Ranula

Sublingual salivary glands Mucous retention cysts, probably represents obstruction of the duct and has an epithelial lining

Treatment: simple incision or marsupialization The differential diagnosis of ranulas

o Cystic hygroma, Thyroglossal duct cyst, Second branchial cleft cysto Enteric cysto Dermoid & Epidermoid cyst

Thyroglossal cysts

Ectopic thyroid tissue may be mistaken for a Thyroglossal cyst {but because the incidence about 1 % of Thyroglossal abnormalities) and also easily recognizable when the lesion is exposed surgically that why no need for preop investigations

The aim of surgery is to remove the entire duct, including the central part of the body of the hyoid bone, to the level of the foramen cecum

Branchial cysts, sinuses, and fistulas

Sinuses and fistulas are encountered more commonly in infants and children, while branchial cysts occur more often in older children and young adults

Its clinical diagnosis that why no special diagnostic imaging is indicatedExternal angular dermoid cyst

It’s a clinical diagnosis Skull X-rays are not helpful for excluding an intracranial extension MRI recommended if the cyst at nasal bridge to excluding an intracranial

extensionSternocleidomastoid torticollis {SCMT}

30 % of patients presenting with SCMT have a history of breech presentation

Indication of surgery: Failure of manual stretching after > 6 months or develop of hemifacial atrophy or older than 1–2 years of age

Ocular exam is indicated in pt. without a clear musculoskeletal cause of torticollis

Cervical spine radiologic evaluation is necessary to exclude congenital osseous deformity of the cervical spine

The standard operation involves division of the SCM at the point where the sternal and clavicular heads converge

Sternal and clavicular heads, including underlying investing cervical fascia, are divided

Lymphatic malformations:

Subdivided into macrocytic, microcytic, and combined varieties Rx of macrocytic: Intralesional sclerotherapy {ethanol, doxycycline,

sodium tetradecyl sulfate, and OK-432}, It is not effective for microcytic components

Surgical excision is warranted for many microcytic and mixed lesions.

Tracheostomy:

Should be as elective operation Thyroid gland isthmus should be divided The first tracheal ring must not be included in the

tracheostomy Tracheostomy tube is changed routinely after 7

days.Indications:

1- Airway obstruction: Congenital anomalies, e.g., laryngeal web, subglottic stenosis. Failed extubation with increasing subglottic edema External trauma, e.g., ‘hanging’-type injuries. Acute infection, e.g., acute epiglottitis Tumors, e.g., hemangioma, lymphangioma. Functional obstruction, e.g., bilateral vocal cord palsy Tracheomalacia or extrinsic compression of the trachea.

2- Long-term respiratory support {Clearance of secretion}3- As a covering procedure to secure the airway for subsequent surgery on

the larynx, pharynx, or temporomandibular joints.

Cricothyrotomy

In an emergency, for example, in the case of acute epiglottitis when sudden airway obstruction may occur and peroral ETT may not be possible

Neck extended and the left hand is placed 1st

finger over cricoid cartilage and 2nd over the thyroid cartilage.

A transverse stab incision is made through the cricothyroid membrane.

The blade of the knife is then turned vertically, establishing the airway so that a tube of suitable size may be inserted to preserve the airway and to enable general anesthesia to be undertaken safely.

Elective tracheostomy is then performed.

Esophageal atresia with and without tracheoesophageal fistula {EA-TEF}

Most common associated anomalies are cardiac malformations, particularly ventricular septal defects and tetralogy of Fallot, and these are responsible for the majority of deaths.

Next most common are gastrointestinal and anorectal anomalies, followed by genitourinary tract abnormalitie

Prognostic criteria based on Weight & cardiac anomalies

Birth weight Major cardiac anomaly{MCA}

Survival (%)

Group I >1500 g No MCA 97Group II <1500 g or MCA 59Group III <1500 g Plus MCA 22

Polyhydramnios is present in 95 percent of patients with isolated atresia without fistula and in 35 percent of cases with a distal fistula. With expert fetal US it is possible to visualize the blind upper esophageal pouch filling and emptying, and an inability to detect the stomach would suggest atresia without fistula.

NGT (8–10 gauges) should be passed at birth in all cases where Polyhydramnios was present during pregnancy. Failure to advance the tube beyond 10 cm from the nose or mouth indicates esophageal atresia.

Immediate surgery for EA-TEF is seldom necessary. Scheduling the operation 12–24 hours after admission allows for full assessment for associated anomalies

But if with respiratory distress requiring assisted ventilation, particularly if associated with gastric distension, should undergo emergency transpleural ligation of the distal fistula, so the repair can be safely postponed for up to 7 days

If echocardiograms detect a right-sided aortic arch, a left-sided thoracotomy may provide easier access to the mediastinum.

Many surgeons advocate preliminary bronchoscopy to define the site of entry of the distal TEF, to exclude the presence of a proximal fistula, and to assess the degree of tracheomalacia

Dissection of the lower esophagus needs care to preserve the vagal attachments and prevent damage to the adjacent thoracic duct and left pleura

Once the anastomosis is complete, the intraesophageal tube may be withdrawn; if it is to be left in situ for feeding, its mobility should be checked to ensure that a suture has not inadvertently passed through or around it

Feeds via the transanastomotic Nasogastric tube may be commenced on the second or third day after operation.

