In an era of increasing specialization, pediatric surgeons have retained the title of the "true" general surgeon by evaluating and treating a wide range of diseases in infants and children. For this reason, in their office practices, pediatric surgeons commonly see children with both complex and routine problems. This article presents problems that are frequently referred to us, but that are often confusing.
The precise incidence of ingrown toenails in children remains unknown. An estimated incidence of 2 per 1,000 in the overall population with a predorninance in the second and third decades of life suggests that ingrown toenails are common in children.1,2 Among children presenting for surgical evaluation and treatment, two peaks are observed: 0 to 3 years and 9 to 12 years. Most ingrown toenails occur in the latter group.3 Ingrown toenails are rare in early infancy.4,5 Similar to observations made in adults, a male predominance has been found among children in all age groups.3
The etiology of ingrown toenails is often attributed to compressive forces that drive the nail into the nail fold. Factors that may cause this type of compression include trauma and ill-fitting shoes. Overaggressive nail cutting will produce a proximal nail edge that can grow into, instead of away from, the nail fold. Structural features of the nail, or soft tissues surrounding the nail, have been observed that may also contribute to the development of an ingrown toenail. One study identified factors that were associated with ingrown toenails, including nail fold width, medial eversión of the great toe, and nail thickness.6 In further support of an anatomic predisposition, ingrown toenails have been observed in infants who have never had their nails cut or worn shoes.4 Malalignment of the nail, distal embedding of the nail, and hypertrophy of the lateral nail folds of the great toe have been identified as anatomic features that may account for infantile ingrown toenails.5
Ingrown toenails present with nail bed pain, erythema, swelling, and sometimes purulent drainage. In severe cases, granulation tissue that is friable and may bleed may form on the lateral wall. The pain is sometimes significant enough to make ambulation painful and limit full participation in recreational activities. Signs and symptoms can sometimes completely clear after being present for weeks or months only to rapidly recur. The great toe is most often affected, but other toes may also be involved. Both the medial and the lateral aspect of the nail can be affected in a synchronous or metachronous fashion.
The choice of treatment depends on the extent of tissue involvement. In mild cases, erythema, pain with compression of the lateral nail fold and drainage may be observed. For these cases we recommend daily soaking in warm water to achieve symptomatic relief. Topical antibiotics are applied if drainage is present. Despite the widespread use of these measures, their contribution to promoting resolution remains unknown. Oral antibiotics do not add anything to the treatment of infected ingrown toenails in healthy patients. They should be avoided even if significant drainage is present.7 Patients and their families should be educated about avoiding compression of the nail into the nail fold by wearing fitted shoes and avoiding local trauma. They should also be educated about proper nail cutting.
In more severe cases, swelling of the lateral nail fold, drainage, superficial infection, and granulation tissue may be observed. An initial trial of conservative treatment, as described for mild cases, may be tried for 1 to 2 weeks. If symptoms persist or worsen, additional treatment should be considered. The decision to refer the patient to a surgical specialist depends on the experience of the pediatrician with surgical options for treating ingrown toenails. Available treatments can be divided into two basic groups. Conservative treatments are directed at elevation of the nail edge. This goal can be achieved by placing tape or cotton wool under the nail, placing a soft plastic tube stent along the nail edge (gutter method), or thinning the central portion of the nail.8-11 Surgical treatments include removal of a wedge of nail with mechanical or chemical ablation of the germinal matrix or surgical displacement of the nail fold away from the nail.3,12 The number of available treatments attests to the frequency of symptomatic recurrence and the need for close follow-up.
When patients are referred to our practice for additional treatment, we place a tube stent. This will preserve the nail, a factor that is often important for young girls who desire a normal appearing nail. For recurrences following placement of a tube stent, we have repeated this procedure with success. We reserve wedge excision and germinal matrix ablation for children in whom placement of a tube stent has not been successful (Fig. 1).
Figure 1. Two useful strategies for treating ingrown toenails: tube stent placement and nail wedge resection with germinal matrix ablation.
