Journal Article Extensive unilateral hyperkeratotic plaques in a blaschkoid distribution Get access M. Lynch, M. Lynch Departments of DermatologyChildren's University Hospital Dublin Ireland Correspondence: Dr Maeve Lynch, Department of Dermatology, Children's University Hospital, Temple Street, Dublin 1, Ireland E‐mail: lynchmaeve@yahoo.ie Search for other works by this author on: Oxford Academic Google Scholar N. Mulligan, N. Mulligan Department of Histopathology Mater Misericordiae University Hospital Dublin Ireland Search for other works by this author on: Oxford Academic Google Scholar D. Devaney, D. Devaney Department of Histopathology Children's University Hospital Dublin Ireland Search for other works by this author on: Oxford Academic Google Scholar C. Schilling, C. Schilling Department of Histopathology Children's University Hospital Dublin Ireland Search for other works by this author on: Oxford Academic Google Scholar A. Beausang, A. Beausang Department of Histopathology Children's University Hospital Dublin Ireland Search for other works by this author on: Oxford Academic Google Scholar P. Lenane P. Lenane Departments of DermatologyChildren's University Hospital Dublin Ireland Search for other works by this author on: Oxford Academic Google Scholar Clinical and Experimental Dermatology, Volume 39, Issue 4, 1 June 2014, Pages 544–546, https://doi.org/10.1111/ced.12307 Published: 01 June 2014

Cases of sudden unexplained death in childhood (SUDC) in Ireland in children aged > 1 year and < 5 years were examined in order to assess the quality of autopsy reporting. All SUDC cases are notified to and documented by the National Sudden Infant Death Register (NSIDR) in Ireland along with all cases of sudden infant death syndrome (SIDS) referring to sudden infant deaths less than one year of age. The database of the NSIDR in Ireland was interrogated and cases of SIDS and SUDC were compared over a fifteen-year period (1995-2009). SIDS cases whose autopsies were conducted in the same hospital in the same year as the index SUDC case were used for comparison. The autopsy report for each case was examined and modified Rushton (MR) score(s1) calculated. MR scores were compared along with the number of paediatric pathology prosectors and the year of autopsy examination between the two groups. 45 cases were registered as SUDC (age 52 - 152 weeks) between 1995-2009. Autopsy reports were available for 43/45 (95%) of these. 43 SIDS cases from the same year and site of autopsy were used for comparison. Overall MR scores were higher in the SIDS cases, with 29/43 (67%) cases obtaining the minimum arbitrary score (MAS) of > 300 compared to 25/43 (58%) of SUDC cases. Paediatric pathologists in specialist centres carried out similar numbers of SIDS autopsies and SUDC autopsies (46% SIDS, 44% SUDC). Autopsies carried out by paediatric pathologists in specialist centres met the MAS in 19/21 (90%) SIDS cases and 18/19 (95%) SUDC cases. Based on our findings we recommend referral of all SUDC cases to specialist centres for optimal autopsy examination and investigation, and that cases of sudden unexpected death in children over 1 year of age are investigated according to the same guidelines as are used for unexpected death under one year of age.

A nine-year-old girl presented with an asymptomatic eruption on her right leg which had been present for 2 years. She complained of severe acral pain and paraesthesiae for several years, despite treatment with numerous analgaesics, amitriptyline, gabapentin and carbamazepine. On occasion she had been confined to a wheelchair and required home schooling. On examination she had a unilateral eruption affecting her right thigh and lower leg (Figure 1). On closer view, there were erythematous, hyperkeratotic and haemorrhagic papules. Histopathological examination of a skin biopsy showed hyperkeratosis and dilated blood vessels in the dermis consistent with angiokeratomas. Electron microscopy demonstrated intralysosomal glycolipid deposits, arranged in a lamellar fashion, within the endothelial cells lining dermal blood vessels (Figure 2).

