CLEFT LIP-OTITIS MEDIA

Cleft lip and cleft palate

is a group of conditions that includes cleft lip (CL), cleft palate (CP), and both together (CLP).

Orofacial Cleft

Cleft lip and cleft palate is also known as

first 6 to 8 weeks of pregnancy

the shape of the embryo’s head is formed

Frontonasal Prominence (1)

Maxillar Prominence (2)

Mandibular Prominence (2)

Five primitive tissue lobes grow

Frontonasal Prominence (1)

– from the top of the head down towards the future upper lip

Maxillar Prominence (2)

– from the cheeks, which meet the first lobe to form the upper lip

Mandibular Prominence (2)

– just below, additional lobes grow from each side, which form the chin and lower lip.

Van Der Woude Syndrome

increases the occurrence of these deformities threefold.

Siderius X

- linked intellectual disability in addition to cleft lip or palate, symptoms include facial dysmorphism and mild mental retardation.

Van der Woude syndrome

is a genetic disorder characterized by the combination of lower lip pits, cleft lip with or without cleft palate, and cleft palate only.

Stickler’s Syndrome

can cause cleft lip and palate, joint pain, and myopia.

Loeys-Dietz Syndrome

can cause cleft palate or bifid uvula, hypertelorism, and aortic aneurysm.

Hardikar Syndrome

can cause cleft lip and palate, Hydronephrosis, Intestinal Obstruction, and other symptoms.

Patau Syndrome (trisomy 13).

Cleft lip/palate may be present in many different chromosome disorders including

ultrasonography (UTZ)

Recent advances in prenatal diagnosis have allowed OB to diagnose facial clefts in utero with

Partial or Incomplete Cleft

Formed on the top of the lip as either a small gap or an indentation

Complete Cleft

cleft lip that continues into the nose

Unilateral

Cleft lip that occur as one-sided condition

Bilateral

Cleft lip that occur as two-sided condition

Cleft palate

A condition in which the two plates of the skull that form the hard palate (roof of the mouth) are not completely joined. The soft palate is in these cases cleft as well. In most cases, cleft lip is also present.

Complete

soft and hard palate, possibly including a gap in the jaw

Incomplete

in the roof of the mouth, usually as a cleft soft palate

cleft palate

When this occurs, the uvula is usually split. It occurs due to the failure of fusion of the lateral palatine processes, the nasal septum, or the median palatine processes (formation of the secondary palate).

first 2-3 months after birth

When is surgery performed to close the cleft lip

Rule of 10

While surgery to repair a cleft lip can be performed soon after birth, the surgeons often preferred the

cheiloplasty

Surgery for cleft lip

10 weeks of age

at least 10 pounds

at least 10g hemoglobin

Rule of 10

If the cleft is bilateral and extensive

two surgeries may be required to close the cleft, one side first, and the second side a few weeks later.

Anorectal Malformations (ARM)

are birth defects in which the rectum is malformed

Anorectal Malformations (ARM)

are a spectrum of different congenital anomalies in males and females which vary from fairly minor lesions to complex anomalies.

unknown

Cause of anorectal malformations

In 8% of patients

percentage of genetic factors clearly associated with ARM.

Low Lesion

colon remains close to the skin. In this case, there may be a stenosis (narrowing) of the anus, or the anus may be missing altogether, with the rectum ending in a blind pouch.

High Lesion

– colon is higher up in the pelvis and there is a fistula connecting the rectum and the bladder, urethra, or the vagina.

Persistent Cloaca

(an analogous orifice in reptiles and amphibians), in which the rectum, vagina and urinary tract are joined into a single channel.

-require a protective colostomy

-does not require protective colostomy

two main categories of ARM

first 24 hours of birth

The decision to open a colostomy is usually taken within

UTZ

can be used to determine the type of imperforate anus.

fistula

Imperforate anus usually requires immediate surgery to open a passage for feces unless a _______ can be relied on until corrective surgery takes place.

perineal anoplasty or colostomy

Depending on the severity of the imperforated anus, it is treated either with a

Spina Bifida

A birth defect where there is incomplete closing of backbone and membranes around the spinal cord.

The most common location is the lower back, but in rare cases it may be the middle back or neck.

Spina Bifida Occulta

Has no or only mild signs.

• May include hairy patch, dimple, dark spot, or swelling on back at the site of the gap in the spin

Spina Bifida Cystica

the protruding sac can contain meninges (meningocele), spinal cord (myelocele), or both (myelomeningocele).

Meningocele

typically causes mild problems with a sac of fluid present at the gap in the spine.

Myelomeningocele

also known as open spina bifida, is the most severe form.