Pos Op complications after TEF repairEarly Anastomotic leakage up to 20%

Anastomotic stricture up to 40% Recurrence of fistula up to 10%

Late – GERD -Tracheomalacia – Dysmotility

Importance of sham feeds

In infants with long-gap EA who have undergone a cervical esophagostomy in simplifying the initiation of oral nutrition following the interposition should not be underestimated.Cervical esophagostomy: indication

1. Long-gap esophageal atresia2. Disruption of a previous primary repair of esophageal atresia3. Extensive stricture of the esophagus – caustic ingestion4. Chronic aspiration5. Foreign-body perforation of the esophagus

H-type tracheoesophageal fistula

Most reliable method for diagnosis of is by a prone tube video esophagogram

Bronchoscopy with esophagoscopy will confirm the diagnosis H-type TEF Mostly pt will require intubation for some considerable time Post op Following extubation, the movement of the vocal cords should be

assessedRecurrent tracheoesophageal fistula {up to 10%}

Thoracotomy: To prevent refistulization, mediastinal pleura, intercostal muscle, or a flap of pericardium may be interposed between the esophagus and trachea

Other methods: Endoscopic use of tissue glues (Histoacryl), fibrin sealant (Tisseel)

Aortopexy

Tracheomalacia is suspected when an infant develops expiratory strider on exertion – feeding and crying. Increasing difficulty with expiration leads to difficulty with feeding, cyanotic spells, and, in extreme cases, apnea and collapse (death attacks)

DD include: Tracheomalacia, severe GERD & recurrent TEF. Diagnosis of Tracheomalacia is confirmed endoscopically when expiratory

collapse of the trachea is seen during quiet respiration The chest is entered through the bed of the 3 rd rib and the internal

mammary vessels are divided at the medial end of the incision

Despite a continued emphasis on saving the child’s native esophagus and on esophageal replacement operations utilizing the transposed stomach, esophageal replacement with colon remains an important technique for replacing the esophagus in children.

It is ideally suited for long-gap EA and may be the only choice in patients with congenital microgastria or in whom the stomach is also damaged

The interposed colon can be placed in the posterior mediastinum or substernally

A colonic segment based on the left colic vessels affords the greatest mobilization and is well vascularized in children

Emptying of the colonic segment is primarily by gravity Patients should be informed that stricture, leak, functional problems with

emptying of the colonic segment, dysphagia, and respiratory problems can result

Preoperative preparations: o Clear liquids for 24 hourso Polyethylene glycol electrolyte solution is given at a rate of 25–40

ml/kg per hour until stools are clear.o Antibiotic bowel preparation is accomplished with neomycin 50 mg/kg

and erythromycin base 60 mg/kg in three doses, the first two doses given 1 hour apart and a third at midnight before surgery, then nothing enterally

Once the colon graft is mobilized, the distal esophageal stump is resected, if possible, at the stomach with a linear stapling device to avoid reflux in the stump.

The distal cologastric anastomosis is created on the anterior stomach wall Serious consideration should be given to constructing a pyloroplasty in

all cases. A temporary gastrostomy is left for gastric decompression and transition

to oral feeds Patching of an area of persistent esophageal stricture can be

accomplished as an alternative to esophageal replacement.

Esophageal replacement with stomach

Gastric transposition involving the whole stomach {advantage of involving only one anastomosis, which is well vascularized and is associated with a low incidence of leakage}

Either by a thoracoabdominal approach or transhiatally via the posterior mediastinum without having to resort to a thoracotomy

Preserve both the right gastroepiploic vessels & Right gastric artery while the left gastric vessels are ligated and divided close to the stomach

anterior and posterior vagal nerves are divided 2nd duodenum may be Kocherized to obtain maximum mobility of the

pylorus A fine-bore feeding jejunostomy has been found to be of considerable

value in providing enteral nutrition in the first few weeks following gastric transposition, before full oral nutrition is established.

Extracorporeal membrane oxygenation {ECME}

Extracorporeal life support is a supportive rather than a therapeutic intervention.

IT provides adequate perfusion and gas exchange (venoarterial bypass) Gas exchange alone (venovenous bypass)

So avoids deleterious effects from high oxygen concentration and positive pressure ventilation while allowing resolution of reversible heart and lung pathology

A double-lumen cannula is placed into the right atrium via the right internal jugular vein. This technique is limited by the size of the vein, because the smallest cannula currently available is 12 Fr.