Prenatal DiagnosisVolume 31, Issue 12 p. 1203-1204 Research Letter Prenatal identification of an accessory lower limb J. Unterscheider, Corresponding Author J. Unterscheider [email protected] Royal College of Surgeons in Ireland, Rotunda Hospital, Dublin, Ireland Julia Unterscheider, Royal College of Surgeons in Ireland, Rotunda Hospital, Dublin, Ireland. E-mail: [email protected]Search for more papers by this authorJ. O'Byrne, J. O'Byrne Department of Neonatology, Rotunda Hospital, Dublin, IrelandSearch for more papers by this authorA. Foran, A. Foran Department of Neonatology, Rotunda Hospital, Dublin, IrelandSearch for more papers by this authorI. Robinson, I. Robinson Department of Radiology, Children's University Hospital, Temple Street, Dublin, IrelandSearch for more papers by this authorS. Ryan, S. Ryan Department of Radiology, Children's University Hospital, Temple Street, Dublin, IrelandSearch for more papers by this authorD. Devaney, D. Devaney Department of Pathology, Rotunda Hospital, Dublin, IrelandSearch for more papers by this authorJ. Gillick, J. Gillick Department of Surgery, Children's University Hospital, Temple Street, Dublin, IrelandSearch for more papers by this authorF. Malone, F. Malone Royal College of Surgeons in Ireland, Rotunda Hospital, Dublin, IrelandSearch for more papers by this authorF. Breathnach, F. Breathnach Royal College of Surgeons in Ireland, Rotunda Hospital, Dublin, IrelandSearch for more papers by this author J. Unterscheider, Corresponding Author J. Unterscheider [email protected] Royal College of Surgeons in Ireland, Rotunda Hospital, Dublin, Ireland Julia Unterscheider, Royal College of Surgeons in Ireland, Rotunda Hospital, Dublin, Ireland. E-mail: [email protected]Search for more papers by this authorJ. O'Byrne, J. O'Byrne Department of Neonatology, Rotunda Hospital, Dublin, IrelandSearch for more papers by this authorA. Foran, A. Foran Department of Neonatology, Rotunda Hospital, Dublin, IrelandSearch for more papers by this authorI. Robinson, I. Robinson Department of Radiology, Children's University Hospital, Temple Street, Dublin, IrelandSearch for more papers by this authorS. Ryan, S. Ryan Department of Radiology, Children's University Hospital, Temple Street, Dublin, IrelandSearch for more papers by this authorD. Devaney, D. Devaney Department of Pathology, Rotunda Hospital, Dublin, IrelandSearch for more papers by this authorJ. Gillick, J. Gillick Department of Surgery, Children's University Hospital, Temple Street, Dublin, IrelandSearch for more papers by this authorF. Malone, F. Malone Royal College of Surgeons in Ireland, Rotunda Hospital, Dublin, IrelandSearch for more papers by this authorF. Breathnach, F. Breathnach Royal College of Surgeons in Ireland, Rotunda Hospital, Dublin, IrelandSearch for more papers by this author First published: 05 September 2011 https://doi.org/10.1002/pd.2846Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume31, Issue12December 2011Pages 1203-1204 RelatedInformation

Background: Despite the marked reduction in SIDS, the rate of childhood mortality has remained fairly constant. Many childhood deaths are unexpected and potentially avoidable. Aim: To analyse data relating to all deaths in CUH, Temple Street, and to assess the feasibility of establishing a National Enquiry in order to identify avoidable factors in childhood death. Methods: We conducted a retrospective review of all post mortems reported over ten years. Deaths were analysed and classified as follows: expected or unexpected; unavoidable, avoidable or potentially avoidable. Results: 246 autopsies were studied. 78% were performed at the direction of a coroner. 11 deaths (4.5%) were referred to the State Pathologist. Deaths were categorised into the following groups; SIDS, (33.7%), infection (19.1%), neurological and metabolic disease (16.7%), injury/suicide (13.4%), cardiac (4.9%), miscellaneous (12.2%). A marked reduction in SIDS was noted reflecting national trends. Of the deaths attributed to accidental injury almost two thirds were in boys. All of these deaths were deemed potentially avoidable or avoidable. Of the total number of deaths at least 48% were unexpected. Just over half the deaths undergoing post mortem were avoidable or potentially avoidable. Conclusion: Our data highlights the reasons why children die in an Irish population. Our ability to assess all associated factors in detail was limited by the data available. Establishing a national enquiry into paediatric mortality would provide information to the avoidable factors associated with childhood deaths. This could inform institutional policies and public health measures to reduce the incidence of untimely and preventable deaths.