UMBIUCAL DRAINAGE AND SWELLING
Most pediatricians have little difficulty recognizing and treating umbilical granulomas in the newborn infant. However, the evaluation and management of umbilical drainage and swelling that persists despite conservative treatment, in the newborn or at an older age, may be challenging and may need to be handled by a surgical specialist. A systematic approach that focuses on the presentation plus basic embryology and the natural history of the umbilical stalk can be used to identify and treat most umbilical problems.
Umbilical granulomas are the most common cause of umbilical swelling and drainage in newborns. They usually present after cord separation. After the umbilical cord sloughs, the umbilical ring is epithelialized. When this process is delayed due to factors such as low-grade infection, granulation tissue can grow on the umbilical stalk. Umbilical granulomas are asymptomatic, pink or red, and shiny.13 The granulation tissue may produce fluid that is noticed on the diaper or clothing. The drainage can appear clear to yellow or bloody and often leads to confusion with other umbilical anomalies. Umbilical granulomas can be treated by the careful application of silver nitrate on one or more occasions.
Although symptomatic anomalies of the urachus are rare, these lesions are the next most common cause of umbilical signs and symptoms in infants and children.14 The allantois obliterates before birth to form the urachus or median umbilical ligament. This fibrous cord runs between the peritoneum and the transversalis muscle from the umbilicus to the dome of the bladder. Lesions of the umbilicus can arise when the entire urachus or portions of the urachus remain patent after birth. One end of the urachus may be patent (urachal sinus), the mid portion may be patent (urachal cyst), or the entire urachus may be patent (patent urachus). Urachal sinuses and cysts are more common than is a patent urachus.15,16
Urachal sinuses can present with simple umbilical drainage or with signs of local infection, such as fever and periumbilical pain, swelling, or erythema.14-16 Chronic irritation of the umbilical skin may result in formation of a granuloma, leading to confusion of this lesion with a simple umbilical granuloma. Eversion of the umbilical skin or visualization using an otoscope may reveal a draining opening that can be cannulated to perform a sinogram, a procedure that is accurate for diagnosing this anomaly.
Urachal cysts typically present as a lower, midline abdominal mass. Most of these lesions are seen in late infancy and childhood and only become apparent when infected. When an infected urachal cyst remains untreated, it may rupture into the umbilicus, causing purulent umbilical drainage. Less commonly, it ruptures into the peritoneal cavity (leading to peritonitis). Ultrasound is the most useful test for diagnosing urachal cysts.16
Drainage at the umbilicus can also result from a patent urachus or vesicoumbilical fístula. Although drainage may be clear and intermittent, frank urine drainage at the umbilicus can be observed when distal urinary tract obstruction is also present. As with urachal sinuses, a sinogram is most useful for diagnoses.16
The treatment of all urachal anomalies is surgical excision through an infraumbilical incision. Laparoscopy may be useful for lesions that cannot be removed through this approach. In some cases, an infected urachal cyst may require antibiotic therapy or initial drainage before definitive resection. Cystoscopy should be considered at the time of excision of a patent urachus to visualize the bladder anatomy and look for an obstructing urinary tract lesion.16
Vitelline Duct Anomalies
Vitelline (omphalomesenteric) duct anomalies causing umbilical symptoms are also rare. Normally, the vitelline duct obliterates before birth and leaves no postnatal remnant. Similar to urachal anomalies, umbilical findings can arise when the vitelline duct or portions of the vitelline duct remain patent after birth.
Umbilical polyps are the most common vitelline duct anomaly and arise from residual intestinal mucosa from the vitelline duct.13 Although most umbilical polyps are believed to arise from a vitelline duct remnant, polyps originating from urachal remnants may also occur.17 An umbilical polyp presents as a cherry-red, polypoid mass.13 Umbilical granulomas and polyps thus have a similar appearance and can be confused. Eversion of the umbilical skin in an attempt to visualize a stalk may be useful for differentiating an umbilical polyp from an umbilical granuloma. Although silver nitrate can also be applied to treat umbilical polyps, removal may be more rapid with the application of a strangulating suture around the stalk.
