PEDIATRIC SURGERY UPDATE ©
VOLUME 25, 2005
Volume 25 No 01 JULY 2005
Osteochondroma
Osteochondroma is the most common benign bone exostosis found in children.
Osteochondroma most frequently arise sporadically and as a solitary lesion,
but may also arise associated with hereditary multiple exostosis. Hereditary
multiple exostosis is an autosomal dominant disorder in which the clinical
hallmark is the growth of bony protuberances from long bones causing a
variety of orthopedic deformities. In hereditary multiple osteochondromas
the prevalence is one in 50,000 individuals. Ten percent of affected children
have no family history of multiple exostosis. Median age at the time of
diagnosis is three years. Most cases present with an obvious deformity
of the forearm, followed by an inequality in the lengths of the limbs,
an angular deformity of the knee, or a deformity of the ankle. Symptomatics
complications of osteochondroma consists of pain, fracture, osseous
deformity limiting range of motion, vascular injury, neurological compromise,
bursa formation and malignant transformation (chondrosarcoma). MRI is the
ideal imaging modality in the diagnostic evaluation of symptomatic complications
of osteochondromas and often avoids the need for further imaging. Spontaneous
resolution of a solitary osteochondroma is rare. Management of symptomatic
osteochondromas is surgical excision. Surgical complications associated
with excision consist of peroneal neurapraxias, arterial laceration, compartment
syndrome and fibular fracture. The surgical risk for the management of
osteochondromas is low.
References:
1- Schmale GA, Conrad EU 3rd, Raskind WH: The natural
history of hereditary multiple exostoses. J Bone Joint Surg Am 76(7):986-92,
1994
2- Wicklund CL, Pauli RM, Johnston D, Hecht JT: Natural
history study of hereditary multiple exostoses. Am J Med Genet 55(1):43-6,
1995
3- Wirganowicz PZ, Watts HG: Surgical risk for elective
excision of benign exostoses. J Pediatr Orthop 17(4):455-9, 1997
4- Mehta M, White LM, Knapp T, Kandel RA, Wunder JS,
Bell RS. MR imaging of symptomatic osteochondromas with pathological correlation.
Skeletal Radiol 27(8):427-33, 1998
5- Ahmed AR, Tan TS, Unni KK, Collins MS, Wenger DE,
Sim FH: Secondary chondrosarcoma in osteochondroma: report of 107 patients.
Clin Orthop Relat Res (411):193-206, 2003
6- Claikens B, Brys P, Samson I, Baert AL: Spontaneous
resolution of a solitary osteochondroma. Skeletal Radiol 27(1):53-5,
1998
Suture Granuloma
Suture granuloma, commonly known as a stitch abscess, is a fairly common
benign complication seen after surgery. Suture material is a foreign body
that causes local irritation and tissue necrosis. Suture granuloma can
occur many years after the primary surgical procedure. The most common
suture causing suture granuloma is braided silk material, a non-absorbable
suture. Monofilament and absorbable sutures carry a lower risk of infection.
At skin or subcutaneous level, the granuloma presents as a chronic intermittent
indolent infection with a burrow sinus, with no fever or signs of systemic
infection. Diagnosis is suggested with ultrasound (hypoechoic lesion).
Removal of the suture material is curative. The intra-abdominal presence
of foreign material is an important cause of adhesion formation.
Therefore, intra-abdominal contamination with foreign material should be
minimized. Suture granulomas mimic neoplasms in clinical appearance. Suture
granuloma can occur in the bronchial stump after lung resection, in the
lung parenchyma after segmentectomy or as a paravesical mass or abscess
after inguinal hernia repair. The paravesical abscess granuloma causes
urinary discomfort, swelling, tenderness and microscopic hematuria. Again,
definitive treatment is removal of the infected suture material (silk).
References:
1- Brunsvold MA, Reding ME, Kornman KS: Infected suture
granuloma: a case report. Int J Oral Maxillofac Implants 6(2):215-7, 1991
2- Lynch TH, Waymont B, Beacock CJ, Wallace DM: Paravesical
suture granuloma: a problem following herniorrhaphy. J Urol 147(2):460-2,
1992
3- Nagar H: Stitch granulomas following inguinal herniotomy:
a 10-year review. J Pediatr Surg. 28(11):1505-7, 1993
4- Luijendijk RW, de Lange DC, Wauters CC, Hop WC, Duron
JJ, Pailler JL, Camprodon BR, Holmdahl L, van Geldorp HJ, Jeekel J: Foreign
material in postoperative adhesions. Ann Surg. 223(3):242-8, 1996
5- Nagar H, Kessler A, Graif M: The role of ultrasound
in the diagnosis of stitch granulomas following paediatric herniotomy.
Pediatr Radiol. 29(11):803-6, 1999
6- Imamoglu M, Cay A, Sarihan H, Ahmetoglu A, Ozdemir
O: Paravesical abscess as an unusual late complication of inguinal hernia
repair in children. J Urol. 171(3):1268-70, 2004
Vacuum-Assisted Closure
Vacuum-assisted closure (VAC) is a novel method utilized to promote
expedite open wound closure. The technique consists of placing an open
cell foam into the wound, sealing the site with an adhesive drape while
applying subatmospheric pressure (125 mmHg below ambient) transmitted
to the wound in a controlled manner. The technique removes chronic edema
and decreases bacterial colonization leading to increased localized blood
flow. VAC utilized in acute and chronic open wounds hasten granulation
tissue formation. VAC has shown to be cost effective in the management
of complex pilonidal sinus disease, sacral and extremity ulcers, sternal
and spinal contaminated wounds, traumatic soft tissue wounds, diabetic
foot ulcers, vascular insufficiency ulcers and extensive compartmental
tissue loss areas. VAC therapy reduces the number of days to healing, reduces
hospital stay, affords fewer dressing changes, allows mobility without
bulky bandages and faster return to school or work. Further advantages
consist of a clean closed system measuring fluid loss, change on a periodic
basis rather than daily and enhancement of wound contraction. Complications
with VAC therapy are uncommon.
