Fecaloma Complicating Colostomy for Hirschprung Disease

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S67-S67
Author(s):  
Samuel Ohayi ◽  
Nnaemeka Onyishi ◽  
Kevin Chukwubuike
Keyword(s):  
Ganglion Cells ◽  
Abdominal Mass ◽  
Rectal Biopsy ◽  
Distal Portion ◽  
Definitive Treatment ◽  
First Year ◽  
Surgical Specimen ◽  
History Of ◽  
Colon Segment ◽  
First Year Of Life

Abstract Background Hirschprung disease (HD) is a congenital disorder characterized by absence of ganglion cells in the distal portion of the large intestine with resultant obstruction. It commonly manifests shortly after birth, hence its usual diagnosis in the first year of life. A defunctioning colostomy precedes definitive treatment. Prolonged obstruction following untreated HD or any condition may progressively lead to fecaloma, a mass of inspissated feces commonly in the rectosigmoid that is much harder than impacted feces. If prolonged, affected intestine looks like a tumor. We present a case of fecaloma of the transverse colon complicating colostomy done for Hirschprung disease. Case Report A 6-year-old boy with a 6-year history of difficulty with passing stool with progressive abdominal swelling had Swenson’s procedure (ie, proctectomy) and prolapsing of ganglionic colon to the anus with left hemicolectomy. He had presented to the pediatric surgery unit of our hospital 4 years previously with these symptoms with a defunctioning colostomy performed for suspected HD. He reportedly passed meconium a day after birth and subsequently passed stool once in 7 to 14 days spontaneously or after digital manipulations. Stooling frequency progressively reduced but no vomiting. Following administration of herbs, he developed diarrhea, and with worsening symptoms, he presented to our hospital. Three years postcolostomy, rectal biopsy was performed with a histology confirming aganglionosis of the rectum (HD). Barium enema had shown long-segment HD with dilated rectosigmoid colon but no fecaloma. Surgical specimen received in the histopathology laboratory consisted of colon segment comprising a colostomy stump with a ring of skin (colostomy stoma) and a proximal markedly dilated portion filled with fecaloma followed by a hypertrophied proximal portion. Histology showed presence of ganglion cells in these segments. Conclusion Fecaloma should be suspected in a protracted colostomy with abdominal mass proximal to the stoma.

scholarly journals The natural history of Canavan disease: 23 new cases and comparison with patients from literature

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Annette Bley ◽  
Jonas Denecke ◽  
Alfried Kohlschütter ◽  
Gerhard Schön ◽  
Sandra Hischke ◽  
...  
Keyword(s):  
Severity Score ◽  
Canavan Disease ◽  
Psychomotor Development ◽  
Rank Test ◽  
First Year ◽  
Study Cohort ◽  
Pooled Data ◽  
Kaplan Meier ◽  
History Of ◽  
First Year Of Life

Abstract Background Canavan disease (CD, MIM # 271900) is a rare and devastating leukodystrophy of early childhood. To identify clinical features that could serve as endpoints for treatment trials, the clinical course of CD was studied retrospectively and prospectively in 23 CD patients. Results were compared with data of CD patients reported in three prior large series. Kaplan Meier survival analysis including log rank test was performed for pooled data of 82 CD patients (study cohort and literature patients). Results Onset of symptoms was between 0 and 6 months. Psychomotor development of patients was limited to abilities that are usually gained within the first year of life. Macrocephaly became apparent between 4 and 18 months of age. Seizure frequency was highest towards the end of the first decade. Ethnic background was more diverse than in studies previously reported. A CD severity score with assessment of 11 symptoms and abilities was developed. Conclusions Early hallmarks of CD are severe psychomotor disability and macrocephaly that develop within the first 18 months of life. While rare in the first year of life, seizures increase in frequency over time in most patients. CD occurs more frequently outside Ashkenazi Jewish communities than previously reported. Concordance of phenotypes between siblings but not patients with identical ASPA mutations suggest the influence of yet unknown modifiers. A CD severity score may allow for assessment of CD disease severity both retrospectively and prospectively.