Other vitelline duct anomalies have been observed. The entire vitelline duct may be patent (umbilical fistula), one end may be patent (vitelline sinus), or the mid portion may be patent (vitelline cyst). Umbilical fistulas may present with clear, feculent, or bilious umbilical drainage.13 Prolapse of intestinal mucosa through the fistula may be observed, establishing the diagnosis. When the diagnosis is in question, a fistulogram demonstrating passage of contrast into the small intestinal lumen will establish it. Vitelline sinuses and cysts present and are diagnosed by means similar to their urachal counterparts. Chronic irritation from both draining umbilical fistulas and vitelline sinuses may lead to formation of a granuloma that may partially or completely reduce umbilical drainage. Standard treatment of vitelline anomalies is surgical excision via an umbilical incision. An attempt should be made to ensure that any residual band connecting the umbilicus to the intestine is divided to prevent intestinal obstruction.
Figure 2. A suggested approach for the diagnosis and treatment of persistent umbilical drainage.
Other Umbilical Lesions
Umbilical lesions related to umbilical vessels are rare. The umbilical vein and arteries thrombose shortly after birth and form the ligamentum teres and the right and the medial umbilical ligaments, respectively. Umbilical drainage may result from infection following catheterization of the umbilical vessels or from formation of an umbilicoperitoneal communication caused by vessel trauma during catheter placement.18,19 These lesions may be difficult to distinguish from other causes of umbilical drainage, but should be considered when umbilical catheters have been previously placed.
Approach to the Draining Umbilicus
A systematic approach to the draining umbilicus, based on performing a physical examination and obtaining a sinogram, is shown in Figure 2. When a periumbilical mass is present, ultrasound should also be included to evaluate for a urachal or a vitelline cyst. Application of silver nitrate may be required for up to 3 weeks to remove an umbilical granuloma or polyp.13 Care should be taken to rninimize contact of silver nitrate with normal skin because burns have been reported in this setting.20 When initial treatment with silver nitrate is not successful, formation of a granuloma may be due to chronic drainage from a urachal or a vitelline anomaly.
Scalp masses can be noted on routine physical examination or by parental inspection. Several different specialists may evaluate and treat scalp masses, including pediatric surgeons, neurosurgeons, plastic surgeons, and dermatologists. This makes it difficult to estimate the incidence of these lesions. Although biased toward reporting lesions with a high likelihood of intracranial extension, the neurosurgical literature suggests that a cautious approach should be taken when evaluating and treating the child with a scalp mass.21,22 Our approach that focuses on appropriate diagnostic evaluation and referral to a surgical specialist is presented.
Scalp masses in children can be due to a wide range of diagnoses (Table). Dermoid and epidermoid tumors, cephaloceles, cephalohematoma deformans, and eosinophilic granulomas account for more than half of all scalp lesions.21,22 Epidermoid and dermoid tumors and cysts predominate from ages 1 to 7 years, whereas fibrous dysplasia is more common in older children. Malignant scalp tumors are rare and usually metastatic. Intracranial extension may occur in more than one-third of these children and is frequently associated with an intracranial mass or dural extension. Most lesions require excision for definitive diagnosis or treatment.21,22
The history and physical examination yield important initial clues to the etiology of scalp lesions. Baldwin et al. have identified a series of features that can be used to determine the etiology of scalp masses and their risk of intracranial extension (Table).23 Once it has been detennined which of these diagnostic features are present, a judgment can be made about whether to observe the mass or refer for a surgical evaluation. Useful information can be obtained using plain skull radiography. This should be performed for most patients. Referral to a surgical specialist and additional imaging are appropriate when the diagnosis remains in doubt after this initial evaluation. When intracranial extension is suspected, initial referral is best made to a neurosurgeon. The best overall diagnostic study is computed tomography of the head, because it will identify intracranial extension and visualize bone destruction. Surgery is indicated when the etiology of the lesion remains in doubt, the lesion has an unpleasant cosmetic appearance or is symptomatic, or a malignant tumor is suspected. Fine needle biopsy has been useful for diagnosing primary scalp lesions in selected children.24
PERIANAL ABSCESS AND FISTULA IN ANO
Perianal infection and abscess are common in infants and children. Between 0.5% and 4.3% of all perianal abscesses are seen in infants and children, with up to 85% occurring during the first year of life.25 A male predominance is observed, particularly before the age of 2 years. Although most of these are idiopathic in infants, perianal abscesses in older children may be associated with inflammatory bowel disease, leukemia, or immunocompromised states.