References:
1- Morykwas MJ, Argenta LC, Shelton-Brown EI, McGuirt
W: Vacuum-assisted closure: a new method for wound control and treatment:
animal studies and basic foundation. Ann Plast Surg. 38(6):553-62, 1997
2- McGuinness JG, Winter DC, O'Connell PR: Vacuum-assisted
closure of a complex pilonidal sinus. Dis Colon Rectum. 46(2):274-6, 2003
3- Sibbald RG, Mahoney J; V.A.C. Therapy Canadian Consensus
Group: A consensus report on the use of vacuum-assisted closure in chronic,
difficult-to-heal wounds. Ostomy Wound Manage. 49(11):52-66, 2003
4- Antony S, Terrazas S: A retrospective study: clinical
experience using vacuum-assisted closure in the treatment of wounds. J
Natl Med Assoc. 96(8):1073-7, 2004
5- Caniano DA, Ruth B, Teich S: Wound management with
vacuum-assisted closure: experience in 51 pediatric patients. J Pediatr
Surg. 40(1):128-32, 2005
6- Clubley L, Harper L: Using negative pressure therapy
for healing of a sternal wound. Nurs Times. 101(16):44-6, 2005
Volume 25 No 02 AUGUST 2005
Gardner Syndrome
Gardner's syndrome refers to a group of children born with familial
adenomatous (multiple) polyposis and significant extracolonic manifestations.
Familial adenomatous polyposis is an autosomal dominant disorder originating
from a germline alteration of the adenomatous polyposis coli gene in the
long arm of chromosome 5. The most significant extracolonic manifestation
of Gardner's syndrome consists of soft tissue (desmoid) tumors and osteomas.
Bowel cancer develops in one-third of patients with Gardner's syndrome
from malignant degeneration of the adenomatous polyps. Desmoid tumors are
typically benign but locally aggressive slow-growing tumors that surround
and compress adjacent vascular structures and viscera. Affected children
are asymptomatic until they manifest rectal bleeding or multiple soft/hard
tissue tumors. Osteomas appear in the mandible causing dental abnormalities
(odontomas, cementomas, cysts, supernumerary teeth). Surveillance by colonoscopy
is imperative in affected family members. Development of a subcutaneous
fibroma single or multiple that recurs as a desmoid tumor is a sentinel
event identifying children with Gardner's syndrome. Management of Gardner's
syndrome consists of excision of the soft/hard tissue tumor and removal
of the affected colon (proctocolectomy) with preservation of the sphincteric
muscle mechanism. Sulindac has been reported to produce drug-induced complete
regression of colonic adenomas in Gardner's syndrome.
References:
1- Svanes K, Bjerkeseth T, Ellekjaer E, Gilhuus-Moe O:
Gardner's syndrome, diagnosis and treatment. Report on a family. Acta Chir
Scand. 145(4):267-72, 1979
2- Naylor EW, Lebenthal E: Early detection of adenomatous
polyposis coli in Gardner's syndrome. Pediatrics. 63(2):222-7, 1979
3- Palmer TH Jr: Gardner's syndrome: six generations.
Am J Surg. 143(4):405-8, 1982
4- Lev R, Lebenthal E, Rossi T, Lance P: Histochemical
and morphological analysis of colonic epithelium from children with Gardner's
syndrome and adults bearing adenomatous polyps. J Pediatr Gastroenterol
Nutr. 6(3):414-25, 1987
5- Wehrli BM, Weiss SW, Yandow S, Coffin CM: Gardner-associated
fibromas (GAF) in young patients: a distinct fibrous lesion that identifies
unsuspected Gardner syndrome and risk for fibromatosis. Am J Surg Pathol.
25(5):645-51, 2001
6- Okai T, Yamaguchi Y, Sakai J, Ohtsubo K, Mouri H,
Sawabu N: Complete regression of colonic adenomas after treatment with
sulindac in Gardner's syndrome: a 4-year follow-up. J Gastroenterol. 36(11):778-82,
2001
7- Campos FG, Habr-Gama A, Kiss DR, Atui FC, Katayama
F, Gama-Rodrigues J: Extracolonic manifestations of familial adenomatous
polyposis: incidence and impact on the disease outcome. Arq Gastroenterol.
40(2):92-8, 2003
8- Huerta S, Heubner DR, Marcus DR: Mesenteric fibromatosis
in a young girl without familial adenomatous polyposis. J Pediatr Surg.
40(5):e33-6, 2005
Turcot Syndrome
Turcot (glioma-polyposis) syndrome refers to the presence of multiple
adenomatous polyposis coli associated with glioblastoma multiforme, medulloblastoma,
or glioma developing during the pediatric teens. The polyposis in Turcot
is associated with a low number of polyps, large polyps over three cm in
diameter, and complication by colonic cancer occurring during the second
or third decades of life. Turcot syndrome is determined by an autosomal
gene with pleiotropic effect and variable expressivity. Children with Turcot
syndrome can develop multiple regions of congenital hypertrophy of the
retinal pigment epithelium with areas of surrounding hypopigmentation
in the fundi of both eyes, a fact which can help in the diagnosis. Two
types of Turcot syndrome have been identified: Type I, also known as true
Turcot syndrome (autosomal recessive) with less than 100 intestinal polyps,
large size and apt to transform to the malignant tumor. Brain tumor is
mainly diagnosed as glioblastoma or astrocytoma and mismatch repair genes
might be involved. Type II with FAP-associated type (autosomal dominant)
predisposing to medulloblastoma. Management in both cases is surgical.
