Management of neutropenic enterocolitis in children with cancer

Aim: To describe the symptoms, clinical management and short‐term outcome in a series of paediatric oncology patients with severe typhlitis following conservative treatment. Methods: Twelve episodes of severe typhlitis in 11 children with cancer treated at the paediatric oncology ward at Queen Silvias Children’s Hospital between 1995 and 2006 were analysed retrospectively. Data on symptoms, radiological findings, laboratory status and treatment as well as outcome were collected and analysed. Results: In all episodes, the classical signs of neutropenia, fever, abdominal pain and thickening of the bowel wall were present. All were successfully treated with bowel rest, broadspectrum antibiotics and supportive care. After recovery from typhlitis, three patients needed surgical intervention because of complications. Conclusion: A high clinical suspicion combined with radiological imaging aids early diagnosis. Predisposing factors for developing typhlitis were haematologic malignancy and treatment with chemotherapy within 3 weeks of onset. Supportive care, bowel rest including parenteral nutrition, correction of cytopenias and aggressive antimicrobial treatment is essential. Measurements of C‐reactive protein in blood may be of benefit when assessing the clinical course.     

Neurological manifestations of osteoid osteoma.

The clinical and radiological features of 38 children with osteoid osteomas were analysed retrospectively. Twenty nine patients had lesions of the femur (n = 17) or tibia (n = 12). The mean duration from the onset of symptoms to diagnosis was 13.8 months. In seven patients the history of pain and abnormalities on examination suggested a possible neurological disorder. Fourteen of 29 patients (48%) with femoral or tibial osteomas had localised muscle atrophy, and 10 patients (34%) had diminished or absent deep tendon reflexes in the affected limb. Two patients had painless lesions. Six patients had normal plain radiographs. Delay in the diagnosis of osteoid osteoma may be prevented by the knowledge that pain may be referred or radicular, that the concomitant occurrence of muscle atrophy and depressed deep tendon reflexes are relatively common findings, and that the characteristic radiological features may only appear late in the course of the disease.     

Acute osteomyelitis and septic arthritis in the neonate,risk factors and outcome

Twenty-two neonates with acute osteomyelitis (AO) or septic arthritis (SA) were included in a study based on a review of medical reports and a long-term clinical and radiological follow up. Clinical symptoms, bacteriology, risk factors, and outcome are discussed. The diagnoses were difficult, the clinical symptoms vague, fever rare and white cell count normal. Detection by plain radiological films was more efficient than by radionuclide bone scan.Staphylococcus aureus was the predominant causative organism and a shift towards group BStreptococcus in recent years was not identified. Risk factors for AO and SA were prematurity (13/22), respiratory distress syndrome (15/22) and perhaps most important: umbilical artery catheterisation (15/22). Severe sequelae were found in only 1 patient, while 3 patients had slight asymptomatic changes. The relatively favourable long-term outcome is unexplained, but may be related to early and appropriate, long lasting antibiotic treatment.     

Exploratory laparoscopy for recurrent right lower quadrant pain in a pediatric population

Recurrent or chronic abdominal pain can be a challenging problem when conventional diagnostic studies fail to identify the cause. It is estimated that up to one-third of children suffer from abdominal pain, and in this population recurrent pain can be even more challenging. Although recurrent right lower quadrant (RLQ) or periumbilical pain may be attributed to chronic appendicitis, this diagnosis remains controversial. Our aim was to evaluate pediatric patients who had undergone laparoscopic exploration for chronic RLQ abdominal pain to determine their histologic diagnosis, etiology of pain, and contributing factors that may predict a positive outcome. Patients with abdominal pain greater than 1 month in duration who ultimately underwent laparoscopic exploration and appendectomy were included in the study. Patients were excluded if an identified source of pain was discovered during preoperative workup, or if postoperative follow-up was less than 2 years. Intraoperative findings were noted, and all specimens were histologically examined with additional, subsequent independent review. Pertinent findings from preoperative diagnostic tests, mental health history, and pre and postoperative symptomatology were noted. Patient outcomes were recorded at the time of follow-up and after 2 years to assess resolution of their symptoms. Of the 44 patients studied, 31 (70.5%) had partial or complete resolution of symptoms at 2 years. Thirteen (29.5%) continued to have pain. Twenty-eight patients (63.6%) had abnormal histology identified on appendiceal examination, and 14 had other abnormalities found at laparoscopy (31.8%). Eighteen patients were being treated for psychiatric diagnosis, and 21 suffered from chronic headaches. There were no long-term complications from surgery. Long-term follow-up revealed that 70% reported complete or partial relief of their RLQ pain at 2 years. No factors were identified that may be helpful in predicating outcome in this population. While exploration was beneficial for a majority of this population, patients and parents should be warned that this intervention might not provide the relief of symptoms or provide the diagnostic answer to their pain.     

