09 March 2010

Surgical Emergencies in the Newborn



Surgical Emergencies in the Newborn
University of North Carolina at Chapel Hill
Pediatric Surgery Division
Patty Lange

Emergencies
* Types
o Airway/Respiratory
o Intestinal Obstruction
o Intestinal Perforation
* Signs
o Respiratory distress
o Abdominal distension
o Peritonitis
o Pneumoperitoneum

Airway/Respiratory
* Neck Masses
* Thoracic masses/pulmonary lesions

Cystic Hygroma
* Multiloculated cystic spaces lined by endothelial cells
* Incidence about 1 in 12,000 births
* Complications
* Treatment
* Postnatal overdistension of one or more lobes of histologically normal lung
* Location

Congenital Lobar Emphysema
* Diagnosis
* Treatment

Congenital Cystic Adenomatous Malformation (CCAM)
* Mass of cysts lined by ciliated cuboidal or columnar pseudostratified epithelium
* Three types
* More common on the left side, 2% bilateral

CCAM
* Diagnosis
* Treatment
Congenital Diaphragmatic Hernia
* Intro
* DX
* Treatment
Tracheoesophageal Fistula and Esophageal Atresia
Intestinal Obstruction
Anatomic Differentiation
* Upper GI
* Lower GI
Anatomic Differentiation
Urgency to Treat
* Emergencies
* Further workup
Common Disorders
* NEC
* Duodenal Atresia
* Small Bowel Atresia
* Malrotation/Volvulus
* Hirschsprung’s
* Presentation

NEC Treatment
* Medical
* Surgical indications
NEC Outcomes
* Overall survival ~ 80%, improving in LBW
* In pts w/perforation, 65% perioperative mortality, no perf--30% mortality
* 25% of Survivors develop stricture
* 6% pts have recurrent NEC
* Postop NEC--Myelomeningocele, Gastroschisis--45-65% mortality

Pneumatosis
Pneumoperitoneum
NEC--Abd Distension/Erythema
Necrotic Segment Ileum
Resection
Specimen--Ileocecectomy
Ileostomy
Common Disorders
* NEC
* Duodenal Atresia
* Small Bowel Atresia
* Malrotation
* Hirschsprung’s

Duodenal Atresia
* Incidence--1 in 5,000 to 10,000 live births
* 75% of stenoses and 40% of atresias are found in Duodenum
* Multiple atresias in 15% of cases
* 50% pts are LBW and premature
* Polyhydramnios in 75%
* Bilious emesis usually present
* Associated Anomalies

Duodenal Atresia Diagnosis
* Radiographs
* Workup of potential associated anomalies
“Double Bubble”
Duodenal Atresia Treatment
* Nasogastric decompression, hydration
* Surgery
Common Disorders
* NEC
* Duodenal Atresia
* Small Bowel Atresia
* Malrotation
* Hirschsprung’s

Small Bowel Atresia
* Jejunal is most common, about 1 per 2,000 live births
* Atresia due to in-utero occlusion of all or part of the blood supply to the bowel
* Classification--Types I-IV
* Presents w/bilious emesis, abd distension, failure to pass meconium (70%)

Intestinal Atresia Classification
* Associated Anomalies
o other atresias
o Hirschsprung’s
o Biliary atresia
o polysplenia syndrome (situs inversus, cardiac anomalies, atresias)
o CF (10%)

Atresia--Diagnosis and Treatment
* Plain films show dilated loops small bowel
* Contrast enema shows small unused colon
* UGI/SBFT shows failure of contrast to pass beyond atretic point
* Treatment is surgical

Common Disorders
* NEC
* Duodenal Atresia
* Small Bowel Atresia
* Malrotation/Volvulus
* Hirschsprung’s

Malrotation
* 1 per 6,000 live births
* can be asymptomatic throughout life
* Usually presents in first 6 months of life
* 18% children w/short gut had malrotation with volvulus
* Etiology
o physiologic umbilical hernia--4th wk gestation
o Reduction of hernia 10th - 12th wks of gestation

Normal Embryology
Malrotation Classification
* Nonrotation
* Abn Rotation of Duodenojejunal limb
* Abn rotation of Cecocolic limb

