PEDIATRIC SURGERY UPDATE ©
VOLUME 26, 2006
Volume 26 No 01 JANUARY 2006
Supernumerary Nipple
Supernumerary (or accessory) nipple, also known as polythelia,
is a congenital developmental abnormality that occurs most commonly over
the anterior aspect of the trunk in the pathway of the embryonic milk line
extending from the axilla to pubic region. Supernumerary nipple shows all
the histologic components observed in the normal nipple including epidermal
thickening, pilosebaceous structures, smooth muscles and mammary glands.
Incidence of supernumerary nipple is 25 per 1000 live births with a higher
prevalence for the left side and male gender. The accessory nipple is a
cosmetic defect with the potential to give rise to a neoplasm since any
disease process that involves anatomically normal breasts may affect aberrantly
located breasts or nipples as well. Due to its atypical appearance and
ectopic location, diagnosis of the anomaly may require a high index of
suspicion along with histologic verification. Once thought to have an association
with renal defects, the presence of supernumerary nipple in asymptomatic
children is not an indication to do additional diagnostic studies of the
urinary tract. Supernumerary nipple and ectopic breast are different entities.
Supernumerary nipples can be identified at birth, whereas ectopic breast
tissue becomes noticeable only after hormonal stimulation, usually during
puberty, pregnancy or lactation. Management of supernumerary nipple consist
of excision for diagnosis, treatment of symptoms, or cosmesis.
References:
1- Mehregan AH: Supernumerary nipple. A histologic study.
J Cutan Pathol 8(2):96-104, 1981
2- Johnson CA, Felson B, Jolles H: Polythelia (supernumerary
nipple): an update. South Med J 79(9):1106-8, 1986
3- Hersh JH, Bloom AS, Cromer AO, Harrison HL, Weisskopf
B: Does a supernumerary nipple/renal field defect exist? Am J Dis Child
141(9):989-91, 1987
4- Velanovich V: Ectopic breast tissue, supernumerary
breasts, and supernumerary nipples. South Med J 88(9):903-6, 1995
5- Schmidt H: Supernumerary nipples: prevalence, size,
sex and side predilection -- a prospective clinical study. Eur J Pediatr
157(10):821-3, 1998
6- Grotto I, Browner-Elhanan K, Mimouni D, Varsano I,
Cohen HA, Mimouni M: Occurrence of supernumerary nipples in children with
kidney and urinary tract malformations. Pediatr Dermatol 18(4):291-4, 2001
Gastric Heterotopia
Gastric heterotopia refers to the finding of normal gastric tissue (mucosa)
in foreign sites entirely separated from the stomach. The gastric mucosa
is of antral type with parietal and chief cells. Heterotopic gastric mucosa
has been reported to occur along the gastrointestinal tract mostly associated
with bowel duplications, Meckel's diverticulum, within the gallbladder
or cystic duct, in omphalomesenteric ducts and in the liver. Alone, intestinal
gastric heterotopia usually is found in the jejunum. A few reports call
to the attention of gastric heterotopia in the urinary bladder, spinal
column, salivary glands, bronchogenic and thyroglossal duct cysts. The
presence of gastric mucosa produces deep acid ulceration surrounding non-gastric
mucosa. This event causes abdominal pain, gastrointestinal bleeding and
rarely perforation. Meckel's diverticulum is the most common site of heterotopic
gastric mucosa. The heterotopic mucosa in Meckel's diverticulum is not
commonly colonized by H. pylori. Repeated abdominal scanning with technetium-99m
pertechnetate can help localize the ectopic gastric heterotopia. Other
children will need tagged RBC scanning or arteriogram to identify the site
of bleeding. Management of gastric heterotopia is resection whenever possible.
Simple transverse resection is not recommended for the short Meckel's diverticulum
suspicious of harboring heterotopic gastric mucosa.
References:
1- Shim YT, Kim SY: Heterotopic gastric mucosa and pancreatic
tissue in the skin of the abdominal
wall. J Pediatr Surg 27(12):1539-40, 1992
2- Karakatsanis KG, Chatzipavlidou V, Zafiriadou E, Mavroudis
N, Patsiaoura K, Gotzamani-Psarrakos A: Abdominal scanning with technetium-99m
pertechnetate localizes ectopic gastric mucosa in the jejunum: case report
and review of the literature. Eur J Nucl Med 20(6):547-50, 1993
3- Kong MS, Huang SC, Tzen KY, Lin JN: Repeated technetium-99m
pertechnetate scanning for children with obscure gastrointestinal bleeding.
J Pediatr Gastroenterol Nutr 18(3):284-7, 1994
4- Varcoe RL, Wong SW, Taylor CF, Newstead GL: Diverticulectomy
is inadequate treatment for short Meckel's diverticulum with heterotopic
mucosa. ANZ J Surg 74(10):869-72, 2004
5- Bazlul Karim AS, Matiur Rahman MD, Kamal M: Perforation
of jejunal duplication lined with ectopic gastric mucosa. J Paediatr Child
Health 40(11):649-50, 2004
6- Jimenez JC, Emil S, Steinmetz B, Romansky S, Weller
M: Recurrent gastrointestinal tract bleeding secondary to jejunal gastric
heterotopia. J Pediatr Surg 40(10): 1654-1657, 2005
Sialadenitis
Sialadenitis, defined as inflammation of a salivary gland, is a common
benign condition seen in children. Sialadenitis can be caused by an acute
viral infection (mumps), suppurative bacterial infection (most commonly
staphylococcus aureus and Haemophilus influenzae), a chronic recurrent
process without determinate cause, or as part of a granulomatous disease
process (tuberculosis, sarcoidosis, Sjögren). The parotid gland of
male children is most often involved. Clinically the child develops episodic
pain, fever and swelling of the parotid gland. Ultrasonography should be
the initial imaging study used for the examination of salivary gland lesions
in children. In newborns, acute suppurative sialoadenitis is associated
with gavage-feeding, prematurity and methicillin-resistant Staphylococcus
aureus. In the infant, the suppurative process is associated with bacteremia,
abscess formation and the need of incision and drainage. Initial management
includes systemic antibiotics therapy. Older children develop mumps sialadenitis
from improper immunization. HIV infection is an important cause of viral
parotitis in children.