The urethral valve of Guérin and lacuna magna: clinical presentations and urodynamic findings

We described the clinical presentations, radiological findings, urodynamic patterns and endoscopic management in a series of patients with symptomatic lacuna magna (LM). The medical records of 14 boys diagnosed as LM were reviewed, retrospectively. The presence of LM was first diagnosed based on clinical history, physical exam, and radiological findings; then confirmed by urethroscopy. Urodynamic study was performed in 10 boys before and after the procedure. Transurethral incision and fulguration of the valve was performed in all patients. The mean (range) age of patients was 3.1 years (6 months to 8 years). The boys presented with dysuria (n = 9), postvoid dribbling (n = 4), hematuria (n = 3), bloody spotting (n = 3), enuresis (n = 4), and glanular irritation and pain (n = 6). The post-operative period was uneventful in all patients. The symptoms were relieved and repeat radiological exam showed complete resolution of LM in all children by the 3rd post-surgical month. Before the procedure, main urodynamic abnormalities in children were high P(detmax) and detrusor instability that significantly improved within 3-6 months after treatment. Despite evidence suggesting that a LM is a frequent, normal anatomical variant, the valve may cause symptoms such as intermittent hematuria, dysuria, and bloody spotting of underwear. The lesion may be associated with obstructive urodynamic patterns in some children. Endoscopic treatment of patients effectively relieves the symptoms and corrects the urodynamic abnormalities.     

Heterotaxia syndrome: the role of screening for intestinal rotation abnormalities.

BACKGROUND: Heterotaxia syndrome involves multiple anomalies, including cardiac malformations and intestinal rotation abnormalities. Most authors recommend routine radiological evaluation, with laparotomy and Ladd procedure if a rotation abnormality is found. AIMS: To determine if routine radiological screening is necessary, and if there is a group of children that can safely be managed expectantly. METHODS: Retrospective chart review of all children with heterotaxia syndrome from 1968 to 2002. RESULTS: Complete data were available for 177 patients. Twenty five (14%) had neonatal gastrointestinal symptoms (feeding intolerance, vomiting). Eleven of these had gastrointestinal contrast studies, of which seven were abnormal and led to surgery. Of the 152 asymptomatic neonates, nine had radiological screening and six of these were abnormal. Only one was thought to have a narrow based mesentery, but did not undergo surgery due to cardiac disease. There were no intestinal complications on follow up in this group. The other 143 asymptomatic children did not undergo radiological screening and were closely followed. Four subsequently developed gastrointestinal symptoms and had contrast studies; only one of these had malrotation and underwent a Ladd procedure. Of the remaining 139 patients who remained asymptomatic, 60 (43%) died of cardiac disease and none developed intestinal symptoms or complications related to malrotation on follow up. CONCLUSION: Asymptomatic children with heterotaxia syndrome have a low risk of adverse outcome related to intestinal rotation abnormalities. Routine screening may not be necessary as long as close follow up is done, and prompt investigation is performed for those that develop gastrointestinal symptomatology.     

Heterotaxia syndrome: the role of screening for intestinal rotation abnormalities

Background: Heterotaxia syndrome involves multiple anomalies, including cardiac malformations and intestinal rotation abnormalities. Most authors recommend routine radiological evaluation, with laparotomy and Ladd procedure if a rotation abnormality is found. Aims: To determine if routine radiological screening is necessary, and if there is a group of children that can safely be managed expectantly. Methods: Retrospective chart review of all children with heterotaxia syndrome from 1968 to 2002. Results: Complete data were available for 177 patients. Twenty five (14%) had neonatal gastrointestinal symptoms (feeding intolerance, vomiting). Eleven of these had gastrointestinal contrast studies, of which seven were abnormal and led to surgery. Of the 152 asymptomatic neonates, nine had radiological screening and six of these were abnormal. Only one was thought to have a narrow based mesentery, but did not undergo surgery due to cardiac disease. There were no intestinal complications on follow up in this group. The other 143 asymptomatic children did not undergo radiological screening and were closely followed. Four subsequently developed gastrointestinal symptoms and had contrast studies; only one of these had malrotation and underwent a Ladd procedure. Of the remaining 139 patients who remained asymptomatic, 60 (43%) died of cardiac disease and none developed intestinal symptoms or complications related to malrotation on follow up. Conclusion: Asymptomatic children with heterotaxia syndrome have a low risk of adverse outcome related to intestinal rotation abnormalities. Routine screening may not be necessary as long as close follow up is done, and prompt investigation is performed for those that develop gastrointestinal symptomatology.     

