Hirschsprung Disease - Approach & ManagementVikas V
Hirschsprung Disease. - A developmental Disorder of Intrinsic Component of Enteric Nervous System.
Also known Congenital Megacolon.
This Presentation deals with The eitology, presentation, diagnosis, medical and surgical management & complications of the same.
POSTERIOR URETHRAL VALVES- Pediatric Surgery
• Dear viewers,
• Greetings from “ Surgical Educator”
• Today I have uploaded one more video in Pediatric Surgery/Pediatric Urology- “ Posterior Urethral Valves”
• Posterior Urethral Valves is the congenital cause for Bladder Outlet Obstruction, resulting in abnormal development of the kidneys as well as the bladder.
• In this video, I talked about the learning outcomes, introduction, etiopathogenesis, clinical features, investigations, differential diagnosis, treatment, follow-up and prognosis of “ Posterior Urethral Valves”
• I hope you will enjoy the video for its educational value.
• You can watch all my teaching videos in the following links
• surgicaleducator.blogspot.com youtube.com/c/surgicaleducator
• Thank you for watching the video.
Hirschsprung Disease - Approach & ManagementVikas V
Hirschsprung Disease. - A developmental Disorder of Intrinsic Component of Enteric Nervous System.
Also known Congenital Megacolon.
This Presentation deals with The eitology, presentation, diagnosis, medical and surgical management & complications of the same.
POSTERIOR URETHRAL VALVES- Pediatric Surgery
• Dear viewers,
• Greetings from “ Surgical Educator”
• Today I have uploaded one more video in Pediatric Surgery/Pediatric Urology- “ Posterior Urethral Valves”
• Posterior Urethral Valves is the congenital cause for Bladder Outlet Obstruction, resulting in abnormal development of the kidneys as well as the bladder.
• In this video, I talked about the learning outcomes, introduction, etiopathogenesis, clinical features, investigations, differential diagnosis, treatment, follow-up and prognosis of “ Posterior Urethral Valves”
• I hope you will enjoy the video for its educational value.
• You can watch all my teaching videos in the following links
• surgicaleducator.blogspot.com youtube.com/c/surgicaleducator
• Thank you for watching the video.
HIRSCHSPRUNG DISEASE of neonate wrr.pptxShambelNegese
disease is a condition that affects the large intestine (colon) and causes problems with passing stool. The condition is present at birth (congenital) as a result of missing nerve cells in the muscles of the baby's colon.
Small intestine/Intestinal obstruction/crohns disease/ileostomy/viscous organ...RajeevPandit10
all about small intestine, anatomy, physiology, intestinal obstruction, crohns disease/ileostomy/viscous organ perforation, meckels diverticulum, mysenteric ischemia, short bowel syndrome, celiac disease
- Video recording of this lecture in English language: https://youtu.be/lK81BzxMqdo
- Video recording of this lecture in Arabic language: https://youtu.be/Ve4P0COk9OI
- Link to download the book free: https://nephrotube.blogspot.com/p/nephrotube-nephrology-books.html
- Link to NephroTube website: www.NephroTube.com
- Link to NephroTube social media accounts: https://nephrotube.blogspot.com/p/join-nephrotube-on-social-media.html
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Ve...kevinkariuki227
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
Ethanol (CH3CH2OH), or beverage alcohol, is a two-carbon alcohol
that is rapidly distributed in the body and brain. Ethanol alters many
neurochemical systems and has rewarding and addictive properties. It
is the oldest recreational drug and likely contributes to more morbidity,
mortality, and public health costs than all illicit drugs combined. The
5th edition of the Diagnostic and Statistical Manual of Mental Disorders
(DSM-5) integrates alcohol abuse and alcohol dependence into a single
disorder called alcohol use disorder (AUD), with mild, moderate,
and severe subclassifications (American Psychiatric Association, 2013).