References:
1- Itoh H, Ohsato K: Turcot syndrome and its characteristic
colonic manifestations. Dis Colon Rectum. 28(6):399-402, 1985
2- Costa OL, Silva DM, Colnago FA, Vieira MS, Musso C:
Turcot syndrome. Autosomal dominant or recessive transmission? Dis Colon
Rectum. 30(5):391-4, 1987
3- Jamjoom ZA, Sadiq S, Mofti AB, al-Mofleh I, Ajarim
D: Turcot syndrome: report of a case and review of the literature. Int
Surg. 74(1):45-50, 1989
4- Munden PM, Sobol WM, Weingeist TA: Ocular findings
in Turcot syndrome (glioma-polyposis). Ophthalmology. 98(1):111-4, 1991
5- Hamilton SR, Liu B, Parsons RE, Papadopoulos N, Jen
J, Powell SM, Krush AJ, Berk T, Cohen Z, Tetu B, et al: The molecular basis
of Turcot's syndrome. N Engl J Med. 332(13):839-47, 1995
6- De Vos M, Hayward BE, Picton S, Sheridan E, Bonthron
DT: Novel PMS2 pseudogenes can conceal recessive mutations causing a distinctive
childhood cancer syndrome. Am J Hum Genet. 74(5):954-64, 2004
Splenic Abscess
Splenic abscess is an uncommon event identified during the pediatric
age. Nevertheless, is a potentially fatal disorder if not diagnosed and
managed in a timely fashion. Children harboring a splenic abscess present
with fever, leukocytosis and left upper quadrant abdominal pain. Simple
chest films could be associated with a left pleural effusion or basal atelectasis.
Most children with splenic abscess have an associated predisposing medical
condition such as sickle cell disease, immune deficiency (HIV), leukemia,
aplastic anemia, perforated bowel, typhoid fever, endocarditis, otitis
media, appendicitis or trauma. Staphylococci, Salmonella and Escherichia
coli are the most common etiologic agents in single abscess, while Candida
species predominates in multiple splenic abscess. Many children are septic
before the diagnosis is made. Blood cultures are seldom positive. Diagnosis
is made with abdominal ultrasound or CT-Scan. Initial management incorporating
the strategy of preserving the spleen consists of CT-guided percutaneous
drainage and antibiotics. This combined approach is effective in two-thirds
of cases. If the child does not improve, splenectomy follows. Children
with immune deficiency and splenic abscess have rapid resolution of symptoms
with immediate splenectomy. Some cases with splenic abscess will completely
resolve with antibiotics alone.
References:
1- Hadas-Halpren I, Hiller N, Dolberg M: Percutaneous
drainage of splenic abscesses: an effective and safe procedure. Br J Radiol.
65(779):968-70, 1992
2- Fernandes ET, Tavares PB, Garcette CB: Conservative
management of splenic abscesses in children. J Pediatr Surg. 27(12):1578-9,
1992
3- Smith MD Jr, Nio M, Camel JE, Sato JK, Atkinson JB:
Management of splenic abscess in immunocompromised children. J Pediatr
Surg. 28(6):823-6, 1993
4- Ooi LL, Leong SS: Splenic abscesses from 1987 to 1995.
Am J Surg. 1997 Jul;174(1):87-93
5- Frumiento C, Sartorelli K, Vane D: Complications of
splenic injuries: expansion of the nonoperative theorem. J Pediatr Surg.
35(5):788-91, 2000
6- Ng KK, Lee TY, Wan YL, Tan CF, Lui KW, Cheung YC,
Cheng YF: Splenic abscess: diagnosis and management. Hepatogastroenterology.
49(44):567-71, 2002
7- Kang M, Saxena AK, Gulati M, Suri S: Ultrasound-guided
percutaneous catheter drainage of splenic abscess. Pediatr Radiol. 34(3):271-3,
2004
Volume 25 No 03 SEPTEMBER 2005
Polydactylism
Polydactylism (accessory finger or toe) is one of the most common congenital
anomaly of hands or feet in children. The prevalence of polydactylism is
almost two cases for every each 1000 born alive newborns. Familial occurrence
is associated in almost 20% of cases with variable gene penetrance. Polydactylism
of the hand is more common than in the foot. In the hand the ulnar accessory
finger predominates. A few cases undergo traumatic intrauterine amputation,
a condition known as rudimentary polydactylism. Different genes are involved
in the pathomorphogenesis of postaxial polydactylism. Polydactyly may be
preaxial (medial, thumb side) or tibial (hallux-side), postaxial (lateral)
or ulnar (side of the little finger or toe), and central (middle fingers
or toes). The duplication may appear at the distal and medial phalanges
or at the whole digit. Surgical amputation of the affected finger or toe
is indicated for cosmetic reasons or for functional disturbances in wearing
shoes respectively. Careful clinical and radiographic evaluation should
be made prior to treatment to achieve good functional and cosmetic results.
Polydactylism can be diagnosed prenatally and when isolated is associated
with good perinatal outcome. Primary suture ligation of accessory
digits in infancy can be associated with later development of neuroma in
the stump. Identification and high transection of the accessory digital
nerve is essential in the treatment of pedunculated supernumerary digits.
References:
1- Perez-Molina JJ, Alfaro-Alfaro N, Lopez-Zermeno MC,
Garcia-Calderon MA: Polydactyly in 26,670 consecutive births. The clinical
characteristics, prevalence and risk factors. Bol Med Hosp Infant Mex.
50(11):803-8, 1993
2- Cifuentes L, Nazer J, Huber ME, Ramirez R, Nazer C,
Morales I: Polydactyly: a genetic epidemiological study in Santiago, Chile.