References:
1- Pershall KE, Koopmann CF Jr, Coulthard SW: Sialadenitis
in children. Int J Pediatr Otorhinolaryngol. 11(2):199-203, 1986
2- Wang SL, Zou ZJ, Yu SF, Zhu JR: Recurrent swelling
of parotid glands and Sjögren's syndrome. Int J Oral Maxillofac Surg.
22(6):362-5, 1993
3- Garcia CJ, Flores PA, Arce JD, Chuaqui B, Schwartz
DS: Ultrasonography in the study of salivary gland lesions in children.
Pediatr Radiol. 28(6):418-25, 1998
4- Fraser GC. Salivary Glands. In: O'Neill JA, Rowe MI,
Grosfeld JL, et al., Pediatric Surgery, 5th ed. St. Louis, MO: Mosby-Year
Book, 1998: 727-735.
5- Brook I: Aerobic and anaerobic microbiology of suppurative
sialadenitis. J Med Microbiol. 51(6):526-9, 2002
6- McAdams RM, Mair EA, Rajnik M: Neonatal suppurative
submandibular sialadenitis: case report and literature review. Int J Pediatr
Otorhinolaryngol. 69(7):993-7, 2005
Volume 26 No 02 FEBRUARY 2006
Mucocele
Mucocele of the appendix is a rare condition described when a thin-walled
dilated appendix filled with mucous (cystic mass) is encountered in the
right lower quadrant. Chronic obstruction of the appendiceal lumen is the
most common etiological factor for a mucocele to appear. The appendiceal
obstruction can be caused by lymph node mucosal hyperplasia or a tumorous
mass such as a cystadenoma, adenocarcinoma or carcinoid. An excess of mucous
is secreted in the face of proximal obstruction enlarging the appendix
lumen. Clinically, the mucocele can present with chronic abdominal pain,
an abdominal mass as an incidental finding during surgery or imaging. Most
cases are seen in older adults with distinct female predominance. Typical
ultrasound findings are a cystic mass with variable internal echogenicity,
layered wall (onion skin sign), and calcifications in the wall. CT findings
are those of a well-encapsulated cystic mass with a wall of variable thickness.
If untreated, mucoceles may rupture producing a potentially fatal entity
known as pseudomyxoma peritonei. Management is open surgical resection.
Appendectomy is used for simple mucocele or for cystadenoma. Right hemicolectomy
is recommended for cystadenocarcinoma. Laparoscopic resection is contraindicated
due to possible dissemination of the tumorous mass.
References:
1- Madwed D, Mindelzun R, Jeffrey RB Jr: Mucocele of
the appendix: imaging findings. AJR Am J Roentgenol. 159(1):69-72, 1992
2- Soweid AM, Clarkston WK, Andrus CH, Janney CG: Diagnosis
and management of appendiceal mucoceles. Dig Dis. 16(3):183-6, 1998
3- Haritopoulos KN, Brown DC, Lewis P, Mansour F, Eltayar
AR, Labruzzo C, Hakim NS: Appendiceal mucocoele: a case report and review
of the literature. Int Surg. 86(4):259-62, 2001
4- Caspi B, Cassif E, Auslender R, Herman A, Hagay Z,
Appelman Z: The onion skin sign: a specific sonographic marker of appendiceal
mucocele. J Ultrasound Med. 23(1):117-21, 2004
5- Rampone B, Roviello F, Marrelli D, Pinto E: Giant
appendiceal mucocele: report of a case and brief review. World J Gastroenterol.
11(30):4761-3, 2005
6- Blecha MJ, Gupta A, Hoover JD, Madonna MB: Chronic
abdominal pain secondary to a mucous cystadenoma of the appendix in a 10-year-old
boy. J Pediatr Surg. 40(11):1792-4, 2005
Hemihypertrophy
Hemihypertrophy refers to soft tissue and bone overgrowth of one half
of the body of the child, others time the upper or lower extremity might
be solely affected. Hemihypertrophy can be congenital (idiopathic), or
most of time the manifestation of a medical condition such as chronic lymphedema,
lymphangiomatous malformations, neurofibromatosis, vascular malformations
including the Klippel-Trenaunay-Weber syndrome, macrodystrophia lipomatosa,
multiple enchondromatosis, or Maffucci's and Proteus syndrome. The idiopathic
congenital variety of hemihypertrophy is a rare condition seen infrequently,
but associated with a high malignant potential. Diagnosis is always performed
at birth or in the weeks following birth. The increase growth rate and
temperature changes in the hypertrophied side increases the ipsilateral
oncogenic potential. Hemihypertrophy is cosmetically unsightly and the
psychological impact can be quite prolonged. Idiopathic hemihypertrophy
is sometimes associated with primitive neoplasms of the liver, adrenals
and kidneys, mild mental retardation, genitourinary anomalies, as well
as with benign organ growth aberrations. Children developing scoliosis
can be managed with orthopedic support or limb lengthening procedures.