儿童原发纤维肌痛综合征的临床分析

目的探讨儿童型纤维肌痛综合征（FMS）的临床特点。方法对1997年3月-2004年11月间我院收治的6例FMS患儿的临床表现、实验室检查以及治疗效果进行回顾性分析,并复习国内外文献。结果6例FMS患儿中,5例女性患儿均以腹痛起病,1例男性患儿以广泛性疼痛及左膝关节痛起病,均误诊为其他疾病。6例FMS患儿均有全身弥漫性疼痛（平均疼痛得分8．8,压痛点数13．7个）、疲乏、睡眠障碍、气温下降及活动后疼痛加重,出现关节痛、肌痉挛、腹痛、肠激惹征及尿急各有5例患儿,痛经4例,抑郁3例,晨僵、感觉异常及焦虑各2例。实验室和器械检查基本正常,治疗效果尚可。结论儿童FMS临床并非少见,应引起重视,避免误诊。     

Cerebral sinus venous thrombosis in children

OBJECTIVE: Cerebral sinus venous thrombosis (cerebral SVT) is rare in children. Information on clinical characteristics, radiological findings and outcome is emerging. METHODS: Cases of cerebral SVT diagnosed between 1995 and 2001 were identified by a computer-assisted search using International Classification of Disease codes. Medical records were reviewed to collect information on clinical presentation, investigations, treatment and outcome. RESULTS: Sixteen cases of cerebral SVT were identified. All cases presented in association with head and neck pathology. The majority of cases presented with symptoms of raised intracranial pressure and focal neurological signs. Magnetic resonance imaging identified all cases of cerebral SVT whilst CT scanning failed to demonstrate the diagnosis in two cases. Management with anticoagulation was associated with radiological resolution of the thrombosis and normal neurological outcome. Long-term follow up demonstrated neurological deficits in greater than 40% of patients. CONCLUSION: Cerebral SVT in children is associated with significant residual neurological morbidity. Prospective studies to identify predictors of outcome and effective management interventions are required.     

Giant inflammatory polyposis causing partial obstruction and pain in "healed" ulcerative colitis in an adolescent

A 16-year-old boy with chronic ulcerative colitis developed unexplained increasingly intractable abdominal pain and rectal bleeding over several months during a period when acute colitic attacks were quiescent. No abdominal mass was palpable. The cause of symptoms was subtotal colonic obstruction due to the development of massive or giant inflammatory (pseudo) polyposis, which had caused nearly complete obliteration of the lumen of the transverse colon. The radiological and histopathological findings are presented; this case confirms the conclusion drawn in a recent retrospective surgical pathology report that giant inflammatory polyposis may produce distinct symptoms, especially pain, independent of the existence of relatively quiescent underlying inflammatory bowel disease. This unusual complication of inflammatory bowel disease deserves greater clinical awareness.     

Chronic constipation in childhood: a longitudinal study of 186 patients

One hundred eighty-six patients with chronic constipation and soiling were seen between 1975 and 1982. On follow-up, 47% had resolution of symptoms, with another 36% having their soiling controlled with either continuous or intermittent laxatives. Thirteen clinical features were analyzed in relation to outcome. The only predictor of poor outcome was the presence of soiling (p = 0.003), while the presence of abdominal pain correlated well with cure (p = 0.007). Fifty-five percent of patients had a positive family history for constipation. Significant psychological problems were present in 20% of patients; however, these did not appear to affect the outcome. Constipation was uncommon over the age of 12 years, and there was no evidence of the development of laxative dependence. These findings suggest that chronic idiopathic constipation of childhood may be a constitutional condition which tends to resolve with age.     