Rev Med Chil. 124(3):313-8, 1996
3- Bader B, Grill F, Lamprecht E: Polydactyly of the
foot. Orthopade. 28(2):125-32, 1999
4- Bromley B, Shipp TD, Benacerraf B: Isolated polydactyly:
prenatal diagnosis and perinatal outcome. Prenat Diagn. 20(11):905-8, 2000
5- Leber GE, Gosain AK: Surgical excision of pedunculated
supernumerary digits prevents traumatic
amputation neuromas. Pediatr Dermatol. 20(2):108-12,
2003
Postsplenectomy Guidelines
Removal of the spleen in children is considered necessary in some hemolytic
diseases and trauma. Hemolytic disease where splenectomy is therapeutic
includes idiopathic thrombocytopenic purpura, congenital spherocytosis
and Sickle cell disease. Removal of the spleen with its attendant immunologic
shortness can cause overwhelming sepsis. Overwhelming postsplenectomy infection
is a fulminant process that carries a poor prognosis.The risk of serious
infection is highest among young children, in immunologically compromised
individuals and in the first few years after splenectomy. Current guidelines
for children undergoing elective splenectomy include immunization for pneumococcus,
meningococcus and hemophilus at least two weeks prior to the procedure.
After emergency splenectomy the immunization protocol is the same, except
that the amnestic response of the host is lower. Another guideline for
splenectomized children includes the use of continuous antibiotic
prophylaxis (penicillin) until the age of twelve years. Compliance with
this antibiotic regimen is poor. It is recommended that parents be advised
to bring the postsplenectomy child to the hospital anytime an illness or
fever develops that might require an immediate loading dose of an appropriate
antibiotic.
References:
1- Posey DL, Marks C: Overwhelming postsplenectomy sepsis
in childhood. Am J Surg. 145(3):318-21, 1983
2- Brigden ML, Pattullo AL: Prevention and management
of overwhelming postsplenectomy infection--an update. Crit Care Med. 27(4):836-42,
1999
3- Waghorn DJ: Overwhelming infection in asplenic patients:
current best practice preventive measures are not being followed. J Clin
Pathol. 54(3):214-8, 2001
4- Davies JM, Barnes R, Milligan D; British Committee
for Standards in Haematology. Working Party of the Haematology/Oncology
Task Force: Update of guidelines for the prevention and treatment of infection
in patients with an absent or dysfunctional spleen. Clin Med. 2(5):440-3,
2002
5- Deodhar M, Kakkar N: An audit of splenectomies in
a teaching hospital in North India. Are postsplenectomy guidelines being
complied with? J Clin Pathol. 57(4):407-10, 2004
6- Bolton-Maggs PH, Stevens RF, Dodd NJ, Lamont G, Tittensor
P, King MJ; General Haematology Task Force of the British Committee for
Standards in Haematology: Guidelines for the diagnosis and management of
hereditary spherocytosis. Br J Haematol. 126(4):455-74, 2004
Splenic Artery Embolization
Splenic artery embolization as an alternative for splenectomy is a minimally
invasive technique that has been utilized for the past 25 years. The procedure
is done under local anesthesia, sedation or general anesthesia. Partial
splenic artery embolization has been used in cases of thalassemia major
to reduce transfusion requirements with variable results. Also in children
with secondary hypersplenism or massive spleens due to portal hypertension,
variceal bleeding (portal vein thrombosis, biliary atresia and biliary
cirrhosis) or myelodysplastic disorders. The leukopenia and thrombocytopenia
is corrected temporarily and variceal hemorrhage is ameliorated in most
cases. Objective is to embolize at least two-third of the splenic circulation.
After splenic artery embolization the child develops prolonged fever, pain
in the left hypochondrium, leukocytosis and ileus. If the embolization
causes total shutdown of the arterial circulation the child might develop
a subcapsular hematoma with effusion, abscess formation or need for open
splenectomy. Embolization allows for safe surgical splenectomy. Long term
follow-up shows evidence of partial splenic regeneration. Partial splenic
embolization is a useful method for reducing serum bilirubin concentrations
in patients with hypersplenism following the Kasai procedure for biliary
atresia, has also been found safe and effective minimally invasive
treatment for patients with bleeding from a blocked distal splenorenal
shunt, and as therapy for post-traumatic splenic artery pseudoaneurysm.
References:
1- Kumpe DA, Rumack CM, Pretorius DH, Stoecker TJ, Stellin
GP: Partial splenic embolization in children with hypersplenism. Radiology.
155(2):357-62, 1985
2- Ionescu GO, Daniil C: Splenic artery embolization
in hematologic diseases in children. Chir Pediatr. 28(6):285-9, 1987
3- Brandt CT, Rothbarth LJ, Kumpe D, Karrer FM, Lilly
JR: Splenic embolization in children: long-term efficacy. J Pediatr Surg.
24(7):642-4, 1989
4- Shah R, Mahour GH, Ford EG, Stanley P: Partial splenic
embolization. An effective alternative to splenectomy for hypersplenism.
Am Surg. 56(12):774-7, 1990
5- Hickman MP, Lucas D, Novak Z, Rao B, Gold RE, Parvey
L, Tonkin IL, Hansen DE: Preoperative embolization of the spleen in children
with hypersplenism. J Vasc Interv Radiol. 3(4):647-52, 1992
6- Ando H, Ito T, Nagaya M: Partial splenic embolization
decreases the serum bilirubin level in patients with hypersplenism following
the Kasai procedure for biliary atresia. J Am Coll Surg. 182(3):206-10,
1996
7- Petersons A, Volrats O, Bernsteins A: The first experience
with non-operative treatment of hypersplenism in children with portal hypertension.