In the DSM-5, all types of substance abuse and dependence have been
combined into a single substance use disorder (SUD) on a continuum
from mild to severe. A diagnosis of AUD requires that at least two of
the 11 DSM-5 behaviors be present within a 12-month period (mild
AUD: 2–3 criteria; moderate AUD: 4–5 criteria; severe AUD: 6–11 criteria).
The four main behavioral effects of AUD are impaired control over
drinking, negative social consequences, risky use, and altered physiological
effects (tolerance, withdrawal). This chapter presents an overview
of the prevalence and harmful consequences of AUD in the U.S.,
the systemic nature of the disease, neurocircuitry and stages of AUD,
comorbidities, fetal alcohol spectrum disorders, genetic risk factors, and
pharmacotherapies for AUD.
New Drug Discovery and Development .....NEHA GUPTA
The "New Drug Discovery and Development" process involves the identification, design, testing, and manufacturing of novel pharmaceutical compounds with the aim of introducing new and improved treatments for various medical conditions. This comprehensive endeavor encompasses various stages, including target identification, preclinical studies, clinical trials, regulatory approval, and post-market surveillance. It involves multidisciplinary collaboration among scientists, researchers, clinicians, regulatory experts, and pharmaceutical companies to bring innovative therapies to market and address unmet medical needs.
These simplified slides by Dr. Sidra Arshad present an overview of the non-respiratory functions of the respiratory tract.
Learning objectives:
1. Enlist the non-respiratory functions of the respiratory tract
2. Briefly explain how these functions are carried out
3. Discuss the significance of dead space
4. Differentiate between minute ventilation and alveolar ventilation
5. Describe the cough and sneeze reflexes
Study Resources:
1. Chapter 39, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 34, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 17, Human Physiology by Lauralee Sherwood, 9th edition
4. Non-respiratory functions of the lungs https://academic.oup.com/bjaed/article/13/3/98/278874
NVBDCP.pptx Nation vector borne disease control programSapna Thakur
NVBDCP was launched in 2003-2004 . Vector-Borne Disease: Disease that results from an infection transmitted to humans and other animals by blood-feeding arthropods, such as mosquitoes, ticks, and fleas. Examples of vector-borne diseases include Dengue fever, West Nile Virus, Lyme disease, and malaria.
Lung Cancer: Artificial Intelligence, Synergetics, Complex System Analysis, S...Oleg Kshivets
RESULTS: Overall life span (LS) was 2252.1±1742.5 days and cumulative 5-year survival (5YS) reached 73.2%, 10 years – 64.8%, 20 years – 42.5%. 513 LCP lived more than 5 years (LS=3124.6±1525.6 days), 148 LCP – more than 10 years (LS=5054.4±1504.1 days).199 LCP died because of LC (LS=562.7±374.5 days). 5YS of LCP after bi/lobectomies was significantly superior in comparison with LCP after pneumonectomies (78.1% vs.63.7%, P=0.00001 by log-rank test). AT significantly improved 5YS (66.3% vs. 34.8%) (P=0.00000 by log-rank test) only for LCP with N1-2. Cox modeling displayed that 5YS of LCP significantly depended on: phase transition (PT) early-invasive LC in terms of synergetics, PT N0—N12, cell ratio factors (ratio between cancer cells- CC and blood cells subpopulations), G1-3, histology, glucose, AT, blood cell circuit, prothrombin index, heparin tolerance, recalcification time (P=0.000-0.038). Neural networks, genetic algorithm selection and bootstrap simulation revealed relationships between 5YS and PT early-invasive LC (rank=1), PT N0—N12 (rank=2), thrombocytes/CC (3), erythrocytes/CC (4), eosinophils/CC (5), healthy cells/CC (6), lymphocytes/CC (7), segmented neutrophils/CC (8), stick neutrophils/CC (9), monocytes/CC (10); leucocytes/CC (11). Correct prediction of 5YS was 100% by neural networks computing (area under ROC curve=1.0; error=0.0).