Almost all perianal abscesses in children, particularly those in infants, are simple and do not extend into the pelvic musculature. Infection begins at the anal glands in the crypts of Morgagni and extends through the soft tissue and intersphinteric space to erupt onto the perineum or the buttock. Congenitally abnormal crypts may be an etiology for abscess formation. Abnormal crypts tend to be deeper (3 to 10 mm) than normal crypts (1 to 2 mm). Deeper crypts may lead to abscess formation by trapping infection and causing cryptitis.26-29 Male predominance may be due to androgen excess or androgen-estrogen imbalance in utero. This predisposes to the formation of these abnormal crypts of Morgagni.28,30
Perianal abscesses present as erythema, pain, swelling, and induration at the perineum or the buttock. The child may be irritable and febrile. Because of their origin, these lesions occur in continuity with or close proximity to the involved crypt. Most are lateral and have an equal distribution on each side. Females tend to have superficial, localized infections, with Staphylococcus aureus being the most frequent isolate. Males tend to have Escherichia coli as a predominate isolate, supporting the theory that the infections originate in the perianal glands. In males, these abscesses, especially those in the anterior position, may have a higher risk of fistula formation.31,32
Medical management is initially indicated for most perianal abscesses because approximately one-third of them will resolve without surgery. Superficial infections may resolve without any treatment or improve with sitz baths and antibiotics. Antibiotics should cover both staphylococcal species and E. colt because these are the most common organisms. Although antibiotics may help resolve cellulitus, they do not always promote faster resolution or prevent formation of a fistula in ano.
Key Diagnostic Features of More Common Scaip Masses in Chiidren*
Figure 3. Typical appearance of a fistula in ano in an infant. (Reprinted with permission from Nakayama DK. Pediatric Surgery: Color Atlas. Philadelphia: Lippincott Williams Sc Wilkins; 1992:361.)
Abscesses that persist or are clearly fluctuant are appropriate for referral for surgical evaluation. The surgical treatment of choice is incision and drainage of the abscess. This procedure can be performed in an office or in an operating room, depending on the extent of the abscess. Larger abscesses should be packed and allowed to heal to prevent recurrences. The latter occur in as many as 85% of patients.31,33 Patients with recurrences should be examined for a fistula tract as an etiology for failure to heal.
