Umbilical Hernia

Abdominal Wall Defects

Umbilical Hernia

• Part of the intestine protrudes through an opening in the abdominal muscles around the umbilical cord
• Creates a bulging protrusion under the skin at the umbilicus
• More common in girls than boys
• Facial ring less than 2 cm usually closes spontaneously
• Surgery is required if more than 2 cm to prevent herniation and intestinal obstruction
• Occurs when protruding abdominal tissue becomes trapped and can't be pushed back
• Reduced blood supply to the trapped intestine can cause umbilical pain and tissue damage
• If the trapped intestine is entirely cut off, tissue death (gangrene) may occur
• Infection can spread throughout the abdominal cavity and cause life-threatening situations
• Symptom is a soft bulge or swelling near the navel, from 1 to 5 cm in diameter
• Evident when the infant cries, coughs, or strains causing the bellybutton to protrude
• Usually painless in children
• Umbilical hernias appearing in adulthood can cause abdominal discomfort
• Diagnostic exam includes a physical exam and imaging studies like abdominal ultrasound or X-ray
• Doctor may push the bulge back during a physical exam
• Surgical management involves a small incision under the belly button to close the hernia with sutures
• After the operation, there are no stitches to remove because they dissolve on their own
• Incision is covered with small bandages called Steri-Strips
• Possibility of the hernia returning is very small

Omphalocele

• Protrusion of stomach contents between the junction of the abdominal wall and the umbilicus
• Umbilical Cord Hernia has a defect less than 4 cm
• True Omphalocele has a defect greater than 10 cm
• Occurs because between the 6th and 8th week of intrauterine life, the fetal abdominal contents are extruded
• At 7 to 10 weeks, the intestine returns to the abdomen as it enlarges
• Omphalocele occurs when the abdominal contents fail to return
• The occurrence is associated with chromosomal aberrations
• Assessments include prenatal sonogram, elevated MSAFP, and obvious inspection at birth
• Management includes small surgery within 24 hours
• Large surgical options include Prosthetic Patch Repair & Partial replacement with "Silo pouch"
• Apply a wet dressing
• Can use Total parenteral nutrition (TPN)

Gastroschisis

• A birth defect where an infant is born with some or all intestines outside the abdomen
• High-risk factors are young mothers who use recreational drugs or smoke early in pregnancy
• Cause is unknown, but there is evidence it could be inherited in an autosomal recessive pattern
• Symptoms include a 5 cm vertical opening in the abdominal wall to the side of the umbilical cord
• Often part of the small intestine sticks out through the opening
• In some, the large intestine and other organs may also come through the opening
• Infants frequently have a low birth weight or are born prematurely
• Can have other birth defects like underdeveloped bowels, or genetic disorders
• Diagnosis involves testing the pregnant mother's blood for elevated levels of AFP
• Managed surgically by stretching and placing intestinal contents back inside
• Surgery is postponed if intestines are swollen; intestines covered with a special pouch ("Silo") until swelling subsides

Intestinal Obstruction

• Canalization of the intestine doesn’t occur in utero
• Atresia refers to a complete closure
• Stenosis refers to a narrowing of the fetal bowel
• The Duodenum is the most common site
• Anticipate if there is hydramnios in the mother
• Aspiration of more than 30 ml of Newborn's stomach contents
• Absence of meconium passage, abdominal distention & tenderness
• Vomiting (sour-smelling; bile stained; dark) and increased bowel sounds
• Peristaltic waves across the abdomen, accompanied by pain and increased RR
• Sonogram reveals no air below level of obstruction
• Management: Orogastric or NGT/Suctioning/IVF
• Immediate laparoscopic or Intestinal incision surgery
• Temporary colostomy with surgery rescheduled at 3-6 months

Meconium Plug Syndrome

• An extremely hard portion of meconium has completely blocked the intestinal lumen
• Cause is unknown, probably reflects normal variations of meconium consistency
• May be associated with Hirschsprung's disease and is strongly associated with cystic fibrosis
• Abdominal distention and vomiting occur more than 24 hours from birth
• No meconium passage in past 24 hours from birth
• Radiograph or Sonogram - reveals distended air-filled loops of bowel up to the point of obstruction
• A slightly hypertonic water-soluble contrast agent enema may reveal the level of obstruction
• Management includes Saline enemas/(Mucomyst) with diatrizoate (Hypaque) rectally/Gastrografin as an enema
• Hydrate well the infant before & after the procedure or they can become hypovolemic from the contrast medium
• Assess family history for cystic fibrosis, Aganglionic magacolon (Hirschsprungs's disease), hypothyridism
• Obtain phenylketonuria screening