Epidemiology of Physician-Diagnosed Allergic Rhinitis in Childhood

PEDIATRICS ◽  
1994 ◽  
Vol 94 (6) ◽  
pp. 895-901
Author(s):  
Anne L. Wright ◽  
Catharine J. Holberg ◽  
Marilyn Halonen ◽  
Fernando D. Martinez ◽  
Wayne Morgan ◽  
...  
Keyword(s):  
Risk Factors ◽  
Allergic Rhinitis ◽  
Immunoglobulin E ◽  
Age At Onset ◽  
First Year ◽  
Healthy Children ◽  
Early Introduction ◽  
Maternal Cigarette Smoking ◽  
History Of ◽  
First Year Of Life

Objective. To investigate the natural history of and risk factors for allergic rhinitis in the first 6 years of life. Methods. Parents of 747 healthy children followed from birth completed a questionnaire when the child was 6 years old. Data were obtained regarding physician-diagnosed allergic rhinitis (PDAR), associated symptoms, and age at onset. Risk-factor data were taken from earlier questionnaires, and data regarding immunoglobulin E (IgE) and skin-test reactivity were obtained at age 6. Results. By the age of 6, 42% of children had PDAR. Children whose rhinitis began in the first year of life had more respiratory symptoms at age 6 and were more likely to have a diagnosis of asthma. Early introduction of foods or formula, heavy maternal cigarette smoking in the first year of life, and higher IgE, as well as parental allergic disorders, were associated with early development of rhinitis. Risk factors for PDAR that remained significant in a multivanate model included maternal history of physician-diagnosed allergy (odds ratio: 2.2, 95% confidence interval: 1.35-3.54), asthma in the child (4.06, 2.06-7.99), and IgE greater than 100 IU/mL at age 6 (1.93, 1.18-3.17). The odds for atopic as opposed to nonatopic PDAR were significantly higher only among those with high IgE and those who had dogs. Conclusion. Allergic rhinitis developing in the first years of life is an early manifestation of an atopic predisposition, which may be triggered by early environmental exposures.

scholarly journals Auditory temporal abilities in children with history of recurrent otitis media in the first years of life and persistent in preschool and school ages

CoDAS ◽  
2014 ◽  
Vol 26 (6) ◽  
pp. 494-502 ◽  
Author(s):  
Priscila Cruvinel Villa ◽  
Sthella Zanchetta
Keyword(s):  
Otitis Media ◽  
Otitis Media With Effusion ◽  
Temporal Frequency ◽  
Control Group ◽  
First Year ◽  
Normal Range ◽  
Frequency Pattern ◽  
History Of ◽  
First Year Of Life ◽  
Recurrent Otitis Media

PURPOSE: To study the temporal auditory ordering and resolution abilities in children with and without a history of early OME and ROME, as well as to study the responses according to age. METHODS: A total of 59 children were evaluated, and all of them presented pure tone thresholds within the normal range at the time of the conduction of the hearing tests. The children were divided into two groups according to the occurrence of episodes of recurrent otitis media. Then, each group was divided into two subgroups according to age: 7- and 8-year olds, and 9- and 10-year olds. All children were assessed with standard tests of temporal frequency (ordination) and gaps-in-noise (resolution). RESULTS: For the temporal abilities studied, children with a history of otitis media presented significantly lower results compared to the control group. In the frequency pattern test, the correct answers increased with age in both groups. In the identification of silence intervals, the control group showed no change in threshold regarding to age, but this change was present in the group with a history of otitis media. CONCLUSION: Episodes of otitis media with effusion in the first year of life, recurrent and persistent in preschool and school ages, negatively influence the temporal ordering and resolution abilities.

scholarly journals Operation procedure of sacrococcygeal fetus in fetu

2011 ◽  
Vol 51 (1) ◽  
pp. 58 ◽  
Author(s):  
Rochadi Rochadi
Keyword(s):  
Adrenal Gland ◽  
Abdominal Mass ◽  
Rare Condition ◽  
Mature Embryo ◽  
First Year ◽  
Fetus In Fetu ◽  
Sacrococcygeal Region ◽  
Parasitic Twin ◽  
Operation Procedure ◽  
First Year Of Life

Fetus in fetu is a condition in wich a fetiform calcified mass often presents in the abdomen of its host, a newborn. It is extremely rare condition, estimated once in 500,000 deliveries and has a 2: 1 male predominantly; with most patient presenting with an abdominal  mass in the first year of life. 5,13 The term fetus in fetu is used to point out an unequal division of totipotential cells of blastocyst where the result is the inclusion of a small cellular mass in the more mature embryo. It was encapsulated, pedunculated and represents a malformed monozygotic, monochorionic, diamniotic parasitic twin. In 80% cases, fetus in fetu is located  retroperitonealy but can be found in unusual location such as in oropharynx, neck, skull mediastinum, pelvis, iliac mesentery, adrenal gland, sacrococcygeal region and scrotal sac.

scholarly journals Cryotherapy for Lichen Striatus in an Adult – a Case Report

2015 ◽  
Vol 7 (4) ◽  
pp. 163-171
Author(s):  
Slobodan Stojanović ◽  
Marina Jovanović ◽  
Nada Vučković
Keyword(s):  
Inflammatory Infiltrate ◽  
Unknown Origin ◽  
Skin Condition ◽  
First Year ◽  
Linear Distribution ◽  
Lichen Striatus ◽  
Full Resolution ◽  
Lines Of Blaschko ◽  
History Of ◽  
First Year Of Life