Abnormal Rotation/Fixation
Malrotation Diagnosis
* Varying symptoms from very mild to catastrophic
* **Bilious emesis is Volvulus until proven otherwise**
* Bilious emesis, bloody diarrhea, abd distension, lethargy, shock
* UGI shows abnormal position of Duodenum
o if Volvulus, see “bird’s beak” in duodenum

Malrotation UGI
Intraop Volvulus
Bowel Necrosis--Volvulus
Malrotation--Treatment
* Surgical--Ladd’s Procedure
Common Disorders
Hirschsprung’s Disease
* Migratory failure of neural crest cells
* Incidence 1 in 5,000 live births, males affected 4:1 over females
* 90% of pts w/H’sprung’s fail to pass meconium in first 24-48 hrs
* Abd distension, bilious emesis, obstructive enterocolitis
Hirschsprung’s Diagnosis
* Barium Enema
* Anorectal Manometry
* Rectal Biopsy

Transition Zone on BE
Hirschsprung’s Treatment
Pull-Through Procedure
Summary
* BILIOUS EMESIS IS VOLVULUS UNTIL PROVEN OTHERWISE
* Signs of surgical emergency
o free air, abd wall cellulitis, fixed loop on xray, rapid distension, peritonitis, clinical deterioration
* History and plain films will guide sequence of additional studies
* Remember associated anomalies

Surgical Emergencies in the Newborn.ppt

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Pediatric Minimally Invasive Surgery



Pediatric Minimally Invasive Surgery
By:Joseph A. Iocono, M.D.
Assistant Professor
Division of Pediatric Surgery
University of Kentucky
Children’s Hospital

Large Operations with Tiny Incisions
Lap Hirschsprung’s pull through 8 weeks post-op pull through

MIS-Advantages
* Cosmesis
+ open operations often leave large, unsightly incisions
+ with some laparoscopic instruments smaller than 2mm in size, it is often difficult to see incisions postoperatively
* Analgesia
o Smaller incisions associated with less pain, lower analgesic use, and quicker recovery.
+ few controlled studies in children, especially in youngest patients
* Adhesions
o several studies suggest the formation of fewer intra-abdominal adhesions after laparoscopic procedures
+ reduces the risk of future postoperative bowel obstructions
+ possibly reduces postoperative pain
* Decreased Ileus
+ Nissen, Appendectomy, Pyloromyotomy, Bowel resection, Spleen
+ Real or perceived?

Pediatric Surgery and MIS
Pediatric Surgeons—already “in the business”
o Small incisions--small scars
o Preemptive anesthesia--decreased pain med needs
o Short hospital stays
o Laparoscopic Cholecystectomy
o Laparoscopic Pyloromyotomy
o First true pediatric MIS procedure
o Laparoscopic appendectomy
o Laparoscopic Nissen Fundoplication
o Laparoscopic Splenectomy

MIS—What’s So Great?
* Why Bother?
o Additional expense
o Prolonged procedures
o Lack of tactile evaluation
o Loss of depth perception
o Complications specific to MIS
“After an advanced MIS case, the patient goes home and the surgeon goes to the ICU”
* Expense
* Length of Procedures
From Curiosity to Standard of Care—How?
o Procedure Driven
o Patient (parent) Driven
o Technology Driven
o Physician Driven
o Care Driven --“re-think” care

Technology – Smaller and Better
Ligation Monopolar Bipolar, harmonic
Instruments 10 mm 3 mm (disposable) (reusable)

Technology
MIS – Indications
* General Indications
* New procedures Developed rapidly
Partial list of described MIS procedures in Children
Achalasia (1) Adhesive Small Bowel Obstruction
Adrenal Tumors (1) Appendicitis (25)
Biliary Atresia Cholelithiasis (5)
Chronic Abdominal Pain (2) Chronic Constipation (ACE procedure) (5)
Crohn’s Disease (2) Diaphragmatic Hernia (1)
Duodenal Atresia Empyema
Gastroesophageal Reflux (25) Gastrostomy Tube Placement (20)
Hirschsprung’s Disease (2) Benign Kidney Disease
Lung tumor (4) Malrotation (1)
Meckel’s Diverticulum Mediastinal Pathology (1)
Ovarian Torsion and Cysts (2) Pancreatic Pseudocyst
Pectus Excavatum (4) Placement of VP Shunt
Pyloromyotomy (32) Recurrent Pneumothorax (1)
Splenic Pathology (5) Tracheoesophageal Fistula
Undescended Testicle (6) Ulcerative Colitis (1)
Urinary Reflux Inguinal Hernia (recurrent) (1)
Patent ductus arteriosus Peritoneal Dialysis access

