous drug users were found to be more than four times more likely to have a BTI. Early detection and prevention is essential to improve prognosis.     

When babies turn yellow

Neonatal jaundice is a common condition seen in the primary care setting. Most afflicted babies have physiological jaundice and their prognosis is good. However, others have pathological jaundice, which must be detected early. High levels of serum bilirubin can also result in bilirubin encephalopathy. This article describes consultation tasks in the primary care setting with the aim of providing a guide for the safe management of neonatal jaundice. They include clinical assessment of the baby’s well-being; looking out for features that suggest pathological jaundice; assessment for the presence of high-risk features; utilising appropriate laboratory tests for monitoring; assessing the degree of jaundice to decide if the child can be safely followed up in primary care; and providing advice on primary prevention measures and allaying parental concerns. The importance of stool colour examination and its role in early detection of cholestatic jaundice is emphasised.     

Central pontine myelinolysis: a rare presentation secondary to hyperglycaemia

Central pontine myelinolysis (CPM) is classically described as a demyelinating condition that results from the rapid correction of hyponatraemia. CPM has also been reported to arise from hyperglycaemia in association with concomitant acidosis, hypernatraemia and hyperosmolar syndrome. Herein, we report a rare presentation of CPM, which was purely secondary to hyperosmolar hyperglycaemia. The patient presented with ataxia and pseudobulbar affect, which evolved subacutely over a duration of two weeks. It is important to note that, in addition to acute changes in osmolality, a subacute shift secondary to hyperglycaemia may also lead to CPM.     

A rare case of multiple bronchial artery aneurysms associated with a double aortic arch

Bronchial artery aneurysm is uncommon, and the occurrence of multiple aneurysms arising from a bronchial artery is even rarer. To date, there has been only one published case report describing double bronchial artery aneurysms. We herein describe a case of three aneurysms arising from a left bronchial artery, accompanied by multiple bilateral hypertrophied bronchial and intercostobronchial arteries, as well as a double aortic arch. Bronchial artery aneurysm is potentially life-threatening, and immediate treatment is recommended to minimise the potential risk of rupture. The aneurysms in our case were successfully treated via transcatheter arterial embolisation using coils.     

Extracorporeal life support for cardiac arrest in a paediatric emergency department

The initiation of extracorporeal membrane oxygenation (ECMO) in the emergency department (ED) is a rare event. Herein, we report a case of acute fulminant myocarditis in a nine-year-old girl who was successfully resuscitated by early initiation of ECMO support in the paediatric ED of KK Women’s and Children’s Hospital, Singapore. The patient had rapidly progressed into a witnessed pulseless ventricular tachycardia on presentation, and ECMO was started in the ED following the failure of standard resuscitation measures to establish spontaneous circulation. ECMO was continued for nine days. The patient recovered well with normal neurocognitive function. The initiation of ECMO in the ED is potentially life-saving in the resuscitation of children with witnessed in-hospital cardiac arrest due to a reversible cause.     

Blue rubber bleb nevus syndrome: a rare cause of chronic gastrointestinal bleed in adults

Blue rubber bleb nevus syndrome (BRBNS) is a rare condition characterised by venous malformations in the skin, gastrointestinal tract and other parts of the body. Its presentation is usually sporadic, although cases of autosomal dominant inheritance have been reported. Usually seen in children, BRBNS presentation in adults is rare. Symptoms at presentation depend on the organs involved; patients with BRBNS may present with acute or chronic gastrointestinal bleed. We herein report a rare presentation of BRBNS in an adult who suffered from intermittent abdominal pain and melaena for three years. Contrast-enhanced computed tomography revealed a jejunojejunal intussusception with a vascular malformation as the lead point. The patient underwent laparotomy with resection of the intussuscepted bowel segment. Recovery was uneventful. In spite of a wide range of therapeutic options for the management of BRBNS described in the literature, the efficacy of those available therapies, including surgical excision, is not well established.     

Malignant mesothelioma of tunica vaginalis: an extremely rare case presenting without risk factors

Testicular tumours have many different manifestations, including hydrocele formation. Herein, we present an extremely rare case of testicular mesothelioma presenting with left hydrocele, but without risk factors. Left radical inguinal orchidectomy was performed, and pathological examination revealed a malignant mesothelioma of the tunica vaginalis of the testis. No infiltration of the spermatic cord was evident, and upon advanced radiological evaluation, no sign of metastasis was detected. Follow-up was still ongoing in our urology outpatient clinic at the time of this report. Although hydrocele is a simple and common condition that is easy to diagnose, a detailed investigation should be performed. Thus, when encountering a patient with hydrocele, the clinician should evaluate the possibility of the presence of an underlying testicular/paratesticular tumour, including a rare one such as mesothelioma of the tunica vaginalis.