Abstract Lichen striatus (linear lichenoid dermatosis) is an uncommon, self-limited, inflammatory, linear skin condition of unknown origin. The causes of linear distribution are unknown, though the pattern of lichen striatus (LS) mostly follows the lines of Blaschko (BL). The condition most commonly occurs in children between 5 and 15 years of age, usually after the first year of life. We report a 27-year-old, otherwise healthy flight attendant with LS whose diagnosis was based on: the history of sudden appearance and rapid linear spread of lesions; clinical presentation of small pink, coalescing scaly papules without umbilication or Wickham’s striae, linear distribution following one BL down a lower limb to the ankle, with a band broadening into plaque on the left buttock; histology showed some hyperkeratosis, lichenoid dermatitis similar to lichen planus, but with the presence of inflammatory infiltrate in the papillary dermis and also deeper in the perifollicular region. The inflammatory infiltrate consisted mainly of limphocytes, with some melanophages and histiocytes. There is no standard treatment for LS, and it is given for cosmetic or psychological reasons only, as we have done in our patient due to slight pruritus and occupational reasons. With regard to her occupational demands, in order to achieve satisfying results, she was successfully treated with cryotherapy, which she tolerated well, without any side effects. Cryotherapy was performed twice, with a two-week interval. Full resolution was achieved twelve weeks after cryotherapy. In conclusion, we present an adult female who developed lichen striatus suddenly three months after delivery and was successfully treated with cryotherapy.

Recurrent Dystonic Crisis and Rhabdomyolysis Treated with Dantrolene in Two Patients with Aromatic L-Amino Acid Decarboxylase Deficiency

2020 ◽  
Vol 51 (03) ◽  
pp. 229-232
Author(s):  
J. Micallef ◽  
S. Stockler-Ipsiroglu ◽  
C.D. van Karnebeek ◽  
R. Salvarinova-Zivkovic ◽  
G. Horvath
Keyword(s):  
Amino Acid ◽  
Inborn Error ◽  
Autosomal Recessive ◽  
Global Developmental Delay ◽  
Acid Decarboxylase ◽  
First Year ◽  
Amino Acid Decarboxylase ◽  
History Of ◽  
First Year Of Life ◽  
Decarboxylase Deficiency

AbstractAromatic L-amino acid decarboxylase (AADC) deficiency is a rare, autosomal recessive inborn error of metabolism in which several neurotransmitters including serotonin, dopamine, norepinephrine and epinephrine are deficient. Symptoms typically appear in the first year of life and include oculogyric crises and dystonia, hypotonia, and global developmental delay. Dystonia is of particular concern as a dystonic storm can ensue leading to rhabdomyolysis. Rhabdomyolysis can become life-threating and therefore its recognition and prompt management is of significant importance. Here we present two cases of patients with AADC deficiency and a history of dystonic crisis causing rhabdomyolysis. We hypothesize that in addition to the hypodopaminergic, a hypercholinergic state is contributing to the pathophysiology of dystonia in AADC deficiency, as well as to the associated rhabdomyolysis. We were able to prevent rhabdomyolysis in both patients with using Dantrolene and we suggest using a trial of this medication in cases of sustained dystonic crisis in AADC deficiency patients.

scholarly journals Pediatric Urachal Anomalies: Monocentric Experience and Mini-Review of Literature

Children ◽  
2022 ◽  
Vol 9 (1) ◽  
pp. 72
Author(s):  
Matthias Nissen ◽  
Phillip Rogge ◽  
Volker Sander ◽  
Mohamad Alrefai ◽  
Anna Romanova ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Abdominal Mass ◽  
First Year ◽  
Complication Rates ◽  
Symptomatic Disease ◽  
Lower Complication ◽  
Complicated Course ◽  
First Year Of Life ◽  
Surgical Unit ◽  
Non Surgical Treatment

Background: Surgery is the current mainstay for the treatment of urachal anomalies (UA). Recent literature data support the theory of a spontaneous resolution within the first year of life. The aim of this study, comprising solely surgically treated children, was to identify age specific patterns regarding symptoms and outcomes that may support the non-surgical treatment of UA. Methods: Retrospective review on the clinico-laboratory characteristics of 52 children aged < 17 years undergoing resection of symptomatic UA at our pediatric surgical unit during 2006–2017. Data was dichotomized into age > 1 (n = 17) versus < 1 year (n = 35), and complicated (pre-/post-surgical abscess formation or peritonitis, n = 10) versus non-complicated course (n = 42). Results: Children aged < 1 year comprised majority (67%) of cohort and had lower complication rates (p = 0.062). Complicated course at surgery exclusively occurred in patients aged > 1 year (p = 0.003). Additionally, complicated group was older (p = 0.018), displayed leukocytosis (p < 0.001) and higher frequencies regarding presence of abdominal pain (p = 0.008) and abdominal mass (p = 0.034) on admission. Regression analysis identified present abdominal pain (OR (95% CI), 11.121 (1.152–107.337); p = 0.037) and leukocytosis (1.435 (1.070–1.925); p = 0.016) being associated with complicated course. Conclusions: This study provides evidence that symptomatic disease course follows an age-dependent complication pattern with lower complication rates at age < 1 year. Larger, studies have to clarify, if waiting for spontaneous urachal obliteration during the first year of life comprises a reasonable alternative to surgery.