Regular clinical surveillance using yearly ultrasound for abdominal tumors
is recommended. Hemihypertrophy is usually not inherited.
References:
1- Beals RK: Hemihypertrophy and hemihypotrophy. Clin
Orthop Relat Res. (166):199-203, 1982
2- Levine C: The imaging of body asymmetry and hemihypertrophy.
Crit Rev Diagn Imaging. 31(1):1-80, 1990
3- Parker L, Kollin J, Vicario D, Nguyen T: Hemihypertrophy
as possible sign of renal cell carcinoma. Urology. 40(3):286-8, 1992
4- Stoll C, Alembik Y, Steib JP, De Saint-Martin A: Twelve
cases with hemihypertrophy: etiology and follow up. Genet Couns. 4(2):119-26,
1993
5- Leung AK, Fong JH, Leong AG: Hemihypertrophy. J R
Soc Health. 122(1):24-7, 2002
6- Memon MA, Mohanty S, Das K, Garg I, D'Cruz AL: Hemihypertrophy,
renal dysplasia and benign nephromegaly. Pediatr Nephrol. 20(6):821-3,
2005
Buried Penis
Buried or hidden penis is a congenital anomaly in which the penile foreskin
is not attached normally to the shaft so that the penis appears to be totally
absent. The effect produces a pseudomicropenis in an otherwise healthy
obese child. This is different to when an obese child has a thick suprapubic
subcutaneous fat pad that conceals the penis. Buried penis includes an
excessive development of the penile fascia which retracts the penis, insufficient
attachment of the penile skin at the base of the penis, often excessive
prepubic fat worsening the appearance of the abnormality but not by itself
totally explaining it, and a tight phimosis often present. A surgical approach
to the buried penis is warranted in most circumstances. There are psychological
benefits to both the patients and the parents. The basis for surgical correction
is directed at freeing the penile shaft from abnormal dartos attachments,
fixing dartos fascia to Buck's fascia to prevent retraction of the penis,
and providing adequate shaft skin coverage with the inner preputial skin.
The surgical procedure usually brings significant cosmetic and functional
improvement to the penis. Additional procedures are rarely needed due to
recurrent retraction.
References:
1- Chuang JH: Penoplasty for buried penis. J Pediatr
Surg. 30(9):1256-7, 1995
2- Lipszyc E, Pfister C, Liard A, Mitrofanoff P: Surgical
treatment of buried penis. Eur J Pediatr Surg. 7(5):292-5, 1997
3- Alter GJ, Ehrlich RM: A new technique for correction
of the hidden penis in children and adults. J Urol. 161(2):455-9, 1999
4- Kojima Y, Hayashi Y, Maruyama T, Sasaki S, Mogami
T, Ueda K, Kohri K: Correction of completely buried penis using the modified
preputial island pedicle flap method. J Pediatr Surg. 34(10):1524-6, 1999
5- Brisson P, Patel H, Chan M, Feins N: Penoplasty for
buried penis in children: report of 50 cases. J Pediatr Surg. 36(3):421-5,
2001
6- Herndon CD, Casale AJ, Cain MP, Rink RC: Long-term
outcome of the surgical treatment of concealed penis. J Urol. 170(4 Pt
2):1695-7, 2003
Volume 26 No 03 MARCH 2006
Situs Inversus
Situs inversus is a defect of lateralization that results in mirror
image positioning of abdominal and thoracic viscera. Complete situs inversus
is estimated to occur in one per 8000 persons and may be isolated, associated
with cardiac or alimentary tract malformations (duodenal obstruction and
midgut volvulus), or occur as a component of Kartagener's syndrome, with
autosomic recessive inheritance and associated disturbance of ciliary function,
causing bronchiectasis, chronic sinusitis and male infertility. Situs inversus
can present with dextrocardia, levocardia or partial heterotaxia. Children
with situs inversus and no cardiac lesion are older than two months when
diagnosed presenting vague symptoms of intermittent vomiting, abdominal
pain, constipation, diarrhea or failure to thrive. The anomaly causes a
rotational defect which can cause midgut volvulus. Situs inversus can be
diagnosed from prenatal ultrasound. An UGIS and barium enema will help
diagnosed the intestinal rotation and fixation anomaly. Liver/spleen scans
will determine the presence or absence of the spleen. A prophylactic Ladd
procedure has proven to be both safe and effective in preventing intestinal
volvulus or obstruction from congenital bands. Some workers advocate use
of laparoscopy in malrotation associated with situs inversus since it's
minimally invasive, accomplish the same surgical act and is associated
with fewer postoperative adhesions.
References:
1- Ruben GD, Templeton JM Jr, Ziegler MM: Situs inversus:
the complex inducing neonatal intestinal obstruction. J Pediatr Surg. 18(6):751-6,
1983
2- Tryfonas GI, Chaidos C, Avtzoglou PP, Zioutis J, Klokaris
A, Papanastasopoulos A: Partial situs inversus: duodenal obstruction in
a neonate with isolated levocardia. J Pediatr Surg. 27(12):1584-6, 1992
3- Chang J, Brueckner M, Touloukian RJ: Intestinal rotation
and fixation abnormalities in heterotaxia: early detection and management.