Eur J Pediatr Surg. 12(5):299-303, 2002
8- Shah SR, Pramesh CS, Deshmukh HL, Mathur SK: Splenic
artery embolization for variceal hemorrhage following blocked distal splenorenal
shunt. Hepatogastroenterology. 50(52):1167-8, 2003
9- Yardeni D, Polley TZ Jr, Coran AG: Splenic artery
embolization for post-traumatic splenic artery pseudoaneurysm in children.
J Trauma. 57(2):404-7, 2004
Volume 25 No 03 OCTOBER 2005
Hemolytic-Uremic Syndrome
Hemolytic-Uremic Syndrome (HUS) refers to the constellation of signs
and symptoms that occurs after enteric infection with the verotoxic producing
bacteria Escherichia Coli 0157:H7. The organisms are carried in the intestines
of cattle; partially cooked contaminated hamburger is the single most common
vector. E. coli produces a potent cytotoxin that gains access to the circulation,
is taken up by glycolipid receptors on intestinal and glomerular endothelial
cells causing cell death. Usually the HUS triad consists of an microangiopathic
hemolytic anemia, thrombocytopenia and acute renal failure. HUS is the
most common cause of acute renal failure in infants and young children.
The prodromal phase of HUS starts with fever, vomiting, crampy abdominal
pain and bloody diarrhea. In a few occasions the child will develop signs
of peritoneal irritation mimicking an acute abdomen. After the prodromal
phase the child develops pallor, severe anemia, petechia, oliguria, edema,
hypertension and electrolyte disturbances. Due to the associated inflammatory
enterocolitis simple abdominal films might show thumbprinting indicative
of edema and intestinal mucosal hemorrhage. Treatment of HUS is supportive,
but plasma exchange may be useful in selected high-risk subsets. Antibiotics
have not yielded any benefit. Plasma infusions and plasma exchange appear
to be efficacious. Dialysis is necessary in many children. Surgical complications
associated with HUS include bowel perforation (pneumoperitoneum), intussusception
or later development of intestinal strictures.
References:
1- Siegler RL: Hemolytic uremic syndrome in children.
Curr Opin Pediatr. 7(2):159-63, 1995
2- Gordjani N, Sutor AH, Zimmerhackl LB, Brandis M: Hemolytic
uremic syndromes in childhood. Semin Thromb Hemost. 23(3):281-93, 1997
3- Trachtman H, Christen E: Pathogenesis, treatment,
and therapeutic trials in hemolytic uremic syndrome. Curr Opin Pediatr.
11(2):162-8, 1999
4- Robson WL: Haemolytic uraemic syndrome. Paediatr Drugs.
2(4):243-52, 2000
5- MacConnachie AA, Todd WT: Potential therapeutic agents
for the prevention and treatment of haemolytic uraemic syndrome in shiga
toxin producing Escherichia coli infection. Curr Opin Infect Dis. 17(5):479-82,
2004
6- Cleary TG: The role of Shiga-toxin-producing Escherichia
coli in hemorrhagic colitis and hemolytic uremic syndrome. Semin Pediatr
Infect Dis. 15(4):260-5, 2004
Giant Pigmented Nevus
Giant Pigmented Nevus (GPN) refers to a congenital melanocytic nevi
characterized by a diameter of 20 cm or greater in adulthood, or a lesion
occupying greater than 2% of the body surface area of an infant or child.
Generally, melanocytic nevi is solitary, but smaller satellite lesions
may be present within a GPN. The mode of inheritance of GPN is probably
multifactorial with a 2:1 female predominance. The lesion is typically
brown pigmented, varies in size and shape, contains hair and can be found
anywhere on the face, neck, trunk or extremity of the child. GNP extensively
involving the extremities can result in reduced growth of the affected
limb. GPN has a higher incidence of malignant transformation (melanoma
formation) due to the increase number of nevus cells at risk for such transformation.
Lifetime incidence of malignant transformation is four to 6%. This capacity
of GPN to developed into malignant melanoma is the main reason for opting
for prophylactic surgical excision as treatment of choice. Tissues expansion
is the primary modality for excision and reconstruction in the face and
scalp since it can be started early in life and repeated as required. For
the trunk excision with abdominoplasty or skin graft is preferred. In the
extremity excision and skin grafting is best option. Dermabrasion or laser
therapy cannot ensure complete removal of nevus cells.
References:
1- Borges AF, Lineberger AS: Malignant melanoma without
metastasis in a giant nevus. Ann Plast Surg. 12(5):454-60, 1984
2- Bauer BS, Vicari FA: An approach to excision of congenital
giant pigmented nevi in infancy and early childhood. J Pediatr Surg. 23(6):509-14,
1988
3- Ruiz-Maldonado R, Tamayo L, Laterza AM, Duran C: Giant
pigmented nevi: clinical, histopathologic, and therapeutic considerations.
J Pediatr. 120(6):906-11, 1992
4- Rompel R, Moser M, Petres J: Dermabrasion of the congenital
nevocellular nevi: experience on 215 patients. Dermatology. 194:261, 1997
5- Hamilton SA, Kirk J, Morris AM: Long-term results
of surgical excision and skin grafting for a giant hairy naevus of the
face: time for a return to conventional wisdom? Br J Plast Surg. 54(6):543-5,
2001
Alveolar Capillary Dysplasia
Alveolar capillary dysplasia (ACD), also known as acinar dysplasia,
is a rare cause of severe and irreversible pulmonary hypertension that
usually presents with acute onset respiratory failure during the first
48 hours of life. ACD is characterized by muscularization of pulmonary
arterioles, deficient number of alveolar units and pulmonary capillary
vessels with thickened interalveolar septa. ACD also shows abnormally
immature parenchymal development in the lungs. The primary pulmonary vascular
anomaly is likely to be a failure of fetal lung vascularization dating
from the second trimester of fetal life. ACD is uniformly fatal due to
inefficient gas exchange. Usual presentation is a term neonate, normal
at delivery that develops sudden hypoxia, respiratory acidosis and hypotension
within 48 hours of life. Associated anomalies include cardiac, intestinal
and GU anomalies. Management consists of mechanical ventilation followed
by high frequency ventilation or ECMO. A lung biopsy establishes the histologic
diagnosis of this uniformly fatal entity. Open lung biopsy may prevent
from using costly, invasive and probably ineffective procedures such as
ECMO.