Mediastinal masses in children: experience with 120 cases

Primary mediastinal malignancies are rare tumors and can originate from any mediastinal organ or tissue such as thymic, neurogenic, lymphatic, germinal, or mesenchymal. The authors reviewed all cases of primary pediatric mediastinal masses diagnosed over a 25-year period to determine the pattern of presentation, the histology, and the outcome of the surgical treatment. In this study, 120 primary pediatric mediastinal mass cases diagnosed between 1985 and 2011 are retrospectively evaluated according to their age, sex, symptoms, anatomical location, surgical treatment, and histopathological evaluation. The median age of the patients was 5.8 years. There were 34 benign and 86 malign tumors. Thirty patients were asymptomatic. Common symptoms in the patients were cough, dyspnea, fatigue, fever, abdomen pain, back pain, and neurological symptoms. According to their origins, they were presented as neurogenic tumors (38.3%), lymphomas (18.3%), undifferentiated sarcomas (15%), germ cell tumors (7.5%), and the other tumors (22%) thymic pathologies, lymphangiomas, rhabdomyosarcomas, lipomas, hemangiomas, and Wilms' tumor. Complete resection of the tumor was performed in 86 patients, partial resection of the tumor was the intervention in 11 patients. In 23 patients, biopsy was undertaken. Because of the high incidence of asymptomatic or nonspecific presentation such as the upper airway disease, the presentation of a mediastinal mass in children may be challenging. Neurogenic tumors or lymphomas are indicating surgery, if possible complete resection, for both benign and malignant conditions. Although surgery is the mainstay of therapy for most mediastinal tumors, an experienced multidisciplinary approach is necessary.     

Predictive value of early radiological findings in inflicted traumatic brain injury

Aim: The aim of this study was to evaluate the value of early radiological investigations in predicting the long‐term neurodevelopmental outcome of patients with inflicted traumatic brain injury (ITBI). Methods: In 28 patients with ITBI, radiological investigations were performed during the acute phase of injury (0–3 days) and during the early post‐injury phase (4 days to 1 month). The clinical outcome in survivors (n = 24) was based on the Glasgow Outcome Score. Results: Four of 28 infants died and five were severely disabled. Six infants had moderate disability. Detection of changes in the basal ganglia (p < 0.000005) or brainstem (p < 0.01), diffuse oedema (p < 0.005), transtentorial herniation (p < 0.01), subarachnoid haemorrhage (p < 0.05) or parenchymal injury (p < 0.05) by neuroimaging during the first 3 days, and detection of changes in the basal ganglia (p < 0.0005) or brainstem (p < 0.05) or parenchymal injury (p < 0.01) during 1 month were significantly associated with poor long‐term outcome. Conclusion: Radiological findings during the first month were significantly associated with the long‐term outcome. Especially, basal ganglia lesions were associated with a poor outcome.     

Disseminated histoplasmosis

We describe two cases of disseminated histoplasmosis, which are of interest due to their severity and the infrequency of this infection in our environment. Both children were immunocompromised immigrants from Latin America who developed prolonged fever, weight loss, hepatosplenomegaly and pancytopenia. One patient had respiratory symptoms with associated alterations on x-ray, while no radiological alterations were found in the other patient. Despite administration of broad-spectrum antibiotics and extraction of samples for microbiological analysis, both patients had a rapid and fatal outcome and the diagnosis was made post mortem. Because of its severity, disseminated histoplasmosis should be considered in the differential diagnosis of fever, hepatosplenomegaly and pancytopenia, with or without alterations on chest x-ray, in immunosuppressed children who were born in or have visited endemic regions. In these patients, therapy should be started immediately without waiting for the results of diagnostic tests.     

The natural history of idiopathic chest pain in children. A follow-up study

Repetitive chest pain of obscure origin is commonly encountered in older children and adolescents. A questionnaire study was conducted to determine the long-term outcome in 31 patients diagnosed as having idiopathic recurrent chest pain after an average 4.1-year follow-up period. Although 45 percent reported having had persistent symptoms, chest pain had disappeared in 81 percent of those followed more than 3 years. Equally reassuring is the fact that in no case did occult disease subsequently appear to account for the initial symptoms. This study supports the current clinical approach of limited diagnostic evaluation and reassurance in the management of these patients.     

Management of intrinsic gliomas of the tectal plate in children. A ten-year review

The natural history, management, and long-term outcome for patients with benign, intrinsic tectal plate gliomas remain controversial in spite of their propensity to cause late-onset hydrocephalus. A 10-year retrospective review has identified 11 consecutive children with tectal plate lesions. Headache, vomiting, a decline in school performance, tremor, and complex partial seizures were common presenting symptoms. All patients presented with signs and symptoms of hydrocephalus. Magnetic resonance (MR) imaging delineated an intra-axial mass lesion of the midbrain primarily localized to the tectal plate which uniformly was hyperintense on T2-weighted imaging and had a more variable appearance on T1-weighted imaging and rare enhancement with gadolinium. No patient underwent surgical resection, chemotherapy, or radiotherapy. Three of 11 patients (27%) showed evidence of progression in size or a new focus of enhancement on MR imaging, which was clinically asymptomatic. In this series, no patient with a tectal plate lesion less than 1.5 cm in maximal diameter and without gadolinium enhancement showed any evidence of clinical or radiological progression. Although intrinsic tectal lesions in children are clinically indolent and the initial management consists of CSF diversion, these lesions may eventually progress and still warrant long-term follow-up with serial MR imaging.     