J Pediatr Surg. 28(10):1281-4, 1993
4- Cheikhelard A, De Lagausie P, Garel C, Maintenant
J, Vuillard E, Blot P, Aigrain Y: Situs inversus and bowel malrotation:
contribution of prenatal diagnosis and laparoscopy. J Pediatr Surg. 35(8):1217-9,
2000
5- Mordehai J, Cohen Z, Kurzbart E, Mares AJ: Preduodenal
portal vein causing duodenal obstruction associated with situs inversus,
intestinal malrotation, and polysplenia: A case report. J Pediatr Surg.
37(4):E5, 2002
6- Nawaz A, Matta H, Hamchou M, Jacobez A, Trad O, Al
Salem AH: Situs inversus abdominus in association with congenital duodenal
obstruction: a report of two cases and review of the literature. Pediatr
Surg Int. 21(7):589-92, 2005
Pre-duodenal Portal Vein Revisited
Pre-duodenal portal vein (PDPV) is a rare congenital vascular anomaly
which is often symptomless leading to duodenal intestinal obstruction requiring
surgical correction. PDPV is frequently associated with other congenital
malformations, among which, and in order of frequency, are duodenal stenosis
and atresia, abnormalities of the biliary tract (atresia and duplications),
annular pancreas, and malrotation (intestinal, situs inversus). The duodenal
atresia associated with PDPV is intrinsic and not due to the external pressure
of the anomalous vein on the duodenum. PDPV occurs because of persistence
of a preduodenal vitelline communicating vein. Most children with PDPV
presents with bowel obstruction and two-thirds are detected in the first
two weeks of life. Identification of PDPV is rarely made preoperative.
Prenatal Ultrasound has demonstrated the unusual position of the portal
vein in cases of PDPV. Presence of PDPV complicates any duodenal surgery
since the integrity of the vessel must be preserved to avoid portal vein
thrombosis. Management consists of duodenoduodenal bypass anastomosis anterior
to the portal vein. Another alternative is gastrojejunostomy with truncal
vagotomy.
References:
1- Esscher T: Preduodenal portal vein--a cause of intestinal
obstruction? J Pediatr Surg. 15(5):609-12, 1980
2- Patti G, Marrocco G, Mazzoni G, Catarci A: Esophageal
and duodenal atresia with preduodenal common bile duct and portal vein
in a newborn. J Pediatr Surg. 20(2):167-8, 1985
3- Fernandes ET, Burton EM, Hixson SD, Hollabaugh RS:
Preduodenal portal vein: surgery and radiographic appearance. J Pediatr
Surg. 25(12):1270-2, 1990
4- Choi SO, Park WH: Preduodenal portal vein: a cause
of prenatally diagnosed duodenal obstruction. J Pediatr Surg. 30(10):1521-2,
1995
5- Mordehai J, Cohen Z, Kurzbart E, Mares AJ: Preduodenal
portal vein causing duodenal obstruction associated with situs inversus,
intestinal malrotation, and polysplenia: A case report. J Pediatr Surg.
37(4):E5, 2002
Congenital Melanocytic Nevus
Skin nevus found in infants and children can be a source of concern
to both parents and physician dealing with pediatric patients. Congenital
melanocytic nevus (CMN) is a characteristic pigmented nevus with mild raised
borders and a strong skin discoloration caused by melanin deposits. Incidence
of CMN is 0.2% with more than 90% small nevi (< 1.5 cm). Congenital
melanocytic nevus may cause cosmetic defects and represent a risk of malignant
transformation, namely melanoma. The incidence of developing melanoma is
associated with location and size of the CMN; those identified in
the face and neck or covering large areas of the body (larger than 5 cm)
has a higher incidence of malignant transformation. Not always there is
a correlation between pre and postoperative diagnosis after prophylactic
excision of a nevi. Digital videomicroscopy using polarize light can help
increase the diagnostic yield of CMN. A high index of suspicion for cutaneous
melanoma is needed by clinicians assessing melanocytic lesions in children
and adolescents for early diagnosis taking into consideration change in
color, growth rate, and bleeding, For large lesions and those in exposed
areas of the body, surgical excision is the treatment of choice. Medium
and small lesions can be managed with Ruby laser treatment. Ruby laser
treatment does not result in scarring, mutilation, or functional impairment.
References:
1- Kruk-Jeromin J, Lewandowicz E, Rykala J: Surgical
treatment of pigmented melanocytic nevi depending upon their size and location.
Acta Chir Plast. 41(1):20-4, 1999
2- Schmid-Wendtner MH, Berking C, Baumert J, Schmidt
M, Sander CA, Plewig G, Volkenandt M: Cutaneous melanoma in childhood and
adolescence: an analysis of 36 patients. J Am Acad Dermatol. 46(6):874-9,
2002
3- Makkar HS, Frieden IJ: Congenital melanocytic nevi:
an update for the pediatrician. Curr Opin Pediatr. 14(4):397-403, 2002
4- Noordzij MJ, van den Broecke DG, Alting MC, Kon M:
Ruby laser treatment of congenital melanocytic nevi: a review of the literature
and report of our own experience. Plast Reconstr Surg. 114(3):660-7, 2004
5- Zaal LH, Mooi WJ, Sillevis Smitt JH, van der Horst
CM: Classification of congenital melanocytic naevi and malignant transformation:
a review of the literature. Br J Plast Surg. 57(8):707-19, 2004
Volume 26 No 04 APRIL 2006
Transverse Vaginal Septum
Transverse vaginal septum is a congenital condition of females that
can block the passage of vaginal secretions causing primary amenorrhea,
hematocolpus and cyclic pelvic pain. Different to imperforate hymen, in
a transverse vaginal septum you find a rim of nonbulging hymenal tissue
on the vestibular floor with an intravaginal bulging membrane. The septum
can be found in the upper, middle or lower vagina varying in thickness.