CONCLUSIONS: 5YS of LCP after radical procedures significantly depended on: 1) PT early-invasive cancer; 2) PT N0--N12; 3) cell ratio factors; 4) blood cell circuit; 5) biochemical factors; 6) hemostasis system; 7) AT; 8) LC characteristics; 9) LC cell dynamics; 10) surgery type: lobectomy/pneumonectomy; 11) anthropometric data. Optimal diagnosis and treatment strategies for LC are: 1) screening and early detection of LC; 2) availability of experienced thoracic surgeons because of complexity of radical procedures; 3) aggressive en block surgery and adequate lymph node dissection for completeness; 4) precise prediction; 5) adjuvant chemoimmunoradiotherapy for LCP with unfavorable prognosis.
Flu Vaccine Alert in Bangalore Karnatakaaddon Scans
As flu season approaches, health officials in Bangalore, Karnataka, are urging residents to get their flu vaccinations. The seasonal flu, while common, can lead to severe health complications, particularly for vulnerable populations such as young children, the elderly, and those with underlying health conditions.
Dr. Vidisha Kumari, a leading epidemiologist in Bangalore, emphasizes the importance of getting vaccinated. "The flu vaccine is our best defense against the influenza virus. It not only protects individuals but also helps prevent the spread of the virus in our communities," he says.
This year, the flu season is expected to coincide with a potential increase in other respiratory illnesses. The Karnataka Health Department has launched an awareness campaign highlighting the significance of flu vaccinations. They have set up multiple vaccination centers across Bangalore, making it convenient for residents to receive their shots.
To encourage widespread vaccination, the government is also collaborating with local schools, workplaces, and community centers to facilitate vaccination drives. Special attention is being given to ensuring that the vaccine is accessible to all, including marginalized communities who may have limited access to healthcare.
Residents are reminded that the flu vaccine is safe and effective. Common side effects are mild and may include soreness at the injection site, mild fever, or muscle aches. These side effects are generally short-lived and far less severe than the flu itself.
Healthcare providers are also stressing the importance of continuing COVID-19 precautions. Wearing masks, practicing good hand hygiene, and maintaining social distancing are still crucial, especially in crowded places.
Protect yourself and your loved ones by getting vaccinated. Together, we can help keep Bangalore healthy and safe this flu season. For more information on vaccination centers and schedules, residents can visit the Karnataka Health Department’s official website or follow their social media pages.
Stay informed, stay safe, and get your flu shot today!
Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
Title: Sense of Taste
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the structure and function of taste buds.
Describe the relationship between the taste threshold and taste index of common substances.
Explain the chemical basis and signal transduction of taste perception for each type of primary taste sensation.
Recognize different abnormalities of taste perception and their causes.
Key Topics:
Significance of Taste Sensation:
Differentiation between pleasant and harmful food
Influence on behavior
Selection of food based on metabolic needs
Receptors of Taste:
Taste buds on the tongue
Influence of sense of smell, texture of food, and pain stimulation (e.g., by pepper)
Primary and Secondary Taste Sensations:
Primary taste sensations: Sweet, Sour, Salty, Bitter, Umami
Chemical basis and signal transduction mechanisms for each taste
Taste Threshold and Index:
Taste threshold values for Sweet (sucrose), Salty (NaCl), Sour (HCl), and Bitter (Quinine)
Taste index relationship: Inversely proportional to taste threshold
Taste Blindness:
Inability to taste certain substances, particularly thiourea compounds
Example: Phenylthiocarbamide
Structure and Function of Taste Buds:
Composition: Epithelial cells, Sustentacular/Supporting cells, Taste cells, Basal cells
Features: Taste pores, Taste hairs/microvilli, and Taste nerve fibers
Location of Taste Buds:
Found in papillae of the tongue (Fungiform, Circumvallate, Foliate)
Also present on the palate, tonsillar pillars, epiglottis, and proximal esophagus
Mechanism of Taste Stimulation:
Interaction of taste substances with receptors on microvilli
Signal transduction pathways for Umami, Sweet, Bitter, Sour, and Salty tastes
Taste Sensitivity and Adaptation:
Decrease in sensitivity with age
Rapid adaptation of taste sensation
Role of Saliva in Taste:
Dissolution of tastants to reach receptors
Washing away the stimulus
Taste Preferences and Aversions:
Mechanisms behind taste preference and aversion
Influence of receptors and neural pathways
Impact of Sensory Nerve Damage:
Degeneration of taste buds if the sensory nerve fiber is cut
Abnormalities of Taste Detection:
Conditions: Ageusia, Hypogeusia, Dysgeusia (parageusia)
Causes: Nerve damage, neurological disorders, infections, poor oral hygiene, adverse drug effects, deficiencies, aging, tobacco use, altered neurotransmitter levels
Neurotransmitters and Taste Threshold:
Effects of serotonin (5-HT) and norepinephrine (NE) on taste sensitivity
Supertasters:
25% of the population with heightened sensitivity to taste, especially bitterness
Increased number of fungiform papillae
Recomendações da OMS sobre cuidados maternos e neonatais para uma experiência pós-natal positiva.
Em consonância com os ODS – Objetivos do Desenvolvimento Sustentável e a Estratégia Global para a Saúde das Mulheres, Crianças e Adolescentes, e aplicando uma abordagem baseada nos direitos humanos, os esforços de cuidados pós-natais devem expandir-se para além da cobertura e da simples sobrevivência, de modo a incluir cuidados de qualidade.
Estas diretrizes visam melhorar a qualidade dos cuidados pós-natais essenciais e de rotina prestados às mulheres e aos recém-nascidos, com o objetivo final de melhorar a saúde e o bem-estar materno e neonatal.
Uma “experiência pós-natal positiva” é um resultado importante para todas as mulheres que dão à luz e para os seus recém-nascidos, estabelecendo as bases para a melhoria da saúde e do bem-estar a curto e longo prazo. Uma experiência pós-natal positiva é definida como aquela em que as mulheres, pessoas que gestam, os recém-nascidos, os casais, os pais, os cuidadores e as famílias recebem informação consistente, garantia e apoio de profissionais de saúde motivados; e onde um sistema de saúde flexível e com recursos reconheça as necessidades das mulheres e dos bebês e respeite o seu contexto cultural.
Estas diretrizes consolidadas apresentam algumas recomendações novas e já bem fundamentadas sobre cuidados pós-natais de rotina para mulheres e neonatos que recebem cuidados no pós-parto em unidades de saúde ou na comunidade, independentemente dos recursos disponíveis.
É fornecido um conjunto abrangente de recomendações para cuidados durante o período puerperal, com ênfase nos cuidados essenciais que todas as mulheres e recém-nascidos devem receber, e com a devida atenção à qualidade dos cuidados; isto é, a entrega e a experiência do cuidado recebido. Estas diretrizes atualizam e ampliam as recomendações da OMS de 2014 sobre cuidados pós-natais da mãe e do recém-nascido e complementam as atuais diretrizes da OMS sobre a gestão de complicações pós-natais.
O estabelecimento da amamentação e o manejo das principais intercorrências é contemplada.
Recomendamos muito.
Vamos discutir essas recomendações no nosso curso de pós-graduação em Aleitamento no Instituto Ciclos.
Esta publicação só está disponível em inglês até o momento.
Prof. Marcus Renato de Carvalho
www.agostodourado.com
12. EMBRYOLOGYAND ETIOLOGY
Omphalocele
• during the fourth week of gestation
• differential growth of the embryo
• Causes infolding in the craniocaudal and mediolateral
directions.
• During the sixth week, rapid intestinal and liver growth
leads to herniation of the midgut into the umbilical
cord.
• Elongation and rotation of the midgut occurs
over the ensuing four weeks.
