What Are the Goals of Hypospadias Repair?

Patient Presentation
A 2-week-old male came to clinic for his well-child care. He had been born at another hospital and this was his first visit. His parents were concerned that his “penis looked funny,” after his circumcision. They reported multiple wet diapers without any blood or odor, and the infant did not seem to be bothered when urinating or at other times. The past medical history revealed a full-term infant without prenatal or natal problems. The family history had no genitourinary or genetic problems.

The pertinent physical exam showed a vigorous infant with a weight of 3645 grams up past his birth weight of 3490 grams (50%), head circumference of 35 cm (50%) and length 51 cm (75%). His examination was normal except for a circumcised phallus that was well-healed. The meatal opening was elongated ventrally from the tip of the glans to almost the corona. The rest of the phallus did not show any abnormalities including no chordee and both testicles were located within the scrotum. The anus was patent and in a normal position.

The diagnosis of a distal hypospadias was made. The diagnosis was discussed with the parents and a referral to urology was made. “At this time, there is nothing special for you to do, but if there is a need for surgical correction, then the pediatric urologists are the ones who will help you. He is able to urinate normally now and doesn’t seem to be bothered. But I understand the concerns about this and sexual function in the future, so we’ll have you talk with the urologist,” said the pediatrician.

Discussion
Hypospadias is one of the most common congenital malformations and is the most common penile malformation. It is defined as the urethral opening occurring on the ventral side of the penis. Other ventral tissues can be affected thus other problems such as penile curvature and/or scrotal problems can also occur. Embryologically the penis is formed in the following way:
“At the beginning of the ambisexual stage, the urethral groove [is] bounded by the urethral folds [and] extends about halfway distally along the ventral aspect of the elongating genital tubercle. At the distal aspect of the urethral groove is the solid urethral plate…that extends to the glans of the developing penis. The urethral groove [then] extends distally to the glans by canalization of the urethral plate…. The urethral folds grow to the midline where they fuse to extend the penile urethra distally to the glans….” Examples of this process can be found here in Yamada et.al.’s article in Figures 4 and 5.

Hypospadias prevalence world-wide and over time is “difficult to estimate,” as published studies show immense heterogenity. Some world-wide geographical areas show increased prevalence but others do not and methodologies are not consistent. A systematic review found the 5 largest studies estimating a prevalence of 1.4-50 per 10,000 live births from various time periods including 1964-2010. Hypospadias is considered a high heterogeneous problem influenced by genetics and environmental factors including ethnic and geographic factors.

Hypospadias treatment has been referenced back to Ancient Greece. Distal hypospadias is much more common than proximal and complications generally are fewer with distal hypospadias. Treatment success with distal hypospadias ranges from 85-95%, while proximal is 75-95% but with many caveats that proximal rates are reported by some groups as much higher. Most complications occur in the first year after repair, but puberty also can also see additional problems including cordee. There are multiple types of procedures and some need to be staged over time as well.

Repair complications can include diverticulum, dehiscence, fistula, strictures, chordee, and residual hypospadias. One study reports that “[p]reserving penile length and correcting curvature was perceived as more important than a distal positioning of the meatus,” by patients.

Learning Point
The desired goals are the same for any hypospadias repair but per Long and Canning, “[o]bjectives for the reconstruction of proximal hypospadias have now been established: to enable voiding with normal velocity and laminar flow, to obtain satisfactory sexual function with a straight penis, and from a cosmetic standpoint, achievement of a slit-like meatus with a well approximated glans. Although nobody would consider sitting to void due to a sprayed urinary stream, or painful or awkward sexual function due to penile curvature to be life threatening, everyone would agree that quality of life is compromsied for many of these boys and men that suffer from said complications.”

Urethral strictures are one of the most common problems. Laminar fluid flow, or a straight, unimpeded fluid flow, is affected by fluid volume, resistance and oriface opening shape. Examples of the fluid flow changes caused by different sized lumens and different shaped openings can be easily understood by looking at piping of icing (a fluid) on confectionaries, and can be found here and here.

Questions for Further Discussion
1. How are circumcisions performed?
2. How is vesicoureteral reflux identified and treated?
3. What are other congenital abnormalities of the male genitourinary system?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Birth Defect and Penis Disorders.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Yamada G, Satoh Y, Baskin L, Cunha G. Cellular and molecular mechanisms of development of the external genitalia. Differ Res Biol Divers. 2003;71:445-460. doi:10.1046/j.1432-0436.2003.7108001.x

Wheeler APS, Morad S, Buchholz N, Knight MM. The Shape of the Urine Stream – From Biophysics to Diagnostics. PLOS ONE. 2012;7(10):e47133. doi:10.1371/journal.pone.0047133

Long CJ, Canning DA. Hypospadias: Are we as good as we think when we correct proximal hypospadias? J Pediatr Urol. 2016;12(4):196.e1-196.e5. doi:10.1016/j.jpurol.2016.05.002