Most common location of the septum is the upper vagina. Histologically,
the diagnosis of transverse vaginal septum is made due to the presence
of müllerian duct (mesodermal origin) tissue in the septum. Transverse
vaginal septum is a defect of vertical fusion during embryogenesis of the
vagina. The estimated incidence is one per 30,000 to 84,000 women. It is
sometimes associated with genitourinary tract, gastrointestinal tract,
musculoskeletal, and cardiac malformations. Physical exam and pelvic ultrasound
are diagnostic. Surgical resection is the treatment of choice. The mucosa
on either side of the blockage should be mobilize for approximation with
interrupted sutures, while the underlying fibrous septum should be excised.
Postoperative dilation may be necessary to prevent restenosis and dyspareunia.
Patients with a complete transverse septum in the middle or upper vagina
are less likely to conceive than patients with a septum in the lower vagina.
Prompt diagnosis and surgical correction to drain accumulated blood may
preserve fertility possibly through the prevention of endometriosis.
References:
1- Rock JA, Zacur HA, Dlugi AM, Jones HW Jr, TeLinde
RW: Pregnancy success following surgical correction of imperforate hymen
and complete transverse vaginal septum. Obstet Gynecol. 59(4):448-51, 1982
2- Ahmed S, Morris LL, Atkinson E: Distal mucocolpos
and proximal hematocolpos secondary to concurrent imperforate hymen and
transverse vaginal septum. J Pediatr Surg. 34(10):1555-6, 1999
3- Quinn T, Erickson V, Knudson MM: Down's syndrome,
precocious puberty, and transverse vaginal septum: an unusual cause of
abdominal pain. J Pediatr Surg. 36(4):641-3, 2001
4- Rana A, Manandhar B, Amatya A, Baral J, Gurung G,
Giri A, Giri K: Mucocolpos due to complete transverse septum in middle
third of vagina in a 17-year-old girl. J Obstet Gynaecol Res. 28(2):86-8,
2002
5- Beyth Y, Klein Z, Weinstein S, Tepper R: Thick transverse
vaginal septum: expectant management followed by surgery. J Pediatr Adolesc
Gynecol. 17(6):379-81, 2004
Septated Vagina
A septate vagina is another congenital condition that occurs from failure
of longitudinal fusion of the lower müllerian ducts leaving a variable
amount of an asymptomatic longitudinal vaginal septum in the child. Two
vaginal canals are created by the septum completely or partially. As time
passes the septate vagina goes unnoticed until the adolescent child start
to use tampons or engage in sexual activity. The affected patient might
complain of menstrual leakage with the use of tampons since menstruation
will continue to egress from the other vaginal canal not occluded by the
tampon. This is the case with complete duplication of the müllerian
system. Reassurance and vaginal hygiene is all that is required in these
cases. Physical exams including endoscopy along with pelvic ultrasound
are diagnostic. The identification of a duplicated cervix and a vaginal
septum is consistent with several uterine malformations, which leads to
frequent misdiagnosis and errors in management. On the other hand when
the defect is an isolated partial vaginal septum the patient will complain
of dyspareunia during sexual intercourse. Management in these cases consists
of excision of the thick septa (septectomy) maintaining good hemostasis.
References:
1- Tolete-Velcek F, Hansbrough F, Kugaczewski J, Coren
CV, Klotz DH, Price AF, Laungani G, Kottmeier PK: Utero vaginal malformations:
a trap for the unsuspecting surgeon. J Pediatr Surg. 24(8):736-40, 1989
2- Mills PL, Pergament E: Urorectal septal defects in
a female and her offspring. Am J Med Genet. 13;70(3):250-2, 1997
3- Haddad B, Barranger E, Paniel BJ: Blind hemivagina:
long-term follow-up and reproductive performance in 42 cases. Hum Reprod.
14(8):1962-4, 1999
4- Patton PE, Novy MJ, Lee DM, Hickok LR: The diagnosis
and reproductive outcome after surgical treatment of the complete septate
uterus, duplicated cervix and vaginal septum. Am J Obstet Gynecol. 190(6):1669-75,
2004
Direct Inguinal Hernias
Direct inguinal hernias in infants and children are extremely rare comprising
less than 2% of all inguinal hernias. Most inguinal hernias in children
are of the indirect type where the defect and sac runs along the spermatic
cord structures from the internal ring. Direct inguinal hernias occur due
to a defect in the transversalis fascia presenting as a bulge medially
in the groin. A direct hernia should be suspected when operating for an
inguinal hernia and a typical indirect inguinal hernia is not found. Otherwise,
most direct inguinal hernias in children are the result of a recurrence
after initial repair of an indirect hernia where the inguinal floor was
injured during the surgical procedure. During repair of a direct inguinal
hernia a defect in the posterior wall of the inguinal canal medial to the
epigastric vessels will be identified. The defect is repaired joining the
transversalis fascia between the inguinal ligament and the conjoined tendon
with interrupted nonabsorbable sutures (Cooper's ligament -McVay- repair).