Once vessel dissection is completed, heparin (100 units/kg) is administered intravenously and 3 minutes allowed for circulation. During this waiting period, papaverine is instilled into the wound to enhance vein dilatation

For VA bypass, the arterial cannula is chosen (usually 10 Fr) and marked with a 2/0 silk ligature, left uncut, at a point that will allow the tip of the cannula to lie at the ostium of the brachiocephalic artery (about 2.5 cm). The venous cannula (usually 12–14 Fr) is similarly marked at a point equal to the distance from the venotomy to the right atrium (roughly 6 cm)

The common carotid artery is ligated distally

Main indications for ECMO:1. Meconium aspiration syndrome2. Congenital diaphragmatic hernia3. Pneumonia/sepsis4. Persistent pulmonary hypertension

Eventration of the diaphragm

Unilateral eventration is suspected when RT hemidiaphragm is greater than two rib levels higher than Lt, or the Lt hemidiaphragm is more than one rib level higher than Rt

Most convincing diagnostic tests are dynamic visualization of diaphragm function during spontaneous respiration {fluoroscopy and US imaging}

The physiologic consequences of the loss of diaphragm function include reduced lung volume, decreased tidal volume, increased work of

breathing, and respiratory insufficiency that may preclude ventilator weaning

The mobility of the mediastinum of an infant may result in significant respiratory embarrassment when the normal hemidiaphragm descends during inspiration, the mediastinum shifts to the normal side, and the eventration side paradoxically elevates.

Recurrent symptoms or respiratory distress that requires mechanical ventilation are indications for surgical correction

A transverse upper abdominal incision is used for bilateral eventration or in cases of unilateral eventration with malrotation

A lateral muscle-sparing seventh intercostal space thoracotomy is used for isolated left or right eventration.

In congenital eventrations with muscular atrophy, the thinned portion of the diaphragm may be excised when the thoracic approach is used and the course of the phrenic nerve is visualized.

Pledgets and non-absorbable sutures provide the most secure closure

Lung surgery Anterolateral thoracotomy: placing the patient supine with a roll just below

the side to be explored to elevate it 30–45 from the table Posterolateral thoracotomy: The patient in a lateral decubitus position In case of lobectomy: is better to control the pulmonary arterial branches

first, venous drainage second and the bronchus lastCLE: In non urgent situations, a bronchoscopy should be done before thoracotomy to ensure that there are no intrinsic bronchial problems that may be treated, thus avoiding a lobectomyCCAM:

The risks of infection and cancer remain the major indications for elective resection. Patients can Waite up to 1 year before resection. But Bronchoalveolar carcinoma, pleuropulmonary blastoma, and rhabdomyosarcoma have been reported, so close surveillance is required.Sequestration: Vascular supply arises from abdominal aorta in 85 % and needs to be ligated, It is usually found in the inferior pulmonary ligament.Empyema: 1. Exudative phase (Stage I): Fluid clear, normal pH & glucose levels + low LDH

levels2. Fibropurulent stage (Stage II): Fluid turbid & decreasing pH and glucose3. Organizing phase (Stage III): thick or no Fluid pH < 7 & glucose < 40 mg/dL

Fibrinolytic therapy: urokinase 20 000 U + 20 mL of sterile water are instilled through the chest tube, which is subsequently clamped for 2 hours. During that time, the patient’s position is frequently changed to facilitate pleural distribution of the agent. Treatment is repeated three times a day for 1–3 days, if necessary. Efficacy of treatment is determined by increased chest tube drainage and/or improvement in the radiographic appearance.

Patent ductus arteriosus {PDA}

70 % of infants < 28 weeks will need PDA to be closed, medically or surgically.

PDA results in increased blood flow to the lungs, exposing them to systemic blood pressures.

Left-to-right shunt decreases organ perfusion, which correlates with such clinical problems as necrotizing enterocolitis and lower glomerular filtration rates.

Clinically: widened pulse pressure, cardiomegaly, and a continuous murmur

Practically: ventilation dependence & persistently high oxygen requirement

Traditional therapy includes: restricting IVF, administering digitalis, diuretics, and dopamine, and adding positive end-expiratory pressure

Non-surgical closure: indomethacin; Two or three courses are usually required to promote closure, and further administration beyond this rarely improves the success rate. Contraindications to giving indomethacin include sepsis, NEC, azotemia, and prolonged coagulation. When these conditions exist, surgical intervention is necessary

PDA ligation Postop complications: chylothorax, transient vocal cord paralysis, and incomplete occlusion. If the ductus is torn, or there is inadvertent ligation of the left pulmonary artery or aorta, the baby is unlikely to survive.

Thoracoscopic sympathectomy

Hyperhidrosis is a condition of unknown etiology whereby excess sweating occurs from the eccrine glands in the palms, axillae, and soles of the feet.

Severe palmar sweating may render schoolwork almost impossible. As a general rule, the condition worsens during adolescence.

The only permanent cure is by interruption of the sympathetic supply to the eccrine glands by sympathectomy. At the present time, plantar hyperhidrosis is not treated, and treatment is confined to the management of palmar and axillary sweating.

