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SURGICAL CASE PRESENTATION

PRABHJOT S PARDESI

Doc, the babys intestines are all over the place....

General Data:
A case of S.F. , newborn, Filipino, RC, from Talisay City, was referred to Surgery from Pediatrics Dept, CCMC, for management.

HPI:
Few hours prior to admission, patient was born via NSVD, to a 30y.o. female, AOG 362/7 wks, BW: 2800g, AS 8.9 ; B.S : 36, with good cry, AGA. Patient was noted to have her intestines exposed completely through the Umbilical cord. Patient was immedialtely referred to Surgery for evaluation & further management.

Antenatal Course:
- Patient is 3rd birth in the family. - The mother claims to have regular prenatal visits at Maternity Center, Talisay. - The mother took Multivitamins and FeS04 with good compliance. - Ultrasound was done at 7th month of pregnancy, which revealed abnormal anterior wall of the fetus.

Antenatal Course:
- Patient sought consult at private physician. Was advised to proceed with Normal Pregnancy and follow-up after the birth of fetus.

Post-natal Course:
- Patient has a good cry, feeds well on Breast milk. - Vit K & Hep B was injected at the time of birth. - Patient is Appropriate for Gestational Age.

Physical Examination:
Patient examined awake, Not in Respiratory Distress, afebrile, with good cry, with the ff v/s: PR: 130 cpm RR: 56 bpm Temp: 36.6C Weight: 2800 gms.

Physical Examination:
- Skin : warm, good turgor. - HEENT : Anicteric Sclerae, Pink Palpebral Conjuctivae - CVS : Normal Rate & Regular Rhythm Distinct Heart Sounds, (-) Murmurs (-) Rubs - C/L : Equal Chest Expansion, Clear Breath Sounds, (-) Rales, (-) Wheeze

Physical Examination:
- Abd : soft, (+) ant wall defect (3x3 cm) with complete small bowel evisceration. - Ext Gen: grossly female. - Ext. : (-) deformities, strong pulses. CRT< 2 sec. Neurologic Exam : Within Normal Limits.

Admitting Impression:

GASTROSCHISIS

GASTROSCHISIS
A

congenital anomaly characterized by a defect in the anterior abdominal wall through which the intestinal contents protrude freely.
There Size

is NO overlying sac.

of the defect is much smaller (<4 cm) unlike with omphalocele.

GASTROSCHISIS
The

abdominal wall defect is located at: - the junction of umbilicus and normal skin - almost always to right of umbilicus.

The

umbilicus becomes partly detached, which allows free communication with the abdominal cavity.

GASTROSCHISIS
The intestine may be normal in appearance, which suggests that the rupture occurred relatively late during the pregnancy.

Mostly, the intestine is thick, edematous, discolored, and covered with exudates, which implies a more longstanding process.

GASTROSCHISIS
The associated anomalies consist mostly of Intestinal Atresia.

This defect can readily be diagnosed on prenatal ultrasonography

There is no advantage to performing a cesarian section rather than a vaginal delivery.

GASTROSCHISIS
The delayed onset of intestinal function in children with gastroschisis has led some to postulate an injurious effect of amniotic fluid on the exposed bowel.

This has led to consideration in some centers of early delivery to minimize intestinal damage and improve outcome. .

GASTROSCHISIS - Management
All infants born with Gastroschisis require urgent surgical treatment.

Of equal importance, these infants require vigorous fluid resuscitation on the order of 160 to 190 mL/kg per day to replace significant evaporative fluid losses.

In many instances, a primary surgical closure of the abdominal wall is performed & the intestine can be returned to the abdominal cavity.

GASTROSCHISIS - Management
Techniques that facilitate primary closure include: mechanical stretching of the abdominal wall thorough orogastric suctioning with foregut decompression and rectal irrigation and evacuation of all the meconium.

GASTROSCHISIS - Management
Care must be taken to prevent increased abdominal pressure during the reduction, which would lead to compression of the inferior vena cava and respiratory embarrassment, and result in abdominal compartment syndrome.

To avoid this complication, it is helpful to monitor the bladder or airway pressure during reduction.

GASTROSCHISIS - Management
In infants whose intestine has become thickened and edematous, it may be impossible to reduce the bowel into the peritoneal cavity in the immediately postnatal period.