Laparoscopic repair of the common inguinal hernia in the child has reported
a higher incidence of direct inguinal hernia.
References:
1- Viidik T, Marshall DG: Direct inguinal hernias in
infancy and early childhood. J Pediatr Surg. 15(5):646-7, 1980
2- Gnidec AA, Marshall DG: Incarcerated direct inguinal
hernia containing uterus, both ovaries, and
fallopian tubes. J Pediatr Surg. 21(11):986, 1986
3- Wright JE, Gill AW: Direct inguinal hernias in the
newborn. Aust N Z J Surg. 61(1):78-81, 1991
4- Grosfeld JL, Minnick K, Shedd F, West KW, Rescorla
FJ, Vane DW: Inguinal hernia in children: factors affecting recurrence
in 62 cases. J Pediatr Surg. 26(3):283-7, 1991
5- Schier F, Montupet P, Esposito C: Laparoscopic inguinal
herniorrhaphy in children: a three-center experience with 933 repairs.
J Pediatr Surg. 37(3):395-7, 2002
6- Graf JL, Caty MG, Martin DJ, Glick PL: Pediatric hernias.
Semin Ultrasound CT MR. 23(2):197-200, 2002
7- Schier F, Klizaite J: Rare inguinal hernia forms in
children. Pediatr Surg Int. 20(10):748-52, 2004
Volume 26 No 05 MAY 2006
Thymoma
The thymus remains quite prominent in the anterior mediastinum during
the first year of life causing discrepancy between a normal and hyperplastic
gland. Involution occurs in response to stress and sepsis. Rebound hyperplasia
after involution can be seen after cardiac surgery, major burns and chemotherapy.
Thymoma is the most common neoplastic tumor found in the thymus of children
and adults. There is a close relationship between myasthenia gravis and
thymoma. Most thymic tumors in children are benign, share a low rate of
association with myasthenia gravis and a favorable prognosis. Thymomas
are considered malignant on the basis of macroscopic and microscopic capsular
invasiveness. The most significant predictors of long-term survival of
thymoma include complete excision, stage I disease, and lymphocytic histology.
Management of thymoma entails surgical resection through a median sternotomy.
To increase survival a policy of aggressive, complete surgical resection
of all thymomas is advice. Thymoma behaves as a rather indolent tumor,
with most deaths from causes unrelated to thymoma or its direct treatment.
Chemotherapy is reserved for patients with refractory or metastatic disease.
Thymomas are moderately radiosensitive but radiation therapy is not an
attractive option for children due to side-effects on developing organs.
References:
1- Ramon y Cajal S, Suster S: Primary thymic epithelial
neoplasms in children. Am J Surg Pathol. 15(5):466-74, 1991
2- Kaplinsky C, Mor C, Cohen IJ, Goshen Y, Yaniv I, Tamary
H, Jaber L, Stark B, Stern S, Zaizov R: Childhood malignant thymoma: clinical,
therapeutic, and immunohistochemical considerations. Pediatr Hematol
Oncol. 9(3):261-8, 1992
3- Pescarmona E, Giardini R, Brisigotti M, Callea F,
Pisacane A, Baroni CD: Thymoma in childhood: a clinicopathological study
of five cases. Histopathology. 21(1):65-8, 1992
4- Wilkins KB, Sheikh E, Green R, Patel M, George S,
Takano M, Diener-West M, Welsh J, Howard S, Askin F, Bulkley GB: Clinical
and pathologic predictors of survival in patients with thymoma. Ann Surg.
230(4):562-72, 1999
5- Dhall G, Ginsburg HB, Bodenstein L, Fefferman NR,
Greco MA, Chang MW, Gardner S: Thymoma in children: report of two cases
and review of literature. J Pediatr Hematol Oncol. 26(10):681-5, 2004
6- Rothstein DH, Voss SD, Isakoff M, Puder M: Thymoma
in a child: case report and review of the literature. Pediatr Surg Int.
21(7):548-51, 2005
Peritoneal Dialysis
Peritoneal dialysis is the preferred technique of management utilized
in almost two-third of children with chronic renal failure. The most common
complications of peritoneal dialysis are peritonitis, catheter infection
and dialysate leaks. Catheter infection risk is higher among children less
than five years of age or with a previous history of infection. Infection
occurs because the dialysate causes an alteration in the normal protective
mechanism of the peritoneum reducing the number and function of macrophages.
In the acute setting the peritoneal catheter can be placed percutaneously
into the peritoneum using the Seldinger technique or alternatively the
child hemodyalised using a central venous access (Quinton Catheter). In
the elective situation the child is taken to the operating room and a peritoneal
(Tenckhoff) cannula placed under direct vision into the peritoneum under
general anesthesia. The open or laparoscopic technique permits removal
of the omentum (omentectomy) to avoid later occlusion of the cannula during
passive effusion of the solution. The catheter should go through the muscles
(rectus) and point inferiorly to reduce the incidence of catheter infection.
Leakage and adequate fluid effusions (at least 80% of the unfused fluid
should drain back rapidly) are tested before terminating the surgical procedure.
The cannula is ready to be used, but low volumes should be initially utilized
to minimized incisional pain and reduce leaks.