Topical medication is ineffective in those who are severely affected. Injection of botulinum toxin provides temporary relief in axillary

hyperhidrosis. Endoscopic thoracic sympathectomy was first performed in 1942 and has

since become the standard treatment for the condition. The sympathetic trunk is identified abutting the heads of the ribs. The first

rib is not visible as it is obscured by Sibson’s fascia. The most proximal rib seen is the second rib

The sympathetic trunk is divided over the second and third ribs, incising all tissue down to bone. For those with axillary hyperhidrosis, the trunk is divided over the fourth rib as well. The length of the incision is about 2 cm in each case

Once the sympathectomy is complete, the lung is inflated under vision and the procedure is repeated on the contralateral side

Post op side effect: Horner’s syndrome 1–2 %

Inguinal Hernia and Hydrocele

Congenital IH: Boys are more commonly affected than girls, in a ratio of 9:1

60% on the right side, 25% left side, and 15 % are bilateral In the case of symptomatic IH in small PT already hospitalized in NICU

because of other illnesses, elective repair is carried out just before discharge and/or at a weight greater than 2.0 kg.

For infants diagnosed after discharge from the hospital who require ventilatory support or experience episodes of apnea and/or bradycardia in the neonatal period, elective repair is usually delayed until 50 wks of corrected conceptual age

Administration of a local anesthetic (e.g., bipivacaine 0.25 %, use 0.25 mL/kg per hernia repair side for a maximum of 3 mg/kg dose) along the ilioinguinal and iliohypogastric nerves will reduce postoperative pain

Operative & Post operative complication:o Spermatic vessels or vas deferens Injury: 0. 3 % {repaired with 7/0

monofilament}o wound infection {1-2%}o scrotal hematomao Postoperative hydrocoele {often resolves spontaneously}o Recurrent inguinal hernia {< 1%}

o Testicular atrophy

# Causes of recurrent inguinal hernia in children include:(1) Missed hernia sac or unrecognized peritoneal tear(2) Broken suture ligature at the neck of the sac(3) Failure to repair (snug) a large internal inguinal ring(4) Injury to the floor of the inguinal canal, resulting in a direct inguinal hernia(5) Severe infection(6) Increased intraabdominal pressure (VP shunts)(7) Cystic fibrosis and chronic cough(8) Connective tissue disorders (i.e., Ehlers–Danlos syndrome)

Femoral hernias: {FH}

Are most common in girls between 5 and 10 years of age. May be noted shortly after an ipsilateral IH repair {due to either a missed

femoral hernia or operative damage involving the femoral canal} 4 methods to repair a femoral hernia: obliterating the femoral canal

medial to the femoral vein. All sutures are placed between Cooper’s ligament and the inguinal ligament under direct vision before tying

1. Langenbeck: Infra-inguinal2. McVay procedure: Transinguinal3. Cheatle–Henry: Extraperitoneal repair4. Laparoscopic repair

Omphalocele/exomphalos

2ry to persistence body stalk in the midline or Failure return of herniated midgut

Covered with a sac consisting of inner peritoneum and outer amnion IV line above diaphragm is preferable to avoid IVC compression Painting sac with eschar forming agent: silver nitrate solutions or silver

sulfadiazine cream; In case of life-threatening or lethal associated anomalies

In small/medium sized omphaloceles: Sac Excision + Primary closure of fascia and skin

In case of increases intra-abdominal pressure cause respiratory embarrassment, skin closure alone, with later repair of the ventral hernia, is advisable.

For giant omphaloceles, frequently containing liver as well as bowel, attaching a Silastic ‘pouch’ to the fascia allows gradual (10–14 days) reduction of the contents into the abdominal cavity, with eventual skin flap closure.

Occasionally a prosthetic patch is needed to close the fascial defect in this setting, but skin must be mobilized sufficiently to cover the prosthesis.

Gastroschisis

Result from a defect at the site of involution of the second (right) umbilical vein

Routine CS is not recommended Intestinal abnormalities, including nonrotation, atresia, perforation, and

infarction, resulting from midgut volvulus or vascular thrombosis.

Gastroschisis requires prompt surgical intervention & require CVL access. Before surgery, patients should be normothermic, hemodynamically

stable, and have normal serum electrolytes following adequate fluid resuscitation.

Intraoperative: inspection of perforation or sites of atresia, although no effort should be made to dissect matted loops of intestine.

It is nearly always necessary to extend the abdominal wall defect to facilitate reduction of the herniated bowel {extending the defect superiorly in the midline by 1–3 cm}

Intra abdominal pressure must be < 20 mmHg {Intragastric and bladder pressure monitoring} to Avoid abdominal compartment syndrome leading to intestinal necrosis, renal hypoperfusion, and difficulty in ventilation, as well as wound disruption

Not recommended to bowel resection or anastomosis because of the marked thickening & inflammation of the intestinal wall.

Intestinal atresia can be managed by the creation of a stoma if primary abdominal wall closure is possible or by leaving the atresia in situ if Staged reduction is undertaken. A stoma can be created at the time of removal of the Silastic sac and primary abdominal wall closure.