References:
1- Newman B, Yunis E: Primary alveolar capillary dysplasia.
Pediatr Radiol. 21(1):20-2, 1990
2- Cullinane C, Cox PN, Silver MM: Persistent pulmonary
hypertension of the newborn due to alveolar capillary dysplasia. Pediatr
Pathol. 12(4):499-514, 1992
3- Alameh J, Bachiri A, Devisme L, Truffert P, Rakza
T, Riou Y, Manouvrier S, Lequien P, Storme L: Alveolar capillary dysplasia:
a cause of persistent pulmonary hypertension of the newborn. Eur J Pediatr.
161(5):262-6, 2002
4- Hugosson CO, Salama HM, Al-Dayel F, Khoumais N, Kattan
AH: Primary alveolar capillary dysplasia (acinar dysplasia) and surfactant
protein B deficiency: a clinical, radiological and pathological study.
Pediatr Radiol. 35(3):311-6, 2005
5- Michalsky MP, Arca MJ, Groenman F, Hammond S, Tibboel
D, Caniano DA: Alveolar capillary dysplasia: a logical approach to a fatal
disease. J Pediatr Surg. 40(6): 1100-1105, 2005
Volume 25 No 05 NOVEMBER 2005
Seprafilm
Adhesion formation after abdominal surgery is an expected sequelae which
can cause future development of chronic abdominal pain, acute or chronic
bowel obstruction or infertility in a minority if patients. Seprafilm is
a sodium hyaluronate and carboxymethylcellulose antiadhesion bio adsorbable
membrane that is available for use during abdominal surgery. Preclinical
studies in animals have shown that Seprafilm is safe and effective in reducing
postsurgical adhesions. In adults, seprafilm has shown to be safe and significantly
reduces the incidence, extent, and severity of postoperative adhesions
to the midline incision compared with no treatment. Has also been found
to reduce the formation of postsurgical adhesions in patients with ulcerative
colitis or familial polyposis who has undergone restorative proctocolectomy
and J-pouch along with gynecologic patients undergoing myomectomy. Seprafilm
had no adverse effect on wound healing. Seprafilm adhesion barrier has
resulted in a significant reduction of adhesion formation to polypropylene
mesh during repair of abdominal wall hernias. The anti-adhesive effects
of seprafilm might be caused by increasing the level of peritoneal hydroxyproline
levels though experimental evidence suggests the physical properties (barrier,
hydroflotation and siliconizing effect) of the membrane are primarily responsible
for adhesion prevention. Wrapping the suture or staple line of a fresh
bowel anastomosis with seprafilm should be avoided, because the data suggest
that this practice may increase the risk of anastomotic leaks.
References:
1- Burns JW, Colt MJ, Burgees LS, Skinner KC: Preclinical
evaluation of Seprafilm bioresorbable membrane. Eur J Surg Suppl. (577):40-8,
1997
2- Beck DE: The role of Seprafilm bioresorbable membrane
in adhesion prevention. Eur J Surg Suppl. (577):49-55, 1997
3- Baptista ML, Bonsack ME, Delaney JP: Seprafilm reduces
adhesions to polypropylene mesh. Surgery. 128(1):86-92, 2000
4- Altuntas I, Tarhan O, Delibas N: Seprafilm reduces
adhesions to polypropylene mesh and increases peritoneal hydroxyproline.
Am Surg. 68(9):759-61, 2002
5- Beck DE, Cohen Z, Fleshman JW, Kaufman HS, van Goor
H, Wolff BG; Adhesion Study Group Steering Committee: A prospective, randomized,
multicenter, controlled study of the safety of Seprafilm adhesion barrier
in abdominopelvic surgery of the intestine. Dis Colon Rectum. 46(10):1310-9,
2003
6- Tarhan OR, Eroglu A, Cetin R, Y Nce A, Bulbul
M, Altuntas YR: Effects of seprafilm on peritoneal fibrinolytic system.
ANZ J Surg. 75(8):690-2, 2005
7- Inoue M, Uchida K, Miki C, Kusunoki M: Efficacy of
Seprafilm for reducing reoperative risk in pediatric surgical patients
undergoing abdominal surgery. J Pediatr Surg. 40(8):1301-6, 2005
Chronic Granulomatous Disease
Chronic Granulomatous Disease (CGD) is a very rare inherited primary
immunodeficiency disease of childhood. Due to defective respiratory burst,
phagocytic cells of children with CGD are not able to kill certain bacterias
and fungi despite normal chemotaxis and phagocytosis. The proper functioning
of the NADPH oxidase of the phagocytic cell is impaired. Children with
CGD are rendered susceptible to infection by a group of catalase positive
microorganisms due to the inability of phagocytic cells to reduce molecular
oxygen and create reactive oxygen metabolites necessary for normal intracellular
killing. Catalase positive organisms affecting children with CGD include
staph aureus, Escherichia Coli, Serratia, Salmonella, Candida and Aspergillus.
Children with CGD present clinically with recurrent abscess involving skin,
soft tissue (most common site), lymph nodes, lung, bone and liver that
respond poorly to antibiotics needing surgical drainage. Palisading granulomas
with central necrosis can be seen in histologic samples. Though four different
types of CGD have been described, most cases are X-linked defects. Diagnosis
of CGD is made by showing the inability of neutrophils from the patient
to undergo respiratory burst after phagocytosis (NBT Test). Fungal infections
account for most deaths. Children with CGD should receive long-term Trimethoprim-Sulfamethoxazole
prophylaxis. Gamma interferon reduces the number of infection requiring
hospitalizations.