Complex regional pain syndromes in children and adolescents

Background: The purpose of the present paper was to assess efficiency of treatment and long-term functional outcome of complex regional pain syndromes (CRPS) in children who were treated in the chronic pain clinic at a major tertiary hospital in Israel.
Methods: The files of 14 children with CRPS were analyzed retrospectively. Demographic data, initiating event, referring source, time needed for referral to pain clinic, clinical evaluation, treatment, recurrence and complications were recorded.
Results: Fourteen children with CRPS types I and II were included in the study. Girls were affected in 71%. Lower extremities were affected in 57%. The median time from onset of symptoms to seeking medical help was 4.46 weeks (range 2–82 weeks). The median time to referral to pain clinic was 24.51 weeks (range 1.2–94). In 45% the referral source was the pediatrician. A total of 85.8% of patients were referred to various consultations before the pain clinic. Most children had reduced pain and improved function on non-invasive treatment approach. Invasive treatments were used in 28.5%. Full or partial recovery was accomplished in 93%. Recurrence was observed in 29%.
Conclusions: CRPS in children and adolescents is still underdiagnosed, although many of the epidemiologic features of pediatric CRPS are similar in different countries/cultures. Early recognition and management is the major factor in improving outcome and preventing resistant CRPS, but even children with delayed diagnosis still have a good outcome. The management of this disease by an experienced multidisciplinary team is recommended. Because psychosocial factors play an important role, it is recommended to provide psychological evaluation and cognitive behavioral treatment as soon as possible.     

Experience with ureteroscopic holmium laser lithotripsy in children

The purpose of the study was to evaluate the outcome of ureteroscopic holmium laser lithotripsy (UHLL) in children, taking into consideration different stone locations. Records of 15 children with ureteral calculi managed with UHLL were reviewed. All patients were evaluated with history, clinical, radiological and laboratory assessment prior to treatment. All patients were managed on an outpatient basis. After stone disintegration, if sizable fragments remained, they were retrieved using grasping forceps or stone basket extraction. Patient records were reviewed for age, sex, stone laterality, location, number and size, need for ureteral dilation, stenting and residual fragment extraction. Of the 15 children, 11 were female and 4 were male. Mean age was 8.5 years (age range 2–15 years). There was no significant difference in stone laterality (eight left and seven right ureteral stones). Main presenting symptoms were renal colic, hematuria and urinary tract infection or a combination of these symptoms. The 15 children harbored 15 ureteral stones (range 5–11 mm, mean 7.8 mm) and underwent 15 UHLL procedures. Ureteral dilation was performed in 14 patients using balloon dilators. Stone retrieval was done in all patients. DJ stents were placed at the conclusion of the procedure in 11 patients. Complete stone clearance was achieved at the end of the procedure in all patients (success rate 100%). No complications were encountered during or after the procedure. This study confirms the effectiveness and safety of ureteroscopy and holmium laser in the treatment of ureteral stones in children regardless of stone location.     

Graft-versus-host disease: gastrointestinal involvement with a rectovaginal fistula

Bone marrow transplantation is an accepted form of treatment for aplastic anemia and end-stage hematological malignancies. Despite a satisfactory outcome in selected patients, more than one-half develop acute graft-versus-host disease (GVHD), characterized typically by involvement of the skin, liver, and gastrointestinal tract. We describe an unusual case in which a 10-year-old girl developed gastrointestinal symptoms as the only manifestation of GVHD. The patient presented with a rectovaginal fistula and had clinical, radiological, and histological features of gastrointestinal GVHD without evidence of liver or skin involvement.     

Intervertebral disc herniations (limbus vertebrae) in pediatric patients: Report of 15 cases

A revision of 15 cases of back pain and radiological features characteristic of anterior or posterior limbus vertebrae is presented. We comment on the radiological findings observed in the various imaging studies performed (conventional radiology, CT and MRI), which were attributed to the herniation of disc material into the vertebral body. In three patients who were followed up 12 years after the diagnosis, the initial roentgenograms of limbus vertebrae progressed in adult hood into radiological images characteristic of Schmorl's hermia as a sequela.