Achalasia

Motility disorder of the esophagus characterized by an absence of peristalsis and a failure of relaxation of the lower esophageal sphincter

Histopathology: significant reduction in all neuropeptides, particularly vasoactive intestinal

Medical Treatment: Nifedipine, a calcium antagonist Forceful Dilatation: to disrupt the muscle fibers of the lower esophageal

sphincter Surgical Treatment: Heller cardiomyotomy {distal 4–6 cm of esophagus &

1 cm onto the anterior wall of the stomach + floppy Fundo}

Esophageal Manometry: The criteria for diagnosis include:1. High-pressure (>30 mmHg) lower esophageal sphincter zone2. Failure of the lower esophagus to relax in response to swallowing3. Absence of propulsive peristalsis4. Uncoordinated tertiary contractions in the body of the esophagus

Congenital atresia and stenosis of the small intestine

When the radiograph suggests a complete low obstruction, a contrast enema is given to rule out associated colonic atresia or functional obstruction, e.g., total colonic aganglionosis or meconium ileus, which may be confused with atresia of the distal ileum

Principles of surgery

Patency of the distal small and large bowel must be confirmed Back resection of the proximal dilated bowel with primary, single-layer, end-

to-end (end-to-back) anastomosis with or without tapering or plication is the most common procedure.

Residual bowel length must be documented. Additional steps may include: de-rotation of the proximal jejunum and

duodenum in high intestinal atresia, with back resection of the dilated bowel followed by tapering or inversion plication of the megaduodenum.

Residual bowel length in excess of 80 cm and the presence of an ileocecal valve usually preclude the short bowel syndrome

Bowel-Saving Procedures1. Tapering enteroplasty : This surgical procedure is indicated for bowel-

length preservation, especially in type IIIb atresia and for high jejunal atresias.

2. Plication enteroplasty 3. Antimesenteric seromuscular stripping and inversion plication:

Multiple atresias

Multiple atresias are often localized to a short segment of intestine, and resection with one anastomosis is preferred if sufficient intestinal length remains.

If the bowel length is critical, multiple anastomoses should be performedIntestinal atresia and gastroschisis

Primary anastomosis may be difficult and hazardous, and the favored option is reduction of the eviscerated intestine with the atresia left intact, closure of the abdominal wall defect, and delayed resection and primary anastomosis of the atresia at 14–21 days.

Meconium ileus {MI}

Cystic fibrosis is autosomal recessive condition with incidence of 1:2500 MI occurs in 10 percent of infants born with cystic fibrosis Abnormal meconium (mucoviscidosis) due to deficient pancreatic and

intestinal secretions & abnormal concentration of the meconium within the duodenum and proximal jejunum.

MI can be classified into uncomplicated and complicated cases Uncomplicated MI: non-operative treatment with a hypertonic contrast

material enema is recommended {hyperosmolar nature of Gastrografin (1100–1900 mOsm/L)}, Meconium pellets, followed by loose meconium, generally pass through the rectum over the next 4–8 hours. If evidence of bowel obstruction persists and the infant remains clinically and hemodynamically stable, a second or third enema may be administered. N-acetylcysteine (5%, 5 mL every 6 hours) by oral gastric tube to aid in clearing the thickened meconium from above

Complicated MI: need prompt explorationOperations for Uncomplicated Meconium Ileus

1. Mikulicz, Bishop–koop or Santulli–blanc enterostomy2. Primary resection and anastomosis3. Tube enterostomy4. Enterostomy and Irrigation5. Appendical Irrigation

Postoperative Care for Uncomplicated Meconium Ileus

Use of N-acetylcysteine (2.5–5 percent, 5–10 mL in 6 hours) through OGT When bowel function returns, OGT removed & feedings initiated along with

pancreatic enzyme supplementation Confirm diagnosis of cystic fibrosis: by sweat chloride testing and the

actual chromosomal defect is identified to assist in genetic counseling. The management of these patients requires a multidisciplinary team,

including the pediatric respiratory physician, in order to optimize the pulmonary status, as pulmonary function deterioration is the major cause of morbidity and mortality

Long-Term Complications of MI

Gastrointestinal complication includes: intussusceptions, Appendical distension with inspissated material and appendicitis, rectal prolapse, and gallbladder disease.

In the early 1990s, colonic strictures were reported by several centers in association with high pancreatic enzyme replacement.

Distal intestinal obstruction syndrome {Rx. By GoLytley (20 mL/kg per hour for 3–4 hours) or NGT Gastrografin 50 mL followed by at least 200 mL of water in children less than 8 years, and 100 mL followed by at least 400 mL of water in children over 8 years of age}

Duplications of the alimentary tract

Because alimentary tract duplications may be complicated by infection, bleeding, intestinal obstruction or volvulus, and the development of malignancy, many surgeons believe that they should be treated by early complete excision when possible

Unresectable asymptomatic duplications is Rx conservatively, as long as the patient is followed on a regular basis {adenocarcinoma of the mucosal lining is rare}.

Unresectable symptomatic duplications should be managed by partial resection in order to deal with the particular symptom

Duodenal duplicationso At second portion should undergo preoperative or Intraoperative

radiographic visualization of the bile ducts and pancreatic ducts. If the biliary or pancreatic ducts are involved, complete excision will be difficult. Partial excision and mucosal stripping are an acceptable alternative in these cases.

o In some cases, a small bowel Roux-en-Y loop can be brought up and a cyst-enterostomy created, similar to what has been described for the treatment of pancreatic pseudocyst

Total colonic duplicationso are long tubular duplications involving the entire colono They are located on the antimesenteric bordero Usually communicates proximally with the normal bowel. o If the duplication also communicates distally and involves most of the

colon and rectum, no treatment is necessary. o If the tubular duplication does not communicate distally, a communication

must be established, and if a small communication is present, this opening may need to be enlarge

Rectal duplication:o Rx options include transanal or posterior sagittal exposure and

excisiono or transanal fenestration of the common wall if the cyst large.