References:
1- Frayha HH, Biggar WD: Chronic granulomatous disease
of childhood: a changing pattern? J Clin Immunol. 3(3):287-91, 1983
2- Leonard AS, Mulholland MW, Filipovich AH: Surgery
of the immunodeficient child. Surg Clin North Am. 65(6):1505-25, 1985
3- Nakhleh RE, Glock M, Snover DC: Hepatic pathology
of chronic granulomatous disease of childhood. Arch Pathol Lab Med. 116(1):71-5,
1992
4- Eckert JW, Abramson SL, Starke J, Brandt ML: The surgical
implications of chronic granulomatous disease. Am J Surg. 169(3):320-3,
1995
5- San Vicente B, Parri FJ, Castanon M, Sancho MA, Martin
Mateos A, Morales L: Chronic granulomatous disease: the surgical aspects.
Cir Pediatr. 12(4):148-51, 1999
6- Chen LE, Minkes RK, Shackelford PG, Strasberg SM,
Kuo EY, Langer JC: Cut it out: Managing hepatic abscesses in patients with
chronic granulomatous disease. J Pediatr Surg. 38(5):709-13, 2003
Primary Peritonitis
Primary peritonitis or spontaneous bacterial peritonitis refers to a
diffuse infection of the peritoneum with no obvious focus of infection.
Includes peritoneal infection in children with indwelling catheters such
as peritoneal cannulas and ventriculo-peritoneal shunts. During the past
20 years the incidence of primary peritonitis has decreased due to the
widespread use of systemic antibiotics. The bloodstream is the most common
pathway of infection toward the peritoneum. Clinically the child develops
high fever, vomiting and generalized peritoneal signs sometimes indistinguishable
from acute appendicitis needing urgent surgery. Paracentesis smear or intraoperative
gram stain culture when no secondary cause for the peritonitis can be established
usually obtains the causative organism of the peritonitis. During exploratory
laparotomy no secondary cause is identified and the peritoneal fluid is
usually cloudy. Antibiotics should be continued until peritoneal cultures
arrive. Most children with primary peritonitis have a smooth recovery.
References:
1- McDougal WS, Izant RJ Jr, Zollinger RM Jr: Primary
peritonitis in infancy and childhood. Ann Surg. 181(3):310-3, 1975
2- Fine RN, Salusky IB, Hall T, et al: Peritonitis in
children undergoing continuous ambulatory peritoneal dialysis. Pediatrics
71:806, 1983
3- Gaskill SJ, Marlin AE: Spontaneous bacterial peritonitis
in patients with ventriculoperitoneal shunts. Pediatr Neurosurg. 26(3):115-9,
1997
4- Hoshii S, Honda M, Itami N, Oh S, Matsumura C, Moriya
S, Mori M, Hatae K, Ito Y, Karashima S: Sclerosing encapsulating peritonitis
in pediatric peritoneal dialysis patients. Pediatr Nephrol. 14(4):275-9,
2000
5- Mayer MP, Schweizer P: Primary peritonitis in a child
caused by Haemophilus parainfluenzae. Pediatr Surg Int. 18(8):728-9, 2002
Volume 25 No 06 DECEMBER 2005
Abdomino-Scrotal Hydrocele
Scrotal hydrocele is fairly commonly seen in infants. Most scrotal hydrocele
will disappear during the first year of life of the child as the fluid
accumulated in the tunica vaginalis after the processus vaginalis have
closed disappears. Abdomino-scrotal hydrocele (ASH), also known as "hydrocele
en bissac", is a very rare condition seen in infants consisting of a collection
of fluid in the tunica vaginalis extending through the inguinal canal into
the abdominal cavity. Clinically, the child with an inguino-scrotal hydrocele
has an abdominal mass of variable size and firm consistency characterized
by increase in tension of the hydrocele when squeezing the abdominal mass
and viceversa. The abdominal compartment can be retroperitoneal or properitoneal.
An increasing pressure within the hydrocele is transmitted above the deep
inguinal ring because of the inexpansible musculofascial covering of the
inguinal canal. The diagnosis of an abdomino-scrotal hydrocele is made
with the help of ultrasound or MRI showing the fluid filled cavities in
both the abdominal (pelvic) and scrotal compartments in communication.
ASH should be differentiated from other cystic tumors of the abdominal
cavity such as hydronephrosis, bladder diverticulum, mesenteric cysts and
lymphangiomas. ASH has been found to cause obstructive uropathy, reduced
blood supply to the testis or hemorrhage. Total excision of the abdomino-scrotal
hydrocele through an inguinal approach is the proposed treatment of choice.
References:
1- Burgues PL, Alvarez JA, Hernandez L, Teixidor JL:
Abdominoscrotal hydrocele. J Pediatr Surg 21(11):987-8, 1986
2- Khan AH, Yazbeck S: Abdominoscrotal hydrocele: a cause
of abdominal mass in children: a case report and review of the literature.
J Pediatr Surg 22(9):809-10, 1987
3- Wlochynski T, Wassermann J, Generowicz Z: Abdominoscrotal
hydrocele in childhood. J Pediatr Surg 28(2):248-50, 1993
4- Spier LN, Cohen H, Kenigsberg K: Bilateral abdominoscrotal
hydrocele: a case report. J Pediatr Surg 30(9):1382-3, 1995
5- Mahomed AA, Stockdale EJ, Varghese J, Youngson GG:
Abdominoscrotal hydrocoeles: little place for conservatism. Pediatr Surg
Int 13(2-3):186-8, 1998
6- Belman AB: Abdominoscrotal hydrocele in infancy: a
review and presentation of the scrotal
approach for correction. J Urol. 165(1):225-7, 2001
7- Celayir AC, Akyuz U, Ciftlik H, Gurbuz A, Danismend
N: A critical observation about the pathogenesis of abdominoscrotal hydrocele.