13. …cont
• By week 10, the midgut returns to the abdominal cavity
• the first, second,and third portions of the duodenum and
the ascending and descending colon assume their fixed,
retroperitoneal positions
14. …cont
• The current understanding of the etiology for an
omphalocele
• not from a failure in body wall closure or migration
• Rather, since the umbilical cord is attached to the sac,
• omphalocele develops due to a failure of the viscera to
return to the abdominal cavity
15. …cont
• liver, bladder, stomach, ovary, and testis
can also be found in the omphalocele sac
• The sac consists of the covering layers of the umbilical
cord and includes amnion,Wharton’s jelly, and
peritoneum
• location of the defect is in the mid-abdominal or central
region, but may occur in the epigastric or hypogastric
regions as well
16. EMBRYOLOGYAND ETIOLOGY
(Gastroschisis)
• etiology for gastroschisis is less clear
• >>One theory suggests that gastroschisis results from
failure of the mesoderm to form in the anterior
abdominal wall
• >>Currently(most widely accepted), the ventral body folds
theory
• which suggests failure of migration of the lateral folds
(more frequent on the right side),
18. GASTROSCHISIS
• 1 in 4,000 live births
• mothers younger than 21 years
• Preterm delivery (28% Vs 6%)
• maternal serum α-fetoprotein
(AFP) level (elevated in the presence of gastroschisis)
• ACHE
19.
20. Diagnostic US by 20 wks
• bowel loops freely floating in the amniotic fluid
• defect in the abdominal wall to the right of a normal
umbilical cord
• Intrauterine growth retardation (IUGR)
22. …cont
• Some authors advocate selective preterm delivery
based on the finding of bowel distention and thickening on
prenatal ultrasound
• Bowel dilitation from 7 to 25 mm is associated with fetal
distress and demise
23. …cont
• duration of amniotic fluid exposure is correlated with the
degree of the inflammatory peel and intestinal dysmotility
• bowel atresia is the most common associated
anomaly(6.9–28%)
• cardiac, pulmonary, nervous, musculoskeletal
genitourinary systems, as well as chromosomal
abnormalities
24. Perinatal Care(gastris…)
• both vaginal delivery and C-section are safe
• Preterm delivery is advocated
• dysmotility and malabsorption(Damage to the
pacemaker cells and nerve plexi )
• evidence does not support elective preterm delivery for
gastroschisis
25. Neonatal Resuscitation
and Management
• Appropriate IV access and fluid resuscitation initiated after
birth
• Nasogastric (NG) decompression
• The bowel should be wrapped in warm saline-soaked
gauze and placed in a central position on the abdominal
wall
• positioned on the right side(prevents kinking)
26. ..cont
• The bowel should be wrapped with plastic wrap or the
infant placed partially in a plastic bag
• gastroschisis >>>isolatedanomaly
• intestinal atresia, necrosis, or
perforation>>>complicated
• excess fluid resuscitation >>poor outcome
27. Surgical Management
• goal >>return the viscera to the abdominal cavity
• In minimizing the risk of damage due to
trauma or increased intra-abdominal pressure.