Learning problems are relatively uncommon

Folic Acid deficiency

during pregnancy also plays a significant role in occurrence of Spina Bifida

12-18 months

When is palatoplasty performed

Posterior sagittal approach

incision down the midline of buttocks to repair defects in anus and rectum

latex

68% of children with spina bifida have an allergy to ______, ranging from mild to life-threatening.

HYDROCEPHALUS

A condition in which accumulation of Cerebrospinal Fluid (CSF) occurs within the brain.

Treatment for spina bifida

surgery by a pediatric neurosurgeon to close the opening on the back after delivery. This surgery aims to prevent further damage of the nervous tissue and to prevent infection.

shunt

may be surgically installed to provide continuous drain for the excess CSF produced in the brain, into the abdomen or chest wall, as happens with hydrocephalus.

Fetoscopy

These techniques may be an option to standard therapy.

Open Fetal Surgery

Risk: Mother - scarring of the uterus, Fetus - risk of preterm birth.

Communicating

Also known as Non-Obstructive Hydrocephalus, is caused by impaired CSF reabsorption in the absence of any CSF-flow obstruction between the ventricles and subarachnoid space.

Non-communicating

Also known as Obstructive Hydrocephalus, is caused by a CSF-flow obstruction.

• Both forms can be either congenital or acquired.

infants with hydrocephalus

CSF builds up in the CNS, causing the fontanelle to bulge and the

head to be larger than expected.

Hydrocephalus

Can be successfully treated by placing a drainage tube (shunt) between the brain ventricles and abdominal cavity. Some risk exists of infection being introduced into the brain through these

, however, the shunts must be replaced as the person grows.

shunt

Treatment for hydrocephalus

External Ventricular Drain

also known as Extra-Ventricular Drain or Ventriculostomy,

External Ventricular Drain

provides relief. It involves the placement of a ventricular catheter into the cerebral ventricles to bypass the flow obstruction and drain the excess fluid into other body cavities, from where it can be resorbed.

Ventriculoperitoneal Shunt

drain fluid into the peritoneal cavity (most)

Ventricuatrial Shunt

– drain fluid into the right atrium

Ventriculopleural Shunt

– drain fluid into the pleural cavity and gall bladder.

Lumbar-Peritoneal Shunt

, placed in the lumbar space of the spine and have the CSF redirected to the peritoneal cavity.

Endoscopic Third Ventriculostomy (ETV).

, an alternative treatment for obstructive hydrocephalus in selected people, a surgical opening in the floor of the third ventricle allows the CSF to flow directly to the basal boilers, thereby shortcutting any obstruction

OTITIS MEDIA

A group of inflammatory diseases of the middle ear.

Eustachian Tube Dysfunction

-The common cause of all forms of otitis media.

Eustachian tube dysfunction

the gas volume in the middle ear is trapped and parts of it are slowly absorbed by the surrounding tissues, leading to negative pressure in the middle ear.

Acute Otitis Media (AOM)

An infection of rapid onset that usually presents with ear pain.

• In young children this may result in pulling at the ear, increased crying, and poor sleep.

• - Decreased eating and a fever may also be present.

Otitis Media with Effusion (OME)

- Also known as Serous Otitis Media (SOM) or Secretory Otitis Media (SOM), and colloquially referred to as Glue Ear

Otitis Media with Effusion (OME)

Fluid accumulation that can occur in the middle ear and mastoid air cells due to negative pressure produced by dysfunction of the Eustachian tube.

• - Typically not associated with symptoms.

• - Occasionally a feeling of fullness is described.

• - Presence of non-infectious fluid in the middle ear for more than three months.

Chronic Suppurative Otitis Media (CSOM)

Middle ear inflammation of greater than 2 weeks that results in episodes of ear discharge for at least 6 weeks.

• It may be a complication of acute otitis media.

• Pain is rarely present.

Adhesive Otitis Media

Occurs when a thin retracted ear drum becomes sucked into the middle-ear space and stuck to the ossicles and other bones of the middle ear.

Antibiotics

A treatment option for CSOM with discharge is topical antibiotics.

Tympanostomy Tube. ":Grommets"

are recommended with 3 or more episodes of AOM in 6 months, or 4 or more in a year, with at least 1 episode or more attacks in preceding 6 months.

Otitis Media with Effusion

Decision to treat is usually made after a combination of physical

exam and laboratory diagnosis (audiometry, tympanogram, temporal bone CT scan and MRI).

tympanostomy tubes

In more severe cases of otitis media, __________ can be inserted

Membrane Rupture

– In severe / untreated cases, tympanic membrane may perforate, allowing pus in the middle-ear space to drain into the ear canal.

Tympanocentesis

– Needle aspiration through tympanic membrane to relieve ear pain and to identify causative organism(s). An option for severe AOM in which analgesics are not controlling ear pain.

Hearing Loss

– Children with recurrent episodes of AOM and those with OME or CSOM have higher risks of developing