Meconium Ileus

• Obstruction of the intestinal lumen by hardened meconium
• A specific phenomenon that occurs almost exclusively in infants with cystic fibrosis
• Reflects extreme meconium plugging

Diaphragmatic Hernia

• Protrusion of an abdominal organ (stomach or intestine) through a defect in the diaphragm into the chest cavity
• Signs and symptoms include Sunken abdomen
• Signs of RDS (cyanosis, intercostal or subcostal retractions Absent breath sounds on the affected side
• Right-to-left shunting
• Management includes Diaphragmatic repair within 24 hours
• Surgical repair is either laparoscopic or thoracic incision

Anorectal Malformations

Imperforate Anus

• Stricture of the anus or the absence of normal anal opening
• More common in boys than in girls
• Assessment involves a prenatal sonogram
• Shortly after birth Dx. is made by a routine physical examination
• Black meconium-filled membrane protruding from anus (occasional)
• Absence of meconium passage (within 24-48 hours)
• Radiograph or sonogram to estimate the distance the intestine is separated from the perineum
• U/A - for presence of meconium
• Management involves Surgical repair
• Step 1: Nursery-Colostomy (palliative) before discharge from Nursery
• Step 2: At 10 months old- Corrective Surgery (Anorectal malformation repair/Perineal Anoplasty/Pull-Through procedure)
• Step 3: After 6 months-Closure of the Colostomy

Persistent Cloaca

• A complex anorectal malformation where the rectum, vagina, & urethra drain into a common channel
• The common channel opens onto the perineum via the usual urethral site.

Cloacal Exstrophy

• A rare, severe defect where there is externalization of the bladder and bowel through the abdominal wall.

Hydrocephalus

• An excess of CSF in the ventricles or the subarachnoid space
• Excess CSF occurs for one of 3 main reasons:
• Overproduction of fluid by a choroid plexus in the 1st or 2nd ventricle, could occur from a growing tumor (rare)
• Obstruction of the passage of fluid in the narrow aqueduct of Sylvius (the most common cause)
• Interference with the absorption of CSF from the sub- arachnoid space if a portion of the subarachnoid membrane is removed
• Assessments involve a prenatal sonogram
• Recording & plotting head circumference for children, ultrasound, Computed tomography (CT), or magnetic resonance imaging (MRI)
• Skull x-ray film reveal that sutures are separated & thinning of the skull
• Transillumination
• Management includes Osmotic Diuretic/Mannitol/Diamox / acetazolamide
• Seizure precautions and Destruction of portion of choroid plexus or tumor removal
• Surgery: Shunting - AV Shunt - atrioventricular
• VP shunt -ventriculoperitonial (most common)

Neural Tube Disorders

Anencephaly

• Absence of the cerebral hemispheres
• Occurs when the upper end of the neural tube fails to close in early intrauterine life
• Infants may have difficulty in labor; usually in breech position
• Obvious on visual inspection at birth
• Cannot survive, so if discovered prenatally, offer abortion option

Microcephaly

• A disorder where brain growth is so slow that it falls more than three standard deviations below normal charts
• Cognitively challenged due to abnormal development.

Spina Bifida Occulta

• Occurs when the posterior laminae of the vertebrae fail to fuse
• Noticeable as a dimpling at the point of poor fusion as well as abnormal tufts of hair or discolored skin

Meningocele

• Protrusion of CSF and Meninges
• Generally occurs in the lumbar region or occurs anywhere along the spinal canal

Meningomyelocele

• The spinal cord & the meninges protrude through the vertebrae
• The Child will have flaccidity & lack of sensation of the lower extremities and loss of bowel and bladder control.

Enphalocele

• A cranial meningocele or myelomeningocele
• Generally are covered fully by skin, but they may be open or covered only by the dura
• Diagnosis: Transillumination, CT scan, UTZ

Anencephaly Assessment

• Prenatal UTZ, Fetoscopy/Amniocentesis or analysis of AFP in maternal serum
• Assess nature & pattern of voiding & defecation
• UTZ, MRI to differentiate meningocele & meningomyelocele
• Spina bifida occulta requires no immediate surgical correction
• Meningocele, myelomeningocele, or encephalocele require immediate surgery to replace replaceable content
• Emotional support to parents is important.

Arnold-Chiari

• Malformation is a neural tube defect caused by overgrowth of the neural tube from weeks 16-20 of fetal life