MIS in Pediatric Surgery
* Indications
* Procedure
* Complications
* Changes in Care
* Controversies
* Cholecystectomy
* Nissen Fundoplication
* Appendectomy
* Splenectomy
* Intestinal Resection
* VATS
* Inguinal Hernias
* Pyloromyotomy
* Hirschsprung’s Pull Through
* Ladd’s Procedure
* Pectus Excavatum- Nuss Procedure
* Congenital Diaphragmatic Hernia

Cholecystectomy--1991

* Indications
* Procedure
* Complications
* Changes to Care
* Controversies
* Gold Standard
* Complications
* Changes to Care
* Controversies

Port size/use
1. 5mm--camera
2. 3mm--liver retractor
3. 5mm--dissection (G-tube)
4. 3mm--dissection
5. 3mm---retraction (optional)

Appendectomy--1992
* Indications
* Procedure
* Complications
* Changes to Care
* Controversies

Splenectomy--1998
* Indications
* Procedure
* Complications
* Changes to Care
* Controversies

Intestinal Resection
* Indications
* Procedure- 2 ways
* Complications
* Changes to Care
* Controversies
Optional Incisions
Thoracoscopy-VATS
* Indications
o Empyema Blebs
o Wedge Biopsy Anterior Spine
o Mediastinal cysts Thymectomy
* Procedure
o 3 ports, low pressure CO2
* Complications
o Conversion rate high
* Changes to Care
o Insufflation better
o Faster recovery
o Start chemo earlier
* Controversies
o Ability to “feel’ lung.

Inguinal Hernias
* Indications
* Procedure
* Complications
* Changes to Care
* Controversies

Pyloromyotomy-1991
* Indications
* Procedure
* Complications
* Changes to Care
* Controversies

Pull-through for Hirschsprung’s--1995
* Indications
* Procedure
* Complications
* Changes to Care
* Controversies

Ladd’s Procedure for Malrotation--1997
* Indications
* Procedure
* Complications
* Changes to Care
* Controversies

Nuss Procedure for Pectus Excavatum --1995
* Indications
* Procedure
* Complications
* Changes to Care
* Controversies
o Need for scope?

Diaphragmatic Hernia
* Indications
* Procedure
* Complications
* Changes to Care
* Controversies

Pediatric Minimally Invasive Surgery
* Conclusions
o Surgeon must decide whether a minimally invasive approach is the safest and most appropriate procedure.
o Must convert to an open procedure at any time that the risks are greater than those of the open technique.
o Must increase his/her repertoire of MIS cases as skills improve.
o Must stay informed about new techniques, tools, and indications and complete CME in order to gain needed training.

Teaching Minimally Invasive Surgery
* Education
* Solution--basic skills need to be mastered

Who gets MIS Procedures and
When do I refer to Pediatric Surgery?
* Who?
* When?
* How?

Future Directions
* Limitations of current MIS technology
* Solution---daVinci operative system
* Ready for Pediatric MIS? Yes Infant MIS? Not quite

Final Thoughts
“Five years ago it would have been unthinkable that an [entire] issue of Seminars in Pediatric Surgery would be discussing intracorporeal anastomoses after intestinal resections and laparoscopic pull-through for high imperforate anus. Yes it is likely that we are only in the infancy of the development of laparoscopic surgery in our patients…Several pediatric surgeons are involved with experimentation and development with robotic surgery…Certainly, it will make intestinal anastomoses easier and make [more complicated] procedures such as portoenterostomy [Kasai procedure] more feasible.”