Fistula in ano
Up to 85% of abscesses progress to fistula in ano (Fig. 3). Fistulas are identified in between 6% and 43% of abscesses at initial drainage and are often associated with recurrent abscesses.32 If they are not correctly treated, the recurrence rate can be as high as 68%.25,33 Fistula in ano was thought to originate from infected anal glands, with the abscess being the "parent of the fistula." Cryptitis from congenitally abnormal crypts of Morgagni is now thought to play a role in fistula formation. When compared with fistula in ano in adults, these lesions have several distinguishing features in children. As with perianal abscesses, an overwhelming male predominance is observed, particularly among children younger than 1 to 2 years. Most fistulas in ano occur in children younger than 1 year. Goodsall's rules can be used to predict the course of perianal fistulas in adults, but do not apply to infants and children because most fistulas are intersphinteric and have a direct course from the anal crypt to the perineum. Ischiorectal and supralevator fistulas that track through the pelvic musculature are rare and are observed only in older children who have underlying diseases such as inflammatory bowel disease, leukemia, or other immunocompromised states. A histologic examination demonstrates an epithelial lined tract that has mixed stratified squamous cells indicating infection, but transitional columnar epithelium may also be observed, suggesting a congenital etiology.26,31
The treatment of choice for fistula in ano is fistulectomy (excision of the fistula tract) or fistulotomy (opening and debriding the fistula tract). With the child under general anesthesia, a probe is passed through the tract, from the abscess and into the anal canal. The tract is then opened from the skin to the anal crypts by incising the tissue overlying the probe. The tract may be seen better by injection of methylene blue. The tract is either opened or removed completely and packed with gauze to allow it to scar and heal by secondary intention. The epithelial lining of the tract is debrided to prevent recurrence. Complete healing may take between 1 and 2 weeks.
After fistulotomy, the rate of recurrence can be high if the specific abnormal crypt causing the fistula is not identified and treated. When abnormal crypts are identified at the time of fistulomy, the recurrence rate is minimal. Antibiotics are not indicated for the treatment of fistula in ano, except when cellulitus is present. Some authors have reported success with a program of conservative management that includes antibiotics and sitz baths.34 However, the time required for complete resolution is much longer than when fistulomy is used. Complex fistulas involving deeper muscle layers often cannot be opened over a probe, because disruption of the anal sphincter and incontinence may result. In these cases, a constricting suture or elastic rubber loop (a seton) is placed through the fistula and tightened slowly during several weeks or months. The seton will pull through the underlying muscle, but will prevent complete division of the anal sphincter. Children with an underlying disease as the cause of their fistula in ano have a much higher recurrence rate. Understanding the etiology of fistula in ano and identifying those patients requiring definitive fistula therapy is essential in preventing recurrence.
Although the presentations of the disorders discussed in this article are straightforward, their treatment often remains controversial. An understanding of the basic pathophysiology and etiology of each is useful when considering either office treatment or referral to a surgical specialist. Consultation with a pediatric surgeon is encouraged when treating infants and children with these disorders.
1. Wallace WA, Milne DD, Andrew T. Gutter treatment for ingrowing toenails. Br Med J. 1979;2:168-171.
2. Lloyd-Davies RW, Brill GC. The aetiology and outpatient management of ingrowing toenails. Br J Surg. 1963;50:592597.
3. Robb JE. Surgical treatment of ingrowing toenails in infancy and childhood. Zeitschrift für Kinderchirurgie. 1982;36:63-65.
4. Bentley-Phillips B, Coll I. Ingrowing toenails of infancy. Int J Dermatol. 1983;22:115-116.
5. Rufli T, von Schulthess A, Irin P. Congenital hypertrophy of the lateral nail folds of the hallux. Dermatology. 1992; 184:296-297.
6. Langford DT, Burke C, Robertson K. Risk factors in onychocryptosis. Br J Surg. 1989;76:45-48.
7. Reyzelman AM, Trombello KA, Vayser DJ, Armstrong DG, Harkless LB. Are antibiotics necessary in the treatment of locally infected ingrown toenails? Arch Pam Med. 2000;9:930-932.
8. Lazar L, Erez I, Katz S. A conservative treatment for ingrown toenails in children. Pediatr Surg Int. 1999;15:121122.
9. Senapati A. Conservative outpatient management of ingrowing toenails. J R Soc Med. 1986;79:339-340.
10. Schulte KW, Neumann NJ, Ruzicka T. Surgical pearl: nail splinting by flexible tube: a new noninvasive treatment for ingrown toenails. J Am Acad Dermatol. 1998;39:629-630.
11. Taylor MD. Practice tip: ingrown toenails. Aust Pam Physician. 1998;27:737.
12. Hanrahan J. Practice tip: wedge resection of nail with delayed nail fold excision. Aust Pam Physician. 1995;24: 213.
13. Campbell J, Beasley SW, McMullin N, Huston JM. amicai diagnosis of umbilical swellings and discharges in children. Med J Aust. 1986;145:450-453.