Two most commonly used treatment options
I. silo + serial reductions +delayed closure,
II. primary closure
28. …cont
• N.B>>inspection of the bowel for obstructing bands,
perforation, or atresia>>>> before silo application or
primary closure
29. Primary Closure
• in neonates in whom reduction of the herniated viscera
appears possible>> it has to be done
• Is in the operating room, but some advocates primary
closure at the bedside without general anesthesia
• close the skin only and leave the fascia separated
30. …cont
• Prosthetic options for primary closure
• preservation of the umbilicus has
been shown to lead to an excellent cosmetic
result(against the previous view)
• Intra-abdominal pressure approximated from either the
bladder pressure or stomach pressure
31. …cont
• Pressures >10–15 mmHg >>decreased renal and
intestinal perfusion>> apply silo or patch
• Pressures higher than 20 mmHg can lead to
renal failure and bowel ischemia
• CVP greater than 4 mmHg has been correlated with the
need for silo placement or patch closure
• Splanchnic perfusion pressure at least 44mmHg is
acceptable
32. Staged Closure
• Spring loaded silo>>> made it possible to insert the silo in
the delivery room or at the bedside
33. …cont
• takes 1 to 14 days with the majority being ready within a
week, depending on the condition of the bowel and the
infant
34. …cont
Definitive closure
• Small skin flaps around the fascia
• Closure of the fascia in vertical or horyzontal direction
• Closure of the skin in a transverse direction Vs vertical
direction(keyhole sign)
35. …cont
• purse-string skin closure around the umbilicus
• the umbilical cord is tailored to fill the gastroschisis defect
and is then covered with an adhesive dressing
• Residual ventral hernia rates are reported to be 60–84%
36. Primary vs staged closure
• Avoidance of ischemic injury
• Need for mechanical ventilator
• Early initiation of PO feeding to the foolest
• Oxygen requirement
• Vasopressor requirement
• Effect on UOP
38. Management of Associated
Intestinal Atresia
• Up to 10% of neonates with gastroschisis have an
associated atresia
• jejunal or ileal
• 5% small bowel atresia 5% and a large bowel atresia IS
2%
39. Management of atresia(gastr..)
Options
• Resection and primary anastomosis + primary closure
• Four to six weeks after the primary closure
• Stoma + primary anastomosis
41. Postoperative Course
• abnormal intestinal motility and nutrient absorption,
gradually improve in most patients
• NGT decompression
• Parenteral nutrition
• Enteral feeding started when the bowel functions(wks)
43. Long-Term Outcomes
• Long-term outcomes for patients born with gastroschisis
are generally excellent( except complex disease)
• complex gastroschisis took a median of 21 days longer to
reach full enteral feedings
44. Poor prognostic factors(complex disease)
• 21 days longer to reach full enteral feedings,
• had a longer total parenteral nutrition (TPN)
use
• had almost 2 months longer length of hospitalization
twice as likely to develop intestinal failure
six times more likely to develop liver disease
45. …cont
• Intestinal transplantation(last resort)
• NEC (up to 18.5%)
• Most patients have some degree of intestinal nonrotation
• Cryptorchidism(15–30%)
• If the umbilicus is sacrificed ,up to 60% of children report
psychosocial stress
46. OMPHALOCELE
• Prenatal Diagnosis And Management
• Elevation of maternal serum AFP(not as much in
gastrisc…..)
• Dx by 2D US at 18wk
• Dx by 3D US at 1st TM
• The incidence of omphalocele seen at 14–18 weeks is as
high as 1 in 1,100
• incidence at birth drops to 1 in 4,000–6,000
• Implies the hidden fetal death
47. …cont
• isolated omphalocele has a survival rate of over 90%, but
is reduced with other defects
• only 14% of omphaloceles were truly isolated anomalies
• cardiac (14–47% incidence of anomalies)
• central nervous (3–33% anomalies) systems
48. postnatal morbidity and survival
• ratios between the greatest omphalocele diameter
compared to abdominal circumference (O/AC),
• The femur length (O/FL),
• head circumference (O/HC)
• the most useful may be the O/HC
50. Ix
• echocardiographic evaluation.
• Renal abnormalities can be detected
by abdominal ultrasound.