Pediatric Minimally Invasive Surgery.ppt

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The Liver and the Biliary Tract



The Liver and the Biliary Tract
By:Brando Cobanov, M.D.
Department of Pathology
UMDNJ-RWJMS

Hepatic Injury
* Inflammation = hepatitis
o Portal tracts, lobules
* Degeneration
o Damage from toxic or immunologic insult
o Accumulation of substances, e.g., steatosis
* Cell death
o Centrilobular, submassive, massive necrosis
* Fibrosis
o Usually irreversible
* Cirrhosis

Bile
Jaundice
* Excessive production of bilirubin
o Hemolytic anemias, ineffective erythropoiesis
* Reduced hepatic uptake
* Impaired conjugation
o Physiologic jaundice of the newborn
o Crigler-Najjar syndromes types I and II
o Gilbert syndrome
o Viral or drug-induced hepatitis, cirrhosis
* Decreased hepatocellular excretion
o Dubin-Johnson syndrome, Rotor syndrome
* Impaired bile flow

Cholestasis
* Systemic retention of not only bilirubin but also other solutes eliminated in bile, particularly bile salts and cholesterol
* Due to hepatocellular dysfunction or biliary obstruction
* Accumulation of bile pigment within the hepatic parenchyma – Kupffer cells
* Bile ductular proliferation
* Bile lakes
* Portal tract fibrosis

Hepatic Failure
Clinical Features
* Jaundice
* Hypoalbuminemia
* Hyperammonemia
* Fetor hepaticus
* Palmar erythema
* Spider angiomas
* Hypogonadism
* Gynecomastia

Complications
* Multiple organ failure
* Coagulopathy
* Hepatic encephalopathy
* Hepatorenal syndrome

Cirrhosis
* Bridging fibrous septa
* Parenchymal nodules
* Disruption of the architecture of the entire liver
* Etiologies

Portal Hypertension
* Prehepatic
* Intrahepatic
* Posthepatic

Clinical Sequelae
* Ascites
* Portosystemic venous shunts
* Splenomegaly
* Hepatic encephalopathy

Drug Induced Liver Disease
* Liver is the major drug metabolizing and detoxifying organ in the body
* Direct toxicity
* Hepatic conversion of a xenobiotic to an active toxin
* Immune mechanisms
* Table 16-6

Alcoholic Liver Disease
* Hepatic steatosis
* Alcoholic hepatitis
* Alcoholic cirrhosis
o Micronodular

Pathogenesis
* Shunting of normal substrates away from catabolism toward lipid biosynthesis
* Induction of cytochrome P-450
* Free radicals generated by microsomal ethanol oxidizing system
* Alcohol directly affects microtubular and mitochondrial function
* Acetaldehyde induces lipid peroxidation
* Neutrophil infiltration
* Immunologic attack of hepatocytes

Causes of Death
* Hepatic failure
* Massive GI hemorrhage
* Infection
* Hepatorenal syndrome
* Hepatocellular carcinoma

Nonalcoholic Fatty Liver
* Elevated serum aminotransferase levels
* Low risk for development of hepatic fibrosis or cirrhosis
* Associated with obesity, type 2 DM, hyperlipidemia
* Need to exclude other causes


Hemochromatosis
* Primary or hereditary
* Secondary
Pathogenesis
Morphology
Clinical Features
Wilson Disease
Morphology
Clinical Features
α1-Antitrypsin Deficiency
Morphology
Neonatal Hepatitis
Reye Syndrome
Morphology
Obstructive Biliary Tract Disease
Secondary Biliary Cirrhosis
Primary Biliary Cirrhosis
Primary Sclerosing Cholangitis
Circulatory Disorders
Hepatic Artery Inflow
Portal Vein Obstruction
Impaired Blood Flow Through the Liver
Hepatic Vein Thrombosis
Veno-Occlusive Disease
Hepatic Neoplasms
Benign Tumors
Focal Nodular Hyperplasia
Liver Cell Adenoma
Hepatocellular Carcinoma
Pathogenesis
Morphology
HCC
Clinical Features
Disorders of the Gallbladder
Cholelithiasis
Clinical Features
Cholecystitis
Choledocholithiasis
Cholangitis
Biliary Atresia
Gallbladder Carcinoma
Cholangiocarcinoma

The Liver and the Biliary Tract.ppt

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