Fenestration is a good alternative in cases where the cyst is infected or a fistula between the cyst and the rectum is already present.

Appendectomy

Incidental appendectomy is relatively contraindicated as part of an abdominal procedure when the intestine will not otherwise be opened.

A normal appendix should not be removed if prosthetic materials are used 10–15 % negative laparotomy or laparoscopy rate is the accepted norm Informed consent also includes the negative appendectomy rate, the

possibility of drains, infectious, and hemorrhagic complications, and remote chance of a stoma

Necrotizing Enterocolitis {NEC}

Aims of Surgery are to control sepsis, remove gangrenous bowel, and preserve as much bowel length as possible

Bowel-lengthening procedures

Bowel-lengthening procedures aim to reduce the diameter of dilated bowel without loss of absorptive mucosa, to establish effective propulsion, and to use the tailored bowel to create additional isoperistaltic length for increased mucosal contact and enhanced absorption.

1 Delay transitand increase contact time

Reversed antiperistaltic segments Prejejunal or pre-ileal colon transposition Intestinal valves Intermittent occlusion tube jejunostomy

2 Improve propulsion Antemesenteric tailoring Plication (de Lorimer, Harrison)

3 Bowel expansion Tube jejunostomy for ‘controlled occlusion–recycle’ (Bianchi)

Nipple valve (Georgeson)

4 Bowel lengthening Longitudinal intestinal lengthening and tailoring

(LILT) (Bianchi) Serial transverse enteroplasty (STEP) (Kim et al.)

5 Antemesenteric blood supply

Isolated bowel segment Iowa model (Kimura) Composite bowel loops (Bianchi)

6 Sequential and combined techniques

Isolated segment +LILT (Georgeson) LILT +reversed segment (Bianchi) LILT+STEP (Kim et al.)

Portal hypertension {PHT}

Therapeutic options: pharmacologic, endoscopic, radiologic, and surgical procedures

Etiology: Portal vein thrombosis (PVT) in 30% & BA Many collateral pathways develop, including

1. Left gastric vein and short gastrics to esophageal veins and thence to azygous/hemiazygous veins in the thorax

2. Superior hemorrhoidal veins to the middle and inferior hemorrhoidal veins and ultimately the inferior vena cava (IVC)

3. Umbilical vein to superficial veins of abdominal wall and superior/inferior epigastric veins.

In general, bleeding in patients with PVT is managed by endoscopic techniques or by portosystemic shunts. Bleeding in patients with end-stage liver disease is managed by methods that do not interfere with subsequent liver transplantation, such as endosclerosis/banding or radiologic techniques (TIPS).

First priority in treatment of GI bleeding is volume replacement and stabilization

Somatostatin and octreotide can be helpful in acute variceal hemorrhage to reduce portal pressure and promote the cessation of persistent bleeding.

Once the patient is stabilized, upper intestinal endoscopy MRA is now a non-invasive alternative to traditional angiography Endoscopic Sclerotherapy: {5 % ethanolamine, 1 % tetradecyl sulfate &

5 % sodium morrhuate} Intravariceal or paravariceal, no >3 varices at time & repeated every few weeks until the varices are obliterated

Endoscopic variceal band ligation Mesocaval shunt: classic mesocaval shunt requires division of the IVC, it

can result in swelling of the lower extremities & now there is interposition graft

Side-to-side splenorenal shunt: Distal splenorenal shunt

Hepatoblastoma {HB}

Is the most common hepatic malignancy in children Majority is sporadic, although they can be associated with Beckwith–

Wiedemann syndrome, hemihypertrophy, renal/adrenal agenesis, familial adenomatous polyposis

Because asymptomatic, majority of HB are unresectable at the time of presentation. Over 90% elevated serum AFP {diagnosis and assessing response to therapy}.

Hepatocellular carcinoma (HCC) is second most common pediatric primary liver tumor Often present in more than one segment of the liver at the time of

presentation, and it has extrahepatic spread or distant metastases at least half of the time

Serum AFP is elevated in two thirds of casesBenign tumors

Account for almost one-third of primary liver tumors. Hemangiomas, including cavernous hemangiomas and

hemangioendotheliomas, are the most common benign liver tumors Non-operative management of symptomatic hemangiomas includes

corticosteroids, alpha-interferon, cyclophosphamide, and angioembolization.

Operative management includes hepatic artery ligation, enucleation, or resection

Other benign tumors

Mesenchymal hamartomas, adenomas, focal nodular hyperplasia, and hepatic cysts.

Resection is generally recommended for large lesions, symptomatic lesions, or lesions in which the diagnosis is uncertain

Liver resections In a patient without concomitant liver disease, resection of 85% of the

liver’s total mass may be safely performed with minimal risk of postoperative hepatic dysfunction

Liver tumors are considered unresectable if they involve all four sectors of the liver, or if multiple tumors involve all four sectors

Extension of a malignant tumor outside the confines of the liver is considered a contraindication to primary surgical resection

Neoadjuvant chemotherapy can benefit children with primary hepatic malignancies. Tumors treated preoperatively are generally smaller and better demarcated, favoring complete resection.