• Neonatal hypoglycemia (Beckwith–
Weidemann syndrome)
• Blood samples for genetic evaluation should be obtained
as well
53. Surgical Management
• Changing rapidly after 1940s
• defining a giant defect is variable as some
surgeons use size alone
• presence or absence of the liver,
• an estimate of the amount of intestinal contents
• others have used a combination of the amount of liver and
intestine in the sac
54. Immediate Primary Closure
• Treatment options in infants with omphalocele depend
• size of the defect
• the baby’s gestational age
• the presence of associated anomalies
55. …cont
• Less than 1.5 cm in diameter are referred to as hernia of
the cord
• repaired shortly after birth without any issues as long as
there are no associated anomalies
• Defects that are still easy to close without much
loss of abdominal domain can also be closed soon after
birth
56. …cont
• Primary closure consists of excision of the sac and
closure of the fascia and skin over the abdominal contents
• omphalomesenteric duct remnant could be found
associated with a small omphalocele
57. …cont
• When dealing with a medium-sized omphalocele, care
must be taken when excising the portion of the sac
covering the liver,
• because the hepatic veins are located
just under the epithelium/sac interface in the midline and
can be injured
58. …connt
• Closing a giant omphalocele immediately after birth is a
controversial issue despite good outcomes are present
59.
60.
61.
62. Staged Neonatal Closure
• Staged closure in the neonatal period involves the use of
different techniques
• classified into methods that utilize the existing amnion sac
with serial inversion
• sac is excised and replaced with mesh and then closed
over time
63. …cont
• Methods involving primary repair with mesh require
removal of the amnion sac with the mesh used to bridge
the fascial gap followed by skin closure
• Vacuum closure>>novel
64. Delayed Staged Closure
• With this method, the omphalocele sac is excised
• Silastic sheeting is sewn to the rectus fascia.
• Alternatively, the silo can be sewn to the full thickness of
the abdominal wall
65. …CONT
• With this method, the omphalocele sac is excised and the
silastic sheeting is sewn to the rectus fascia
• Alternatively, the silo can be sewn to the full thickness of
the abdominal wall
66.
67.
68. …c ont
• the use of preformed spring-loaded
silos is usually unsuccessful in babies with
omphalocele(EASILY DISPLACED)
69. Scarification Treatment
• Nonoperative techniques >>allows an eschar to develop
over the intact amnion sac
• eschar epithelializes over time, leaving
a ventral hernia that will likely require repair later in life
• defect too large to allow for a safe primary repair, or
if the neonate has significant cardiac or respiratory
issues
71. …cont
• silver sulfadiazine
• povidone-iodine solution
• silver-impregnated dressings
• Neomycin
• polymixin/bacitracin ointments arte used this days
• The eschar and epithelialization may take 4-10 weeks
72. …cont
• eventually require closure of a ventral hernia between 1
and 5 Yrs of age
• By primary fascial closure, autologous repair with
component separation, or mesh repair
• Innoviative methods like use of tissue expanders
73. Postoperative Course
• If primary closure has been accomplished, the majority of
patients will require mechanical ventilation
• Feeding when the bowel is active
• Abcs for 48 hrs
• If a ventral hernia develops, repair may be possible after
age >1yr
74. Primary vs staged closure
• Same stay of hospital
• Primary closure>>>early enteral feeding
• Pressure relaped complications Hepatic
congestion,renal failure,bowel infarction >>are common in
primary closure(12%)
• Skin and fascia dehiscence are common in primary
closure(25%)
75. Giant omphalocele
• 75% or more of the liver in the sac
• Poor prognosis
• More than half of the survivors had associated anomalies,
• more than half had neurodevelopmental disability at 1
year of age
• three fourths had feeding problems
76. Long-Term Outcomes
• gastroesophageal reflux (GERD)(43%)
• pulmonary insufficiency(20%)
• recurrent lung infections or asthma,
• feedingdifficulty with failure to thrive(60% with giant
omphalocele)