J Pediatr Surg 36(7):1082-4, 2001
8- Estevao-Costa J, Morgado H, Soares-Oliveira M, Campos
M, Carvalho JL: Hemorrhagic abdominoscrotal hydrocele. A challenging entity.
J Pediatr Surg 40: 731-733, 2005
Group B Streptococcus associated Diaphragmatic Hernia
Group B streptococcus (GBS) is a possible cause of chorioamnionitis,
endometritis and urinary tract infections in pregnant woman. Maternal transmission
of GBS occurs following fever during labor, the rupturing of membranes
for more than 18 hours before delivery, prematurity and chorioamnionitis.
Group B Beta Hemolytic streptococcal (GBS) infection among newborn infants
is usually confined to lung pneumonia, sepsis or meningitis with a high
mortality rate. An association between GBS lung infection and late-onset
diaphragmatic hernia development has been reported previously. Whenever
a child has radiologic evidence of opacification of the diaphragm, persistent
atelectasis of the lower lung lobes or an evolving pleural effusion, the
diagnosis of late-presentation diaphragmatic hernia should be entertained.
The mechanism for the association between GBS and late-presentation right
diaphragmatic hernia focus on the abnormal pulmonary compliance produce
by the GBS inflammatory process which delay visceral herniation splinting
the defect buttressed by the liver. When the GBS inflammation subsides,
the intrathoracic pressure reduces promoting the herniation of visceral
content through the diaphragm. Diagnosis is made with simple chest films.
Management of the late-presenting diaphragmatic hernia is operative after
the pneumonia is gone. Prognosis is excellent in most cases.
References:
1- Velaphi S, Siegel JD, Wendel GD Jr, Cushion N, Eid
WM, Sanchez PJ: Early-onset group B streptococcal infection after a combined
maternal and neonatal group B streptococcal chemoprophylaxis strategy.
Pediatrics. 111(3):541-7, 2003
2- McCarten KM, Rosenberg HK, Borden S 4th, Mandell GA:
Delayed appearance of right diaphragmatic hernia associated with group
B streptococcal infection in newborns. Radiology. 139(2):385-9, 1981
3- Vachharajani AJ, Shah JK, Paes BA: Late-onset left
diaphragmatic hernia after group B streptococcal sepsis: An unusual presentation.
J Pediatr Surg. 37(6):932-3, 2002
4- Rescorla FJ, Yoder MC, West KW, Grosfeld JL: Delayed
presentation of a right-sided diaphragmatic hernia and group B streptococcal
sepsis. Two case reports and a review of the literature. Arch Surg. 124(9):1083-6,
1989
5- Handa N, Suita S, Shono T, Kukita J: Right-sided diaphragmatic
hernia following group B streptococcal pneumonia and sepsis. J Pediatr
Surg. 27(6):764-6, 1992
6- Ashcraft KW, Holder TM, Amoury RA, Hall FK, Rising
WD, Hall RT, Sharp RJ: Diagnosis and treatment of right Bochdalek hernia
associated with group B streptococcal pneumonia and sepsis in the neonate.
J Pediatr Surg. 18(4):480-5, 1983
7- Jain M, Ford WD, Hayward C: Beta haemolytic streptococcal
infection does not cause right diaphragmatic eventration. Pediatr
Surg Int. 12(2-3):188-9, 1997
Ribs Exostosis
Exostosis of the ribs is a rare benign condition seen sporadically or
most commonly as manifestation of a genetic disorder known as Hereditary
Multiple Exostosis (HME). Children with exostosis of the ribs can
develop acute or chronic thoracic pain, hemothorax, pericardial effusion,
localized bronchiectasis, brachial plexus palsy and blood vessel entrapment,
or create disfiguring bony deformities. Hereditary multiple exostosis is
characterized by osteochondroma development in multiple areas of the body.
HME is inherited as autosomal dominant, commonly diagnosed by the first
decade of life, affecting mainly the long bones of the extremity. The rib
exostosis associated with HME can protrude toward the thoracic cavity pleural
space and produce hemothorax or pleural effusion due to direct irritation
of the diaphragm or pericardium by the sharp growth. Diagnosis is made
with CT-Scan. Management consists of resection of the exostosis or the
rib either by open thoracotomy or video-assisted thoracoscopy in symptomatic
individuals. Prognosis is good in most cases.
References:
1- Teijeira FJ, Baril C, Younge D: Spontaneous hemothorax
in a patient with hereditary multiple exostoses. Ann Thorac Surg. 48(5):717-8,
1989
2- Reynolds JR, Morgan E: Haemothorax caused by a solitary
costal exostosis. Thorax. 45(1):68-9, 1990
3- Simansky DA, Paley M, Werczberger A, Bar Ziv Y, Yellin
A: Exostosis of a rib causing laceration of the diaphragm: diagnosis and
management. Ann Thorac Surg. 63(3):856-7, 1997
4- Tang AT, Hulin SJ, Weeden DF: Surgical treatment for
an unusual cause of localized bronchiectasis. Ann Thorac Surg. 69(5):1586-7,
2000
5- Alifano M, Morcos M, Molina T, Regnard JF: An unusual
cause of hiccup: costal exostosis. Treatment by video-assisted thoracic
surgery. Eur J Cardiothorac Surg. 23(6):1056-8, 2003
6- Cowles RA, Rowe DH, Arkovitz MS: Hereditary multiple
exostoses of the ribs: an unusual cause of hemothorax and pericardial effusion.
J Pediatr Surg 40(7): 1197-1200, 2005