References:
1- Warady BA, Sullivan EK, Alexander SR: Lessons from
the peritoneal dialysis patient database: a report of the North American
Pediatric Renal Transplant Cooperative Study. Kidney Int Suppl. 53:S68-71,
1996
2- Honda M, Iitaka K, Kawaguchi H, Hoshii S, Akashi S,
Kohsaka T, Tuzuki K, Yamaoka K, Yoshikawa N, Karashima S, Itoh Y, Hatae
K: The Japanese National Registry data on pediatric CAPD patients: a ten-year
experience. A report of the Study Group of Pediatric PD Conference. Perit
Dial Int. 16(3):269-75, 1996
3- Lewis MA, Smith T, Roberts D: Peritonitis, functional
catheter loss and the sitting of the Dacron cuff in chronic peritoneal
dialysis catheters in children. Eur J Pediatr Surg. 6(5):285-7, 1996
4- Beanes SR, Kling KM, Fonkalsrud EW, Torres M, Salusky
IB, Quinones-Baldrich WJ, Atkinson JB: Surgical aspects of dialysis in
newborns and infants weighing less than ten kilograms. J Pediatr Surg.
35(11):1543-8, 2000
5- Leblanc M, Ouimet D, Pichette V: Dialysate leaks in
peritoneal dialysis. Semin Dial. 14(1):50-4, 2001
6- Daschner M, Gfrorer S, Zachariou Z, Mehls O, Schaefer
F: Laparoscopic Tenckhoff catheter implantation in children. Perit Dial
Int. 22(1):22-6, 2002
7- Washburn KK, Currier H, Salter KJ, Brandt ML: Surgical
technique for peritoneal dialysis catheter placement in the pediatric patient:
a North American survey. Adv Perit Dial. 20:218-21, 2004
Myasthenia Gravis
Children constitute 10% of all cases of Myasthenia Gravis (MG) with
three individual forms identified: neonatal, genetic or juvenile. The neonatal
phase is transient, associated with a newborn whose mothers have MG and
the baby recovers completely after several days or weeks. Genetic MG is
not associated to a parent with MG with symptoms confined to ptosis and
almost no weakness. The juvenile phase of MG is similar to the adult phase
occurring after the age of ten. Symptoms include fluctuating weakness and
fatigue in the ocular (diplopia), facial (ptosis), bulbar or limb muscles.
weakness, fatigability, ptosis and diplopia. The child develops motor weakness,
preservation of sensation, coordination and deep tendon reflex. MG
is an autoimmune disease in which there is loss of acetylcholine receptors
at the neuromuscular junction. Thymic enlargement occurs in patients with
MG. MG is best managed: 1) enhancing neuromuscular transmission with cholinesterase
inhibitors though the effect is partial with time; 2) using immune suppression
with steroids, azathioprine or cyclophosphamide; 3) with short term immune
therapy including plasma exchange or intravenous immune globulin; 4) removal
of the thymus (thymectomy) if its enlarged or the child has increase medication
requirements.
References:
1- Vincent A: Acetylcholine receptors and myasthenia gravis. Clin Endocrinol
Metab. 12(1):57-78, 1983
2- Lopate G, Pestronk A: Autoimmune myasthenia gravis. Hosp Pract 15;28(1):109-12,
1993
3- Shillito P, Vincent A, Newsom-Davis J: Congenital myasthenic syndromes.
Neuromuscul Disord. 3(3):183-90, 1993
4- Marx A, Wilisch A, Schultz A, Gattenlohner S, Nenninger R, Muller-Hermelink
HK: Pathogenesis of myasthenia gravis. Virchows Arch. 430(5):355-64, 1997
5- Anlar B: Juvenile myasthenia: diagnosis and treatment. Paediatr
Drugs. 2(3):161-9, 2000
6- Andrews PI: Autoimmune myasthenia gravis in childhood. Semin Neurol.
24(1):101-10, 2004
Volume 26 No 05 MAY 2006
Traumatic Diaphragmatic Hernia
Motor vehicle trauma is the leading cause of an acquired diaphragmatic
hernia in a child and adult. The traumatic event can either be penetrating
directly injuring the diaphragm, or most commonly blunt abdominal causing
a sharp increase in intraabdominal pressure with rupture of the diaphragmatic
muscle. The more medial and lateral fibers of the posterior diaphragm arising
from the lumbocostal arch and the vertebrocostal trigone are the weakest
points of rupture. The posterolateral portion is virtually always the area
that ruptures with trauma. Diaphragmatic injuries are difficult to diagnose
preoperatively and can be missed easily. Traumatic diaphragmatic hernia
should be suspected on the basis of an abnormal chest radiograph in the
trauma victim with multiple injuries. If diaphragmatic injury is suspected,
ultrasound or CT Scan investigation must be performed. Most cases involve
the left diaphragm due to the buttressing effect of the liver. The incidence
of bowel strangulation is high in traumatic diaphragmatic hernias. In the
acute setting, transabdominal repair after palpation of both hemidiaphragms
is the procedure of choice because of the high incidence of associated
trauma. Injury severity score and hemorrhagic shock upon admission strongly
influence the outcome. Delayed presentation can be repaired through the
chest.
References:
1- Brown GL, Richardson JD: Traumatic diaphragmatic hernia:
a continuing challenge. Ann Thorac Surg. 39(2):170-3, 1985
2- Sukul DM, Kats E, Johannes EJ: Sixty-three cases of
traumatic injury of the diaphragm. Injury. 22(4):303-6, 1991
3- Meyers BF, McCabe CJ: Traumatic diaphragmatic hernia.