14. Suita S, Nagasaki A. Urachal remnants. Semin Pediatr Surg. 1996;5:107-115.
15. Mesrobian HG, Zacharias A, Balcom AH, Cohen RD. Ten years of experience with isolated urachal anomalies in children. J Urol. 1997;158:1316-1318.
16. Cilento BG Jr, Bauer SB, Renk AB, et al. Urachal anomalies: defining the best diagnostic modality. Urology. 1998;52:120-122.
17. Oguzkurt P, Kotiloglu E, Tanyel FC, Hicsonmez A. Umbilical polyp originating from urachal remnants. Turk J Pediatr. 1996;38:371-374.
18. Kotnis R, Salloum A, Hitchcock R. Retained umbilical artery catheter presenting as an umbilical abscess. Arch Dis Child Fetal Neonatal Ed. 2000;83:F77.
19. Choi SO, Park WH, Kang JS. Purulent umbilical drainage from infection of left umbilical artery associated with open umbilicoperitoneal communication. J Pediatr Surg. 1988;23:382-383.
20. Banwell PE, Cole RP. Umbilical burns in infants: an unusual complication of topical silver nitrate therapy. Burns. 2001;27:301-302.
21. Ruge JR, Tornita T, Naidich TP, Hahn YS, McLone DG. Scalp and calvarial masses of infants and children. Neurosurgery. 1988;22:1037-1042.
22. Martinez-Lage JF, Capel A, Costa TR, Perez-Espejo MA, Poza M. The child with a mass on its head: diagnostic and surgical strategies. Childs Nerv Syst. 1992;8:247-252.
23. Baldwin HE, Berck CM, Lynfield YL. Subcutaneous nodules of the scalp: preoperative management. J Am Acad Dermatol. 1991;25:819-830.
24. Garcia-Rojo B, Garcia-Solano J, Sanchez-Sanchez C, Montalban-Romero S, Martinez-Parra D, Perez-Guillermo M. On the utility of fine-needle aspiration in the diagnosis of primary scalp lesions. Diagn Cytopathol. 2001;24:104111.
25. Festen C, vanHarten H. Perianal abscess and fistula-inano in infants. J Pediatr Surg. 1998;33:711-713.
26. Grant CS, al-Salem AH, Arum JT, et al. Childhood fistulain-ano: a clinico-pathological study. Pediatr Surg Int. 1991; 6:207-209.
27. Shafer AD, McGlone TP, Flanagan RA. Abnormal crypts of Morgagni: the cause of perianal abscess and fistula-inano. J Pediatr Surg. 1987;22:203-204.
28. Fitzgerald RJ, Harding B, Ryan W. Fistula-in-ano in childhood: a congenital etiology. J Pediatr Surg. 1985;20:80-81.
29. Poenaru D, Yazbeck S. Anal fistula in infants: etiology, features, management. J Pediatr Surg. 1993;28:1194-1195.
30. Takatsuki S. An etiology of anal fistula in infants. Keio J Med. 1976;25:1-4.
31. al-Salem AH, Laing W, Talwalker V. Fistula-in-ano in infancy and childhood. J Pediatr Surg. 1994;29:436-438.
32. Hamalainen KPJ, Sainio AP. Incidence of fistulas after drainage of acute anorectal abscesses. Dis Colon Rectum. 1998;41:1357-1362.
33. Piazza DJ, Radhakrishnan J. Perianal abscess and fistulain-ano in children. Dis Colon Rectum. 1990;33:1014-1016.
34. Rosen NG, Gibbs DL, Soffer SZ, Hong A, Sher M, Pena A. The nonoperative management of fistula-in-ano. J Pediatr Surg. 2000;35:938-939.
Key Diagnostic Features of More Common Scaip Masses in Chiidren*