Mudher Al-Adnani

Maternal pancreatic carcinoma metastatic to the placenta: a case report and literature review.

In this article, we report a case of maternal pancreatic carcinoma metastatic to the placenta. The baby is alive and well, with no evidence of tumor metastasis at the age of 1 year. We also present a comprehensive review of all maternal cancers with metastasis to the placenta and/or baby published in the English language.

Recurrent placental massive perivillous fibrin deposition associated with polymyositis: a case report and review of the literature.

We present a case of recurrent massive perivillous fibrin deposition (MPVFD) in a patient with polymyositis (PM). Massive perivillous fibrin deposition occurred in 2 consecutive pregnancies: the 1st ended in an intrauterine death at 34 weeks of gestation, while the 2nd pregnancy resulted in a live-born baby at 32 weeks of gestation. The association between MPVFD and PM has been rarely reported in the literature in the form of case reports of single pregnancies.

Pediatric Columnar Lined Esophagus vs Barrett's Esophagus: Is it the Time for a Consensus Definition?

We describe the clinical, endoscopic, and histological features of all cases of Barretts esophagus (BE) diagnosed at our institution between 2000 and 2007 following the criteria of the British Society of Gastroenterology. This society defines BE as a segment of columnar metaplasia (CLO) (whether intestinalized or not) of any length, visible endoscopically above the gastroesophageal junction and confirmed histologically. The diagnosis was challenged after immunostaining for Cdx2 (marker of intestinal differentiation). Information was collected with respect to age, symptoms, treatment, and endoscopic and histological features. Twelve children (10 males and 2 females) with a median age of 11.7 (2 to 17) years had been diagnosed with CLO-BE. Histology confirmed BE in 31 of 38 endoscopies. The initial diagnosis was reviewed according to Cdx2 results in 10 of 12 patients: Cdx2 strongly expressed in 4 cases with intestinal metaplasia (the diagnosis of BE was maintained); was negative in 4 other patients with “CLO” mucosa (reviewed diagnosis was that of expansion of the gastric cardia into the distal esophagus); and 2 patients had occasional Cdx2-positive cells showing cardia-type mucosa with goblet cells (early BE?). The estimated prevalence of CLO-BE in the pediatric population of South Yorkshire (United Kingdom) is 0.0024%, 0.8% in children referred for endoscopy and 5.5% in the children with reflux esophagitis. Characterization of the BE and confirmation of intestinal differentiation may have prognostic implications that can impact the surveillance program. Our results showed that intestinal differentiation as demonstrated with Cdx2 was only seen if goblet cells were present. A consensus definition and further studies to understand the molecular mechanisms involved in the development of BE at this age are needed.

Fowler Syndrome Presenting as a Dandy-Walker Malformation: A Second Case Report

Fowler syndrome, also known as proliferative vasculopathy and hydrocephaly-hydranencephaly, is a lethal condition characterized by hydrocephalus associated with progressive destruction of central nervous system tissue as a result of an unusual and characteristic proliferative vasculopathy. The occurrence of Fowler syndrome in consanguineous families and recurrence in both sexes are suggestive of an autosomal recessive transmission. We present the second case of Fowler syndrome presenting as a Dandy-Walker malformation, in a consanguineous family.

Eosinophils in the oesophageal mucosa: clinical, pathological and epidemiological relevance in children: a cohort study

Objectives Eosinophilic oesophagitis (EO) shows eosinophilic infiltration of the mucosa and can present with symptoms indistinguishable from gastrooesophageal reflux disease (GORD). The authors describe the clinical, endoscopic and histopathological features of all cases of histological EO presenting during 2007–2008 with a 2-year follow-up. The incidence of paediatric EO and the features of a subgroup with features of both GORD and EO (‘overlap’ syndrome (OS)) are described. Design Biopsies with an average of 15 eosinophils/high-power field (HPF) were reviewed in the cohort. OS was suggested when there was coexistence of clinical and histological features of EO and GORD (abnormal pH study), which improved with proton pump inhibitors. Setting Tertiary care. Participants All cases with ≥15 eosinophils/HPF entered the study. Primary outcome measures Patients with EO had an average of 15 eosinophils/HPF. Secondary outcome measures Other histological features of EO included microabscesses, dilated intercellular spaces, basal cell hyperplasia, papillary elongation, etc. Results 24 cases of EO were identified, 13 men and 11 women. The incidence of paediatric oesophageal eosinophilia in the region was 9/100 000 children. 11 of the 24 patients (46%) presented with some form of allergy, six with poor feeding/food aversion, five with dysphagia and four with vomiting. After follow-up, 56.5% were confirmed to have EO, 30.5% responded to treatment for GORD and were categorised as OS, 9% developed eosinophilic gastroenteritis and 4% did not have further upper gastrointestinal symptoms. Conclusions Accurate diagnosis of EO, especially the differentiation from GORD, requires appropriate clinicopathological correlation. A significant proportion of patients with eosinophilia in the mucosa also have GORD (OS). These patients improve after treating the underlying GORD. The study was registered as a Service Evaluation with the Trust (number SE74).