Occult marker of serious injury. Ann Surg. 218(6):783-90, 1993
4- Catasca JV, Siegel MJ: Posttraumatic diaphragmatic
herniation: CT findings in two children. Pediatr Radiol. 25(4):262-4, 1995
5- Ramos CT, Koplewitz BZ, Babyn PS, Manson PS, Ein SH:
What have we learned about traumatic diaphragmatic hernias in children?
J Pediatr Surg. 35(4):601-4, 2000
6- Mihos P, Potaris K, Gakidis J, Paraskevopoulos J,
Varvatsoulis P, Gougoutas B, Papadakis G, Lapidakis E: Traumatic rupture
of the diaphragm: experience with 65 patients. Injury. 34(3):169-72, 2003
Mediastinal Teratomas
Mediastinal teratomas are rare tumors that originate in the anterior
mediastinum and comprise almost one-fifth of all mediastinal masses. Most
grow to large size before causing symptoms. Mediastinal teratoma can appear
in any age of the child. Primary symptom is respiratory distress caused
by airway compression, followed by feeding problems related to dyspnea,
coughing, wheezing and chest pain. Most mediastinal teratomas are mature
and benign. Teratomas arise from pluripotent cells and are composed of
a wide diversity of tissues originating from three germ layers ectoderm,
mesoderm and endoderm. Besides an anterior mediastinal mass, plain chest
films can show calcifications. CT Scan is the study of choice to demonstrate
the extent of the tumor and its relationship with other structures. As
with any other suspected teratoma preoperative alpha fetoprotein and human
chorionic gonadotropin markers levels should be obtained. Teratomas are
classified as mature, immature and malignant. Mature teratomas are predominantly
cystic, while malignant teratomas are mainly solid lesions. Immature teratomas
have immature tissue with mature elements. Surgical excision through a
median sternotomy is the treatment of choice for mediastinal teratomas.
Adjuvant chemotherapy is used in immature and malignant teratoma to increase
survival.
References:
1- Quillin SP, Siegel MJ: CT features of benign and malignant
teratomas in children. J Comput Assist Tomogr. 16(5):722-6, 1992
2- Lakhoo K, Boyle M, Drake DP: Mediastinal teratomas:
review of 15 pediatric cases. J Pediatr Surg. 28(9):1161-4, 1993
3- Arai K, Ohta S, Suzuki M, Suzuki H: Primary immature
mediastinal teratoma in adulthood. Eur J Surg Oncol. 23(1):64-7,
1997
4- Hiroshima K, Toyozaki T, Iyoda A, Yusa T, Fujisawa
T, Ohwada H: Apoptosis and proliferative activity in mature and immature
teratomas of the mediastinum. Cancer. 92(7):1798-806, 2001
5- Koga H, Yamataka A, Kobayashi H, Miyamoto H, Lane
GJ, Miyano T: Median sternotomy provides excellent exposure for excising
anterior mediastinal tumors in children. Pediatr Surg Int. 21(11):864-7,
2005
Juvenile Secretory Carcinoma
Carcinoma of the breast in a child is a rare pathologic entity, constitutes
less than 1% of all breast lesions in this age group. Juvenile secretory
carcinoma is an uncommon malignant tumor that can develop in the breasts
of both sexes with a mean age of occurrence at 10 years. It's a slow growing
tumor that can recur locally and metastasize to the ipsilateral axillary
lymph nodes. Juvenile secretory carcinoma component are devoid of estrogen
and progesterone receptors. The child develops a painless mass often near
the areola in the breast early in life. The lesion is well demarcated and
unencapsulated invading the adjacent tissue. Immunohistochemical staining
for alpha lactalbumin is present. When in doubt fine needle aspiration
biopsy (cytology) can provide a preoperative diagnosis of the lesion. In
all cases, the samples are cellular and feature diffuse, prominent, intracytoplasmic
vacuoles and secretion in malignant cells with occasional signet-ring like
forms. Management consists of simple mastectomy (wide local excision) with
sentinel axillary lymph node biopsy, especially in males cases were metastasis
to the axilla are more common. Biological behavior seems to be similarly
favorable in both sexes.
References:
1- Ferguson TB Jr, McCarty KS Jr, Filston HC: Juvenile
secretory carcinoma and juvenile papillomatosis: diagnosis and treatment.
J Pediatr Surg. 22(7):637-9, 1987
2- Rosen PP, Cranor ML: Secretory carcinoma of the breast.
Arch Pathol Lab Med. 115(2):141-4, 1991
3- Serour F, Gilad A, Kopolovic J, Krispin M: Secretory
breast cancer in childhood and adolescence: report of a case and review
of the literature. Med Pediatr Oncol. 20(4):341-4, 1992
4- Gupta RK, Kenwright D, Naran S, Lallu S, Fauck R:
Fine needle aspiration cytodiagnosis of secretory carcinoma of the breast.
Cytopathology. 11(6):496-502, 2000
5- de Bree E, Askoxylakis J, Giannikaki E, Chroniaris
N, Sanidas E, Tsiftsis DD: Secretory carcinoma of the male breast. Ann
Surg Oncol. 9(7):663-7, 2002
6- Bond SJ, Buchino JJ, Nagaraj HS, McMasters KM: Sentinel
lymph node biopsy in juvenile secretory carcinoma. J Pediatr Surg. 39(1):120-1,
2004