scholarly journals A Case Report on 2 years Child: Hirschprung’s Disease

2021 ◽  
pp. 558-564
Author(s):  
Anushri Kale ◽  
Aditi Badwaik ◽  
Pallavi Dhulse ◽  
Archana Maurya ◽  
Bibin Kurian
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Ganglion Cells ◽  
Past History ◽  
Rectal Biopsy ◽  
Case Presentation ◽  
Surgical History ◽  
History Of ◽  
Pull Through ◽  
Hirschprung's Disease

Introduction: Hirschsprung's disease is the most common cause of large intestinal obstructing in neonates. Hirschsprung's disease is a congenital anomaly caused by migratory failure of neural crest cells leading to abnormal innervations of the bowel .The defect begins in the internal and sphincter and extends proximally for a variable length of gut. Hirschprung's disease is a disorder of the gut caused due to congenital absence of ganglion cells in the sub-mucosal and myenteric plexus of intestine. This disease is also known as megacolon or congenital Aganglionic megacolon. Case presentation: A 2 years old male child, known case of Hirschprung’s disease, was brought to AVBRH for further management. As narrated by the mother, the child was not passing stool since birth. There was no history of abdominal distention, vomiting or fever. The child pass the stool with the help of enema which was given to the child in the morning and evening. Mast. Devansh has past history of NICU stay. In View of not passing stools, USG of abdomen and pelvis done. Endorectal pull through surgery was done on 4/02/2021 under general anesthesia. No any past surgical history. There is a past history of rectal biopsy done in 1 1/2 month of age. Conclusion:The patient was admitted in AVBRH for further management. Then the report mainly focused on surgical management and quality nursing care due to which patient was discharged without any further complication and satisfaction.

scholarly journals Epidemiological aspects of and risk factors for wheezing in the first year of life

2014 ◽  
Vol 40 (6) ◽  
pp. 617-625 ◽  
Author(s):  
Hamilton Rosendo Fogaça ◽  
Fernando Augusto de Lima Marson ◽  
Adyléia Aparecida Dalbo Contrera Toro ◽  
Dirceu Solé ◽  
José Dirceu Ribeiro
Keyword(s):  
Risk Factors ◽  
International Study ◽  
First Episode ◽  
First Year ◽  
Recurrent Wheezing ◽  
Factors Associated ◽  
High Prevalence ◽  
History Of ◽  
Tract Infections ◽  
First Year Of Life

OBJECTIVE: To determine, in a sample of infants, the prevalence of and risk factors for occasional wheezing (OW) and recurrent wheezing-wheezy baby syndrome (WBS). METHODS: Parents of infants (12-15 months of age) completed the International Study of Wheezing in Infants questionnaire. RESULTS: We included 1,269 infants residing in the city of Blumenau, Brazil. Of those, 715 (56.34%) had a history of wheezing, which was more common among boys. The prevalences of OW and WBS were 27.03% (n = 343) and 29.31% (n = 372), respectively. On average, the first wheezing episode occurred at 5.55 ± 2.87 months of age. Among the 715 infants with a history of wheezing, the first episode occurred within the first six months of life in 479 (66.99%), and 372 (52.03%) had had three or more episodes. Factors associated with wheezing in general were pneumonia; oral corticosteroid use; a cold; attending daycare; having a parent with asthma or allergies; mother working outside the home; male gender; no breastfeeding; and mold. Factors associated with WBS were a cold; physician-diagnosed asthma; ER visits; corticosteroid use; pneumonia; bronchitis; dyspnea; attending daycare; bronchodilator use; having a parent with asthma; no breastfeeding; mother working outside the home; and a dog in the household. CONCLUSIONS: The prevalence of wheezing in the studied population was high (56.34%). The etiology was multifactorial, and the risk factors were intrinsic and extrinsic (respiratory tract infections, allergies, attending daycare, and early wheezing). The high prevalence and the intrinsic risk factors indicate the need and the opportunity for epidemiological and genetic studies in this population. In addition, mothers should be encouraged to prolong breastfeeding and to keep infants under six months of age out of daycare.

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