77. UMBILICAL HERNIA
• After birth, closure of the umbilical ring is the result of
complex interactions of lateral body wall folding in a
medial direction,
• fusion of the rectus abdominis muscles into the linea alba,
• umbilical orifice contraction which is aided by elastic fibers
from the obliterated umbilical arteries
78. …cont
• Failure of these closure processes results in
umbilical hernia
• The actual fascial defect can range from several
millimeters to 5 cm or more in diameter
• incidence in African-American children from birth to 1-
year-old ranges from 25–58%,
• Caucasian children in the same age group have
an incidence of 2–18.5%
79. treatment
• observe the hernia until ages 3 to 4 years to allow closure
to occur
• spontaneous resolution rates of 83–95% by 6 years of
age
• defects greater than 1.5 cm are unlikely to close
• Incidence of incarceration and strangulation is incidence
of less than 0.2%
81. summary
• Omphalocele and gastrischisis are the commonest
congenital abdominal wall defects
• The diseases do have embryological origin is The current
most accepted theory for omphalocele is failure of the
midgut to return back to the cavity
• And the current accepted theory for occurrence of
gastrischis is a mesothelial failure
• Primary closure or staged closure can be used by using
different criterias for both condition
• Umblical hernias are usually managed conservatively
82. Reference
1. ASCHCRAFT’S PEDIATRIC SURGERY,6TH ED.
2. PEDIATRIC SURGERY(ARNOLD G.CORAN),7TH ED.
3. OPERATIVE PEDIATRIC SURGERY,2ND ED
4. ATLAS OF PEDIATRIC SURGERY,2ND ED
5. UPTODATE 21.2
gastroschisis develops early in gestation and prior to development of an omphalocele
estation and prior todevelopment of an omphalocele. Due to the increasingincidence of gastroschisis, there are a number of possiblecausative factors including tobacco, certain environmental exposures, lower maternal age and low socioeconomicstatus, all suggested by epidemiologic studies, but notproven.1–4
Efforts to reduce this exposure by eitheramniotic fluid exchange or intrauterine furosemide treatment, which induces fetal diuresis
Therefore, thedelivery method should be at the discretion of the obstetrician and the mother, with C-section reserved forobstetric indications or fetal distress.
to limit exposure of the bowel to theamniotic fluid.12 Interleukin-6, interleukin-8, and ferritinare elevated in the amniotic fluid in fetuses with gastroschisis when compared with controlsPreterm delivery is advocated to limit exposure of the bowel to the amniotic fluid
Routine endotrachealintubation is not necessary
After placement, the bowel is reduced daily into the abdominal cavityas the silo is shortened by sequential ligation. When thecontents are entirely reduced, fascial and skin closure areperformed
‘keyhole’ appearance
An intestinal atresia should be differentiated from‘vanishing bowel’ in infants with gastroschisis. This condition is usually associated with a very small abdominalwall defect and is characterized by necrosis and disappearance of some or all of the intestine (Fig. 48-7).Although this is a rare finding, it usually results in shortbowel syndrome.75
cisapride improved contractilityof newborn intestine whereas erythromycin improvedmotility in control adult tissue only.77 However, a randomized controlled trial of erythromycin versus placebofound that enterally administered erythromycin did notimprove time to achieve full enteral feedings
90% survival of gastrischisis
twice as likely to developintestinal failure and six times more likely to develop liverdisease
Beckwith–Weidemann syndrome
macroglossia (large tongue),
macrosomia (above average birth weight and length),
microcephaly
midline abdominal wall defects (omphalocele/exomphalos, umbilical hernia, diastasis recti),
ear creases or ear pits,
neonatal hypoglycemia (low blood sugar after birth).
Hepatoblastom
In a review of treatmentof omphaloceles at one institution, the authors reporteda 12% incidence of complications of increased intraabdominal pressure after closure, including acute hepaticcongestion requiring reoperation, renal failure requiringdialysis, and bowel infarction
Giant omphalocele requires some imagination and creativity to treat.149 Suggestions have included painting thesac with antiseptic,150 the use of skin flaps with grafting tothe open areas remaining,151 use of tissue expander,152 andsplit-thickness skin grafting
43% were found to have GERD by esophageal biopsy or pH monitoring. Patients younger than 2years had an increased rate of reflux compared with thoseolder than two years of age