Springer A, van den Heijkant M, Baumann S. Worldwide prevalence of hypospadias. J Pediatr Urol. 2016;12(3):152.e1-152.e7. doi:10.1016/j.jpurol.2015.12.002

Gulseth E, Urdal A, Andersen MA, et al. High satisfaction on genital self-perception and sexual function in healthy Norwegian male adolescents. J Pediatr Urol. 2021;17(4). doi:10.1016/j.jpurol.2021.02.015

Sinatti C, Schechter MY, Spinoit AF, Hoebeke P. Long-term outcome of urethral and genital reconstruction in hypospadias and exstrophy-epispadias complex. Curr Opin Urol. 2021;31(5):480-485. doi:10.1097/MOU.0000000000000920

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

How is Osgood-Schlatter Apophysitis Treated?

Patient Presentation
A 14-year-old female came to clinic with a history of bilateral knee pain. She noticed more pain when she was playing late winter volleyball and had added 3 times/week indoor soccer practice to get ready for the spring high school season. The pain was symmetric and would worsen during the practices and improve after stopping practice and with ice. She did not limp during the day but said that going up stairs seemed to make it worse. She denied any specific trauma but was falling on her knees with volleyball and would get tackled in soccer. She denied anything that made sounds and her knees did not “catch,” and did not have morning stiffness or swelling. The past medical history was positive for an ankle sprain more than 12 months before.

The pertinent physical exam showed a healthy female in no specific distress. Bilateral tibial tuberosities were slightly swollen and the pain was reproduced with palpation of the area and quadriceps activity. The patella did not appear misaligned at rest and through range of motion. There was no pain along the joint line and meniscal and cruciate maneuvers were normal. She did seem to have tight quadriceps and hamstring muscle groups.

The diagnosis of Osgood-Schlatter apophysitis was made. Bilateral knee plain radiographs were normal and were completed to confirm no specific osseous problems as the patient had ongoing knee trauma due to her sports.

The patient’s clinical course 2 months later, showed that she had stopped winter soccer training and had started physical therapy and cross-training. She did continue modified volleyball practices in the winter, and transitioned to soccer practices in the early spring. Her pain was markedly improved and only mildly affecting her with intensive soccer practice or competition. She continued supervised athletic training and physical therapy.

Discussion
An apopysis is a secondary ossification center located at the tendinous insertion into a bone. Site irritation is called apophysitis and several proposed causes include genetics, rapid growth, trauma (compression or traction), anatomical differences and diet. Whatever the etiological factors, it causes pain. Examples include Sever’s disease of the calcaneus.

Anterior knee pain is a common presenting problem in the pediatric age group, and the differential diagnosis is broad. Trauma, infection, tumors, and referred pain need to be considered but soft tissue problems tend to predominate if not a traumatic cause. Trauma could be overlooked by the patient though. The knee’s anatomy is complex, and this along with normal pubertal growth and biomechanics can make the diagnosis more challenging. Sports participation for recreational or competition is a common cause. Females have had increased knee problems because of increased activity and hips/legs anatomy (e.g. anterior cruciate ligament problems have rapidly increased in female athletes) in the past few years. High levels of repetitive activity where the knee is loaded such as kicking, jumping, squatting and sprinting place increased risk for overuse knee injuries. Thus common sports include baseball/softball, basketball, gymnastics, soccer and volleyball.

Osgood-Schlatter apophysitis is one of the most common apophysites (21% of teen athletes and 4.5% of non-athletes) and affects the tibial tuberosity. The cause is felt to be repetitive overload of the patella, knee and tibial structures, along with potential alignment problems, tight tissue and muscle imbalances, which may cause strain and small avulsion fractures of the tibial tuberosity. The pain commonly has a gradual onset and is worsened with specific loading knee activities such as walking up stairs, kneeling or repetitive sport activity. Pain is localized to the tibial tuberosity which may be mildly swollen, and the patellar tendon may feel thickened. Pain may be provoked with quadriceps activity even in the office. The pain usually is improved with stopping or decreasing activity. Even with treatment, pain can persist until the tibial tubercle apophysis closes or after.
While a patient’s gender, size and height are usually not modifiable risk factors for prevention, training programs and routines can be. Increased training should be done stepwise with small increases. Cross-training and multiple sport participation can also help. Training surfaces should provide safety and comfort and be exchanged on a regular basis.

Learning Point
Treatment for Osgood-Schlatter apophysitis is symptomatic and rehabilitative. About 90% of patients respond to this treatment. Symptomatically rest and icing can improve the pain. Non-steroidal anti-inflammatory drugs can also help. Rehabilitation includes physical therapy to help with biomechanics, flexibility, and strength. Although patients and families often wish to focus solely on the painful area, all areas of the lower extremity need improved flexibility and strengthening to help with muscular imbalance. Important areas do include quadriceps, iliotibial bands, hamstrings, and gluteals. Strengthening of the core musculature and overall balance is also helpful.