Under such circumstances, a plastic springloaded silo can be placed onto the bowel and secured beneath the fascia.

The Silo covers the bowel and allows for graduated reduction on a daily basis as the edema in the bowel wall decreases.

A piece of prosthetic material may be required to bring the edges of the fascia together.

Surgical closure can be accomplished within approx. 1 week.

If Atresia is noted at the time of closure, it is prudent to reduce the bowel at the first operation, then return after several weeks once the edema has resolved to correct the atresia.

Intestinal function does not typically return for several weeks in patients with gastroschisis (in pts where bowel is thickened and edematous).

As a result, these patients require central line placement and institution of TPN to grow.

OMPHALOCELE

A congenital defect of the abdominal wall in which the bowel and solid viscera are covered by peritoneum and amniotic membrane.

Herniation or protrusion of the abdominal contents into the base of the umbilical cord. In contrast to umbilical hernia, the sac is covered with peritoneum without overlying skin.

The umbilical cord inserts into the sac. The abdominal wall defect measures 4 cm in diameter.

OMPHALOCELE
It occurs in association with special syndromes such as: Exstrophy of cloaca (vesicointestinal fissure) the Beckwith-Wiedemann constellation of anomalies (macroglossia, macrosomia, hypoglycemia, visceromegaly and omphalocele) the CANTRELL PENTALOGY which include lower thoracic wall malformations such as: (cleft sternum, ectopia cordis, epigastric omphalocele, anterior midline diaphragmatic hernia, cardiac anomalies).

OMPHALOCELE
The defect may be very small or large enough that it contains most of the abdominal viscera.

There is a 60 to 70% incidence of associated anomalies, especially cardiac anomalies (20 to 40% of cases) and chromosomal abnormalities (more common with smaller defects).

Omphalocele is associated with: Prematurity (10 to 50% cases), Intra-Uterine Growth Restriction (20%).

OMPHALOCELE - Repair
Primary repair involves resection of the omphalocele membrane and closure of the fascia using a layer of prosthetic material.

The omphalocele should be covered with salinesoaked gauze and the trunk should be wrapped circumferentially.

No pressure should be placed on the omphalocele sac in an effort to reduce its contents, because this maneuver may increase the risk of rupture of the sac or may interfere with abdominal venous return.

OMPHALOCELE - Repair
In infants with a giant omphalocele (defect >7 cm in diameter, liver present within the sac), the defect cannot be closed primarily because there is simply no room to reduce the viscera into the abdominal cavity.

OMPHALOCELE - Repair
Prophylactic

antibiotics should be administered in case of rupture.


A

primary repair involves resection of the omphalocele membrane and closure of the fascia.
A

layer of prosthetic material may be required to achieve closure.


Small

to medium sized defects have better prognosis than extremely large defects in which the liver is present.

OMPHALOCELE - Repair
In

large omphalocele cases, patients often have co-morbidities (pulmonary insufficiency, that can lead to significant morbidity and mortality).
Under

these circumstances, a nonoperative approach can be used.


The

omphalocele sac can be treated with desiccating substances such as povidone-iodine (Betadine), silver sulfadiazine (Silvadene), or sulfasalazine.

OMPHALOCELE - Repair
Typically 2 to 3 months are required before reepithelialization occurs. After epithelialization has occurred, attempts should be made to achieve closure of the anterior abdominal wall.

Such procedures typically require extensive measures to achieve skin closure, including the use of biosynthetic materials and also requires prolonged hospitalizations.

Can u identify??

OMPHALOCELE Incidence Covering Sac 1:6,000-10,000

GASTROSCHISIS 1:20,000-30,000 Absent

Fascial Defect
Cord Attach.

QUICK REVIEW Small to large


Present (may be ruptured) Umbilical the sac

Small (vascular compromise) Abd wall

OMPHALOCELE Herniated Bowel Other organs IUGR Protected

GASTROSCHISIS Edematous and matted Remain in abd.

Liver often in sac

Less common

Common

NEC

If sac is ruptured

18 %

OMPHALOCELE

GASTROSCHISIS 10% to 15%

Assoc. 55% to 80% Anomalies


GI

18 % (stenosis, 37 % (Volvulus Meckels D., atresia, atresias) duplications) 20 %


30 % 2% No increase

Cardiac Trisomy

THANK U!!!

11/29/11