Drug- and alcohol-related deaths at a pediatric institution in the United kingdom.

AimThe study aimed to identify the incidence, clinical presentation, and demographic features of drug- and alcohol-related deaths diagnosed at a pediatric pathology department between 2004 and 2010. Material and MethodsDatabases of the histopathology and toxicology departments were searched. Three groups were defined as follows: (1) cause of death is toxicologically related; (2) drugs present are consistent with therapeutic range use; and (3) a drug was detected, but the contribution of this drug to the mechanism of death was not clear. ResultsFifty-five cases (36 males, 19 females; mean, 4.8 years; range, 2 hours to 17 years) were identified. This corresponded to 3.3% (55/1669) of all postmortems. Ten cases were group 1, 42 cases were group 2, and 3 cases were group 3. The results in group 1 were methadone (n = 2); methadone, alcohol, and dothiepin (n = 1); diazepam (n = 1); dothiepin (n = 1); carbon monoxide (n = 2); tramadol (n = 1); codeine and paracetamol (n = 1); and dihydrocodeine, citalopram, amitriptyline, and paracetamol (n = 1). The types of death were considered accidental (n = 2), suicide (n = 2), and undetermined (n = 6). ConclusionsThe presence of a toxin in lethal concentration was found in 10 (0.6%) of 1669 of any kind of postmortem examinations. This increased to 2.2% when the analysis was restricted to “sudden deaths.” These results demonstrate the need to conduct toxicological screening in all postmortems of this sort.

Soft Tissue Perineurioma in a Child With Neurofibromatosis Type 1: A Case Report and Review of the Literature

Soft tissue perineurioma is a rare benign peripheral nerve sheath tumor, especially in children. This manuscript presents an unusual case of soft tissue perineurioma in a 10-year-old boy with neurofibromatosis type 1. The patient presented with a lump in the region of the right breast. A subcutaneous, well circumscribed mass was removed. The cut surface was cream with a vaguely nodular appearance. Histology showed a spindle cell lesion with variable architecture and biphasic morphology. There was no nuclear atypia or pleomorphism. The differential diagnosis and other soft tissue lesions that can be seen in neurofibromatosis type 1 are discussed.

Increased number of fetal nucleated red blood cells in the placentas of term or near-term stillborn and neonates correlates with the presence of diffuse intradural hemorrhage in the perinatal period.

Release of nucleated red blood cells (nRBCs) into the peripheral blood occurs in stillbirths/neonates with a probable hypoxic mode of death and antenatal stress. We correlated the number of nRBCs in the placenta with the occurrence of intradural (IDH) and subdural hemorrhage (SDH) and the potential link with fetal hypoxia. Two groups of 22 cases each of nonmacerated term or near-term (≥36 weeks of gestational age) stillborn or newborns dying in the 1st day of life were studied. One group had IDH (with or without SDH) and the other did not have IDH or SDH. In each case, the number of nRBCs was determined in 10 consecutive placental fields at ×40. Data were analyzed with Fisher exact test, receiver operating characteristic (ROC) curve analysis, and logistic regression. There was a significant association between the diffuse IDH and increased number of nRBCs (Fisher exact test P = 0.0165). An ROC curve analysis showed that the cut-off number of nRBCs with the highest accuracy was 2.15 nRBCs/high-power field, with 79% sensitivity and 67% specificity. The presence of diffuse IDH was associated with SDH (Fisher exact test, P = 0.002). The absence of hypoxic brain change was associated with the absence of diffuse IDH (odds ratio 0.308; P = 0.039). We established a significant correlation between the release of nRBCs into the placental circulation and the occurrence of diffuse IDH and between diffuse IDH and the presence of SDH.

Complete Bilateral Agenesis of the Diaphragm: A Case Report and Literature Review

Bilateral agenesis of the diaphragm is a very rare congenital diaphragmatic defect. Bilateral congenital diaphragmatic hernia (CDH) is much more frequently associated with other anomalies compared with unilateral CDH (70% vs 30%–40%). These include cardiovascular, respiratory (other than lung hypoplasia), gastrointestinal, renal, and genital malformations. We report a case of complete bilateral agenesis of the diaphragm associated with a horseshoe kidney and an imperforate anus. These additional malformations have not previously been reported in association with complete bilateral agenesis of the diaphragm. We also present a review of previous cases of total diaphragm agenesis published in the English language and provide some insights about pathogenesis.