A 7-year-old boy with a history of VACTERL syndrome was found collapsed in bed. MRI had shown basilar invagination of the skull base and narrowing of the foramen magnum. Angulation, swelling and abnormal high signal at the cervicomedullary junction were felt to be secondary to compression of the medulla. Neuropathologic examination showed bilateral replacement of the medullary tegmentum by an irregularly circumscribed cellular lesion which was composed of elongated GFAP/S 100-positive cells with spindled nuclei and minimal atypia. The pathologic findings were interpreted as intramedullary schwannosis with mass effect. Schwannosis, is observed in traumatized spinal cords where its presence may represent attempted, albeit aberrant, repair by inwardly migrating Schwann cells of peripheral origin. In our view the compressive effect of the basilar invagination on this boy's medulla was of sufficient magnitude to have caused tumoral medullary schwannosis with resultant intermittent respiratory compromise leading to reflex anoxic seizures.

A 37-year-old woman was referred to the Fetal Medicine Unit for management of Rhesus alloimmunisation. The patient’s blood group was B-Rh negative and she had high titres of anti-D antibodies (20.8 IU/l), confirming rhesus alloimmunisation. Close fetal surveillance was instituted, including middle cerebral artery peak systolic velocity (MCA-PSV) Doppler assessment for fetal anaemia. At 27 weeks’ gestation the MCA-PSV Doppler became elevated suggesting significant fetal anaemia. A successful intrauterine fetal transfusion was performed through the umbilical vein at the placental cord insertion site of an anterior placenta. A transfusion of 53 cc of packed red blood cells was performed resulting in a final haematocrit of 41%. A second fetal intravascular transfusion of 50 cc of packed red cells was performed at 29 weeks’ gestation, resulting in a final fetal haematocrit of 29%. A third and final fetal transfusion was scheduled at 32 weeks’ gestation. An uncomplicated intravascular transfusion of 87 cc of packed red blood cells was performed and the final haematocrit was 47%. Real-time sonographic guidance confirmed a normal fetal heart rate throughout the 15 min procedure and for a further 5 min after completion of the transfusion. However, on reaching the prenatal ward 25 min later, nursing staff could not trace the fetal heart rate and immediate sonographic surveillance confirmed a significant fetal bradycardia at 30 b.p.m. An emergency caesarean section was performed, with a live born female infant being delivered approximately 7 min after the initial sonographic confirmation of fetal bradycardia. Despite extensive resuscitation, the infant was pronounced dead shortly thereafter. Birth weight was 1.96 kg and no obvious signs of placental abruption were noted. Histological examination of the placenta and the umbilical cord revealed a haematoma in the umbilical cord near the placental insertion site at the point of the insertion of the needle (Figure 1). Clinicopathological correlation suggested that delayed vasospasm occurred at this point in the umbilical cord resulting in decreased perfusion to the fetus and the resultant fetal bradycardia that was noted on ultrasound approximately 25 min after the successful completion of the procedure.

Malignant schwannoma (malignant peripheral nerve sheath tumour, MPNST) is a rare high-grade tumour arising from peripheral nerves. We report the case of a 3-year-old male who presented with a non-tender lesion on the dorsum of his penis. The lesion was excised and a formal circumcision performed. Histology of the lesion revealed a spindle cell tumour. Immunohistochemistry showed the tumour cells to be strongly positive for S100 and Vimentin. A diagnosis of intermediate grade malignant peripheral nerve sheath tumour was made. Malignant schwannoma is rare in children and is previously unreported in the penis in the paediatric age group without evidence of neurofibromatosis.

Ovarian masses in children are an uncommon occurrence. They represent less than 2% of all tumours in girls less than 16 years of age. Mucinous tumours of the ovary occur principally in middle adult life and are extremely rare prior to menarche. To the best of our knowledge, there are only 13 previous cases of benign mucinous cystadenoma (MCA) of the ovary in perimenarchal girls reported in the literature. We present six cases of this rare tumour. We reviewed the charts of six patients who presented with large MCA of the ovary. The patient's ages ranged from 13 to 14 years (mean 13.6 years). Two were premenarchal and four were within 1 year of menarche. All children presented with marked abdominal distension and discomfort. Except for one child who had ultrasound scan alone, all the others had either CT or MRI scan as well. Ultrasound demonstrated a large multiloculated cystic mass arising from the pelvis reaching the level of the xiphoid. CT demonstrated an enormous mass occupying almost the entire abdomen. The mass was partly solid, partly cystic and the cystic elements were multiloculated in all patients. Three patients demonstrated contralateral hydronephrosis on imaging. Laparotomy revealed a tumour arising from the left ovary in five patients and from the right ovary in one. Several litres of fluid were aspirated in order to deliver the tumour from the abdomen. All patients underwent oophorectomy or salpingo-oophorectomy. Histology revealed benign MCA of the ovary in all cases. On follow up, ranging from 2.4 to 5 years, all patients were well with no evidence of recurrence. MCA in perimenarchal girls usually affects the left ovary. Although this tumour is rare, this diagnosis should be considered in 11 to 15-year-old girls presenting with a very large abdominal mass.

