walaupun jarang, reseksi dengan anastomosis primer kadang-kadang diperlukan dan pertama kali

dijelaskan pada tahun 1962.01. Kebocoran anastomosis mempersulit upaya awal dengan pendekatan ini,
tetapi hasil yang meningkat baru-baru ini dilaporkan. Hasil yang memuaskan setelah reseksi dengan
anastomosis primer tergantung pada reseksi yang adekuat dari usus yang terganggu. , evakuasi
mekonium proksimal dan distal lengkap, dan pemeliharaan pasokan darah yang memadai.

Pendekatan bedah lain melibatkan reseksi, anastomosis, dan enterostomi sementara melalui mana
irigasi pasca operasi dapat disampaikan (Gambar 32-8). Beberapa stoma telah digunakan: enterostomi
laras ganda Mikulicz, enterostomi cerobong asap Uskup-Koop dan enterostomi proksimal Santulli dan
Blanc. Kerugian dari ini dan prosedur lain yang menggunakan reseksi dan stoma (s) termasuk potensi
output stoma volume tinggi, kehilangan panjang usus karena reseksi, dan kebutuhan untuk prosedur
kedua untuk membangun kembali kelangsungan usus.

Enteral feeds in infants with uncomplicated MI and CF may be initiated with breast milk or infant
formula, along with supplemental pancreatic enzymes and vitamins. Caution must be used when
prescribing enteric enzyme medication to patients with MICF Treatment failures and complications
include fibrosing colonopathy from excessive enzyme doses and MI equivalent, or distal intes- tinal
obstruction syndrome (DIOS) from inadequate enzyme therapy or generic substitutions for proprietary
medications.Often patients with a complicated postoperative course will require either continuous
enteral feeds or total parenteral nutrition (TPN).

Dila- tion of the small bowel by the obstructing meconium may lead to mucosal damage that could
contribute to poor peristalsis or malabsorption. In parients with complicated MI or in those with
significant loss of intestinal length, initiating the enteral feeding with a predigested, diluted formula at
low continuous volumes is best. If this is well tolerated, the concentration should be increased followed
by the volume. Pancreatic enzymes should be given with enteral feeds (even with predigested formula)
starting at 2,000-4,000 lipase units per 120 mL of full strength formula. Capsules containing enteric-
coated micro- formula or applesauce in older infants. The microcap- sules should not be crushed as this
will expose the enzymes the acid of the stomach where they will be destroyed. Uncrushed pancreatic
enzymes should be given EVEN WITH MCT-OIL CONTAINING FORMULA

Infants with MI are at increased risk for cholestasis, articularly if they have had or are receiving TPN.
Alka- spheres can be opened and the contents mixed with MCT-oil containing formulas. 1 e phosphatase,
alanine aminotransferase (ALT),aspartate aminotransferase (AST), and bilirubin should be monitored
weekly. The fluid and nutritional status of infants who have had significant bowel resection (greater than
one-third) may be difficult to manage. In addition, the presence of an ileostomy may lead to excessive
losses fluid and sodium. If access to the distal, defunctional- ized bowel is possible, drip feeds of
glutamine-enriched formula or instillation of the effluent from the proximal stoma may be given at low
volumes enhance bowel growth and help prevent bacterial translocation. Gastric acid hypersecretion is
seen in patients who have short bowel syndrome." An acidic intestinal envi- ronment inactivates
pancreatic enzymes and prevents dis- solution of enteric-coated microcapsules. Hi-receptor antagonists
or proton pump inhibitors may be used as an creatic enzyme therapy in patients who have had
significant bowel resections. Patients with excessive sweat and intestinal sodium losses may develop a
total body sodium deficit. Urine sodium should be measured in infants with ileostomies, especially when
there is growth failure, even if serum sodium levels are normal. Those with a urine sodium less than
adjunct with 10 mEq/L will need sodium (and possibly bicarbonate supplementation

Pulmonary Management

Although clinical lung disease is usually a delayed com- plication, mucous plugging and atelectasis can be
seen Prophylactic pulmonary care with chest physiotherapy should be initiated early in the postoperative
period. The head-down position should not be used as this increases the risk of gastroesophageal reflux
(GER) and aspiration Infants should receive nebulized albuterol twice daily fol- lowed by chest
physiotherapy. Prophylactic antibiotics are contraindicated, and antibiotic therapy should be directed by
respiratory cultures, if needed

Prognosis

The prognosis for infants with MI was uniformly poor, despite operative treatment, prior to the mid-
1900s Early series reported mortality rates of 50-67 % . " The improved survival in infants with MI can be
attributed to ny factors. Advances in prenatal diagnosis, pulmonary and neonatal intensive care,
nutrition, antibiotics, anesthesia, operative management, and an improved understanding of the
pathophysiology and treatment of the CF complications have resulted in dramatic prognos tic
improvement for infants with both complicated and 90 have been reported in uncomplicated MI , and up
to 93 % in com simple M122 113 Survival rates o f 85 to 100 plicated cases.14.11 4,84,113 Previously, it
was thought that patients with CF pre senting with MI have worse outcomes than those without MI.
However, it is no longer clear if this is accurate Several long-term follow-up studies of patients with MI
report pulmonary function at age 13 years to be no dif- ferent between those born with and those
without MI.114.115 However, a recent prospective study found chil- dren with MI have worse lung
function and more obstruc- tive lung disease than those with CF but without MI

Furthermore, comparison of the nutritional status of a similar population of patients with CF suggests
that those who presented with MI suffer long-term nutritional com- plications and other problems.