Overall mortality related to major liver resection should be less than 5 percent

five most commonly performed liver resections are:1. Right hepatectomy (right lobectomy)2. Left hepatectomy (left lobectomy)3. Extended right hepatectomy (right trisegmentectomy)4. Extended left hepatectomy (left trisegmentectomy)5. Left lateral lobectomy (left lateral segmentectomy).

Management of major abdominal trauma

Head CT, if indicated, should be performed first without contrast, to avoid contrast concealing a hemorrhagic brain injure Enteral contrast for GIT is generally not required in acute trauma setting

US: Four-view FAST examination includes Morrison’s pouch/right upper quadrant, the left flank to include the perisplenic anatomy/left upper quadrant, and a subxiphoid view to visualize the pericardium and the pouch of Douglas/pelvis. This bedside examination may be useful as a rapid screening study

Routine follow-up imaging studies have identified pseudocysts and pseudoaneurysms following splenic injury. Splenic pseudoaneurysms often cause no symptoms and appear to resolve with time. These lesions can be treated successfully with angiographic embolization techniques. Laparoscopic excision and marsupialization is highly effective

Even the most severe solid organ injuries can be treated without surgery if there is prompt response to resuscitation. In contrast, emergency laparotomy and/or embolization are indicated in patients who are hemodynamically unstable despite fluid and red blood cell transfusion. Most spleen and liver injuries requiring operation are amenable to simple methods of hemostasis using a combination of manual compression, direct suture, topical hemostatic agents, and woven polyglycolic mesh wrapping

In young children with significant hepatic injury, the sternum can be divided rapidly to expose the suprahepatic or intrapericardial IVC, allowing for total hepatic vascular isolation with occlusion of the porta hepatis, suprahepatic and infrahepatic inferior vena cava, and supraceliac aorta (optional).

Hypothermia, coagulopathy, and acidosis, triad creates a vicious cycle in which each derangement exacerbates the others

Abbreviated laparotomy with packing for hemostasis allowing resuscitation prior to plan reoperation is an alternative in unstable patients where further blood loss would be untenable. This ‘damage control’ philosophy is a systematic, phased approach to the management of the exsanguinating trauma patient

To Avoid ‘abdominal compartment syndrome: temporary abdominal wall expansion in all patients requiring packing until the hemostasis is obtained and visceral edema subsides

Surgical options in duodenal trauma

Repair of the duodenum Diversion of GIT (pyloric exclusion or a duodenal diverticulization). Gastric decompression (gastric tube insertion or gastrojejunostomy).

GIT access for feeding (jejunostomy tube or gastrojejunal anastomosis). Decompression of the duodenum (duodenostomy tube). Biliary tube drainage. Wide drainage of the repaired area (lateral duodenal drains) (Whipple procedure) should rarely be required. This

pancreaticoduodenectomy should be reserved for the most severe injuries to the duodenum and pancreas when the common blood supply is destroyed and any possibility of reconstruction is impossible.

Renal Injury

Renal injuries such as ureteropelvic junction (UPJ) disruption or segmental arterial thrombosis may occur without the presence of hematuria or hypotension. Therefore, a high index of suspicion is necessary to diagnose these injuries.

Non-visualization of the injured kidney on intravenous pyelogram and failure to uptake contrast with a large associated perirenal hematoma on CT are hallmark findings for renal artery thrombosis.

Ureteropelvic junction disruption is classically seen as perihilar extravasation of contrast with non-visualization of the distal ureter

Intravenous infusion of indigo-carmine (a vital dye excreted in the urine) at operation may help identify sites of extravasation

Proximal control of the renal vessels prior to opening Gerota’s fascia may facilitate retroperitoneal exploration.

The ‘seatbelt syndrome’

Abd wall contusion, injury hollow viscus & vertebral fracture

The mechanism of injury is hyperflexion of the torso caused by deceleration forces with the lap-belt as a fulcrum

Mechanisms of Bowel injuries: o Compression against the vertebral column with a crush injury, o Shearing of the bowel and mesentery at fixed points in the

retroperitoneum,o Immediate closed loop burst injuries on the anti-mesenteric border

when fluid filled loops are subjected to sudden increases in intraluminal pressure.

o The crush and shearing mechanisms may lead to progressive ischemia with delayed perforation or stricture.