A large environmental influence on phenotypic estimates of disease resistance and the complex polygenic nature of Fusarium head blight (FHB) resistance in wheat (Triticum aestivum) are impediments to developing resistant cultivars. The objective of this research was to investigate the utility of a detached leaf assay, inoculated using inoculum from isolates of Microdochium nivale var. majus, to identify components of FHB resistance among 30 entries of U.S. soft red winter wheat in the 2002 Uniform Southern FHB Nursery (USFHBN). Whole plant FHB resistance of the USFHBN entries was evaluated in replicated, mist-irrigated field trials at 10 locations in eight states during the 2001-2002 season. Incubation period (days from inoculation to the first appearance of a dull gray-green water-soaked lesion) was the only detached leaf variable significantly correlated across all FHB resistance parameters accounting for 45% of the variation in FHB incidence, 27% of FHB severity, 30% of Fusarium damaged kernels, and 26% of the variation in grain deoxynivalenol (DON) concentration. The results for incubation period contrasted with previous studies of moderately resistant European cultivars, in that longer incubation period was correlated with greater FHB susceptibility, but agreed with previous findings for the Chinese cultivar Sumai 3 and CIMMYT germ plasm containing diverse sources of FHB resistance. The results support the view that the detached leaf assay method has potential for use to distinguish between specific sources of FHB resistance when combined with data on FHB reaction and pedigree information. For example, entry 28, a di-haploid line from the cross between the moderately resistant U.S. cultivar Roane and the resistant Chinese line W14, exhibited detached leaf parameters that suggested a combination of both sources of FHB resistance. The USFHBN represents the combination of adapted and exotic germ plasm, but four moderately resistant U.S. commercial cultivars (Roane, McCormick, NC-Neuse, and Pat) had long incubation and latent periods and short lesion lengths in the detached leaf assay as observed in moderately FHB resistant European cultivars. The dichotomy in the relationship between incubation period and FHB resistance indicates that this may need to be considered to effectively combine exotic and existing/adapted sources of FHB resistance.

Gastrointestinal stromal tumors (GIST) in children are rare and their behavior has been regarded as difficult to predict on pathological criteria. We report our experience with two gastric GISTs in children aged 10 and 11 years. Both remain alive and free of disease at 5 years and 2 years respectively. Comparison of the pathological features in the resected specimens with a recently proposed guidelines for predicting outcome in this group of tumors is reported.

Case report A 14-year-old girl presented to hospital with cyclical lower abdominal pain and swelling over a 6-month period. She also had primary amenorrhoea. She had a background history of moderate developmental delay (46XX 7/14 translocation). Examination was difficult as the girl was unable to co-operate, but fullness and tenderness were detected in the lower abdomen. Pelvic ultrasound showed a grossly enlarged uterus with dilated fallopian tubes. The kidneys were outlined and appeared normal. Magnetic resonance imaging showed an enlarged bifid uterus with very dilated tubes. The uterus and tubes contained areas of mixed signal consistent with haematometra which did not extend downward to the level of the vagina. The cervix was not visualised. A diagnosis of obstruction at the level of the cervix was made. Examination under anaesthesia showed a blind ending vagina of normal length, an enlarged mobile uterus equal in size to 20 weeks’ gestational age with no communication between the vagina and the uterus.

OBJECTIVES Raised concentrations of antimony have been found in infants dying of sudden infant death syndrome (SIDS). The presumed source of this antimony is toxic gases generated from fire retardants that are present in cot mattresses. The aim of this study was to determine the role of antimony in SIDS. DESIGN Samples of liver, brain, serum, and urine were collected from all patients dying from SIDS and a group of aged matched control infants who had died of other causes. SETTING Nationwide study in Ireland. SUBJECTS 52 infants dying from SIDS and 19 control infants aged > 7 days and < 1 year. RESULTS The median concentration of antimony in the liver and brain of infants dying of SIDS was < 1 ng/g, with no difference detected between the infants dying from SIDS and the control infants. The range of antimony in the serum of infants dying of SIDS was 0.09–0.71 μg/litre (median, 0.26). Although no difference was found between infants dying from SIDS and control infants, SIDS infants were found to have higher concentrations when compared with healthy infants in the 1st year of life, probably as a result of release of antimony into serum after death. Urine antimony concentrations in infants dying from SIDS were < 3.91 ng/mg (corrected for creatinine) and similar to values found both in control infants and healthy infants. CONCLUSION There is no evidence to support a causal role for antimony in SIDS.