Children with the ‘seatbelt sign’ across their lower abdomen should be admitted for serial examinations even if the initial examination and diagnostic tests are normal

Burn

Palmar surface of open hand of patient is 1 % TBSA {Can used for measurement}

ABC & Remove hot clothing immediate Hydrotherapy: small burns (<25 percent TBSA) are immersed in cold

water Copious irrigation of the wound with water is indicated for chemical burn For suspected inhalation injury, 100 % oxygen is given by facemask Escharotomy should always be considered Tetanus toxoid should be administered Parkland IVF formula: 4 mL/kg per BSA burn

Principles of maternal–fetal surgery

Advanced fetal diagnostic techniques (e.g., chorionic villous sampling, amniocentesis) and serial imaging (e.g., ultrasound, magnetic resonance imaging) have led to an increased understanding of the natural history and outcome of many fetal anomalies

The EXIT procedure is designed to achieve cardiorespiratory stabilization while maintaining uteroplacental blood flow. It is most commonly used for fetuses with potential airway obstruction due to a neck mass such as a teratoma or lymphangioma. It is similar to a Cesarean delivery except myometrial bleeding is controlled and the umbilical cord is not cut until an

airway is obtained – by orotracheal intubation, tracheostomy, or mass resection followed by intubation or tracheostomy

Conjoined twins The most widely held theory of their occurrence is that of failure to

undergo complete separation of the embryonic disc at around the fifteenth to seventeenth day of gestation (fissure theory). An alternative postulation, by Spencer, is that secondary fusion occurs between two originally separate monovular embryonic discs (fusion theory).

Classification of conjoined twinso Thoracopagus 40%o Omphalopagus 33%o Pygopagus 19%o Ischiopagus 6%o Craniopagus 2%

Once the diagnosis of conjoined twins is suspected on prenatal scan at 12 weeks’ gestation, it is important to define the anatomy of the union. Termination of the pregnancy is recommended where there is complex cardiac fusion or extensive cerebral fusion.

Pediatric Surgery Best Managements

Gastric volvulus Gastropexy {Gastrostomy} Repair diaphragmatic defects

Jejunoileal atresia Resection of the dilated and hypertrophied proximal bowel & primary end-to-end anastomosis

with or without tapering of the proximal bowelMalrotation

5 Key in Correction1. Detorsion: Counterclockwise of the bowel2. Division of Ladd’s cecal band3. Broadening of the small intestine mesentery4. Incidental appendectomy5. Placement of small bowel right and colon left

Meconium Ileus a) Nonoperative: hyperosmolar enema washoutsb) Operative: Enterostomy and intraoperative

saline irrigation for mechanical separation of the pellets from the bowel wall and evacuation of the Meconium

Rectal prolapse Sclerotherapy (30% saline, Deflux, 5% sodium

morrhuate) injected into the retro rectal space Endorectal cauterization Mucosal stripping

Biliary Hypoplasia Not surgical ; ursodeoxycholic acid and Phenobarbital

Cloacas + Common Channel less Than 3 cm

Complete urogenital sinus mobilization after rectal separation

Cloacas + Common Channel more Than 3 cm

Require a laparotomy after initial exploration by the posterior Sagittal approach

A midline laparotomy is recommended. The bladder is opened in the midline, and feeding tubes are placed into the ureters to protect them. The ureters run through the common wall between the vagina and bladder, and the stents allow for their identification during the difficult dissection of vaginas from the bladder neck.

Confirm the patency of the müllerian structures by injecting saline through a 3-Fr feeding tube through the fimbriae of the fallopian tubes.

Bladder exstrophy A. Urinary diversionB. Staged bladder reconstruction Stage 1: Initial Early Bladder Exstrophy Closure Stage 2: Epispadias Repair- at 6 to 12 months Stage 3: Bladder Neck Repair around age 4 to 5

yearsLiver Hemangioendothelioma

Asymptomatic lesions are simply monitored, and no specific therapy is instituted until symptoms occur

BCG adenitis Excision of involved LNs under cover of rifampicin and isoniazid

Traumatic pancreatitis

Management of duct disruption remains controversial

Some advocate conservative management, accepting the risk of a pseudocyst, while others advocate an early distal pancreatectomy.

Disease Pathology

NEUROBLASTOMA 1-Neuroblastoma

Small, round, blue cell tumors (scant cytoplasm and hyperchromatic nuclei) with dense nests of cells in a fibrovascular matrix and Homer-Wright pseudorosettes in 15-50%.

2-Ganglioneuroblastoma

increased schwannian Stroma

blueberry muffin baby in type 4s 3-Ganglioneuroma

Mature ganglion cells, Schwann cells, and neuritic processes

There are five types of major resection.

1. Right hepatic lobectomy (A) (segments V, VI, VII, VIII)

2. Left hepatic lobectomy (B) (segments II, III, IV)

3. Extended right hepatic lobectomy (C) (segments IV, V, VI, VII, VIII, sometimes includes segment I)

4. Left lateral segmentectomy (D) (segments II and III)

5. Extended left lobectomy (E).

Biliary atresia

Bile ductular proliferation Bile plugging. Portal-portal fibrosis. Acute inflammatory reaction.

Biliary atresia

Type I : 3% obliteration of the CBD (proximal patent)

Type II: 6%, obliteration of the CBD (cystic structures at the Porta hepatis )

Type III: 19%, atresia of the right and left hepatic ducts.

Type 4: 72%, complete extra hepatic BA

Laparoscopic view of the right ovotestis (OT) and

müllerian structures (M) in a 7-year-old with ovotesticular disorder of sexual development (formerly true hermaphroditism).

Removal of the ovarian tissue and orchiopexy was performed