Polymorphic lymphoproliferative disorder is a recognised cause of upper airway obstruction in children [N. Sculerati, M. Arriga, Ann. Otol. Rhinol. Laryngol 99 (1990) 445–450]. It is associated with long-term immunosuppression therapy and frequently with Epstein–Barr virus (EBV) infection [D.W. Hanto, Annu. Rev. Med. 46 (1995) 381–394; B.D. Fletcher, H.E. Heslop, H.C. Kaste, S. Bodner, Upper airway obstruction and pulmonary abnormalities due to lymphoproliferative disease following bone marrow transplantation in children, Pediatr. Radiol. 28 (1998) 492–496]. The prevalence in reported series ranges from 4 to 13% among post-transplant children [M. Ho, R. Jaffe, G. Miller, Transplantation 45 (1988) 719–727; G.B. Hammer, S. Cao, M.G. Boltz, A. Messner, Anesthesiology 89 (1998) 263–265; B.V. Lattyak, P. Rosenthal, Post-transplant lymphoproliferative disorder presenting in the head and neck, Laryngoscope 108 (1998) 1195–1198]. This condition may present in the transplanted allograft, the gastrointestinal tract, the head and neck, and in particular in the upper airway. Previously reported cases of upper airway obstruction have been in the supraglottis, Waldeyer's ring, the glottis, and one case of an intra tracheal mass [M. Ho, R. Jaffe, G. Miller, Transplantation 45 (1988) 719–727; G.B. Hammer, S. Cao, M.G. Boltz, A. Messner, Anesthesiology 89 (1998) 263–265]. We report a case of post-transplant lymphoproliferative disorder in the sub-glottis causing acute upper airway obstruction with negative (EBV) serology.

Alveolar Rhabdomyosarcoma is a small round cell tumour in which may be radiologically and histologically difficult to differentiate from other small round cell tumours such as lymphoma, neuroblastoma and Ewing's tumours. We report a case in infancy of disseminated alveolar rhabdomyosarcoma with symmetrical renal and bony metastases.

Persistent hypoglycaemia in infancy is most commonly caused by hyperinsulinism. A case is reported of the somatic loss of the maternal 11p in an insulin secreting focal adenoma in association with a germline SUR-1 mutation on the paternal allele in a baby boy with hyperinsulinism diagnosed at 49 days old. A reduction to homozygosity of an SUR-1 mutation is proposed as a critical part of the cause of focal hyperinsulinism.

No AccessJournal of UrologyPediatric Urology1 Jul 1997Mesothelioma of Tunica Vaginalis Testis in a Child M.A. Khan, P. Puri, and D. Devaney M.A. KhanM.A. Khan , P. PuriP. Puri , and D. DevaneyD. Devaney View All Author Informationhttps://doi.org/10.1097/00005392-199707000-00070AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "Mesothelioma of Tunica Vaginalis Testis in a Child." The Journal of Urology, 158(1), pp. 198–199 References 1 : Malignant mesothelioma of the tunica vaginalis.. A clinicopathologic analysis of 11 cases with review of the literature. Amer. J. Surg. Path.1995; 19: 815. Google Scholar 2 : Malignant mesothelioma of the tunica vaginalis testis.. J. Clin. Oncol.1984; 2: 447. Google Scholar 3 : Nodular mesothelial hyperplasia in hernia sacs: a benign reactive condition simulating a neoplastic process.. Cancer1975; 35: 165. Google Scholar From the Departments of Paediatric Surgery and Pathology, Children's Hospital and Children's Research Centre, Our Lady's Hospital for Sick Children, Dublin, Ireland.© 1997 by American Urological Association, Inc.FiguresReferencesRelatedDetails Volume 158Issue 1July 1997Page: 198-199 Advertisement Copyright & Permissions© 1997 by American Urological Association, Inc.MetricsAuthor Information M.A. Khan More articles by this author P. Puri More articles by this author D. Devaney More articles by this author Expand All Advertisement PDF downloadLoading ...