Patients should rest the affected joint but still can exercise. Patients can cross-train to continue cardiovascular fitness and maintain conditioning of other body areas. Good non-impact, non-loading choices would be swimming, biking or an elliptical.

Knee braces or sleeves and taping can help with short-term pain but have not been shown to be helpful long-term. The outcomes of cushioning devices such as shoe inserts are inconclusive. Steroid injections are usually not used because of the potential for patellar tendon rupture. Other injections may have some effects and more studies are being completed. Surgical interventions can be used for potential relief long-term pain if necessary but usually are not needed.

Patients can return to activity if they are pain-free, or for some patients if supervised, they can return if pain can be tolerated, is not increasing with the exercise using proper form, and does not cause other functional problems such as difficulty walking, sitting etc.

Questions for Further Discussion
1. How do you treat anterior knee pain and what are your indications for referral?
2. What is the long-term outcome of anterior cruciate ligament repair? A review can be found here
3. What causes limping? A review can be found here

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Knee Injuries and Disorders

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Slotkin S, Thome A, Ricketts C, Georgiadis A, Cruz A, Seeley M. Anterior Knee Pain in Children and Adolescents: Overview and Management. J Knee Surg. 2018;31(05):392-398. doi:10.1055/s-0038-1632376

Ladenhauf HN, Seitlinger G, Green DW. Osgood-Schlatter disease: a 2020 update of a common knee condition in children. Curr Opin Pediatr. 2020;32(1):107-112. doi:10.1097/MOP.0000000000000842

Kraus E, Rizzone K, Walker M, et al. Stress Injuries of the Knee. Clin Sports Med. 2022;41(4):707-727. doi:10.1016/j.csm.2022.05.008

Molony JT, Greenberg EM, Weaver AP, Racicot M, Merkel D, Zwolski C. Rehabilitation After Pediatric and Adolescent Knee Injuries. Clin Sports Med. 2022;41(4):687-705. doi:10.1016/j.csm.2022.05.007

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

What Causes Parotitis?

Patient Presentation
A 10-year-old male came to clinic with a 1 day history of left cheek swelling and pain. He denied any trauma and it was described as having an insidious onset with a temperature of 99.8F and some general malaise. He said his “cheek just hurt” in general and a little more with chewing but denied any actual tooth pain, ear pain, or any new sounds with chewing. He had no identified sick contacts. The past medical history showed he was fully vaccinated including for mumps at age 5.5 years. He had regular dental care.

The pertinent physical exam showed a healthy male with normal vital signs and growth parameters. He was afebrile. His left cheek was slightly more prominent and anterior to the ear was slightly swollen and warmer. The angle of the jaw was palpable and there was no specific crepitus or problems with the temporomandibular joint with movement. His ears were normal bilaterally. Intraorally, the cheek potentially was more erythematous and there was some mucopurulent fluid when the parotid glad was milked. No dental pain was elicited when each tooth was tapped and the maxillary sinuses also did not have pain with palpation. The facial nerve did not appear to be affected.

The diagnosis of parotitis was made. As the patient was fully vaccinated, the pediatrician was unsure if testing for mumps would be helpful so he contacted dentistry. They recommended to try to get a culture of the intraoral fluid from the duct if possible, and to start the patient on antibiotics for a probable bacterial cause, along with symptomatic treatment. A dental appointment was made for the following day. The patient’s clinical course showed that he improved over the following week with resolution of all symptoms. The culture grew intraoral flora without a specific predominant organism.

Discussion
The salivary glands are important for creating saliva which helps with taste, digestion, oral health, and speech. Salivary gland problems are not that common but can be painful, annoying or herald potential systemic diseases. The parotid gland is the largest salivary gland lying anterior to the ear within the cheek structures. There are 2 lobes (superficial and deep) and the facial nerve runs between them. The salivary duct opens adjacent to the upper second molar. Infections are the most common cause of parotitis with mumps being the most commonly associated infection especially in unimmunized patients.

Patients with parotitis present with pain or discomfort, swelling, and potentially systemic fever and/or localized temperature elevation. The jaw angle may not be discernable because of the swelling and there may be mucopurulent discharge from the salivary duct. Special attention should be paid to the facial nerve innervation and adjacent structures when examining the patient.

Patients are usually treated supportively for infectious causes with analgesics, sialoguges, parotid gland massage, oral hygiene practices and if appropriate, antibiotics. Consultations with dentists or otorhinolaryngologists may assist primary care providers with outpatient management and is helpful if the diagnosis is not clear.

Learning Point
The differential diagnosis for parotitis and other salivary gland disorders includes:

  • Infections
    • Viral
      • Mumps
      • Adenovirus
      • Coxsackie virus
      • Cytomegalovirus
      • Epstein-Barr virus
      • Influenza
      • Parainfluenza
      • Parvovirus B-19
    • Bacterial
      • Staphylococcus aureus
      • Streptococcus
      • Mycobacterium tuberculosis
      • Mycobacterium avium
    • Other
      • Toxoplasmosis
      • Abscess
  • Inflammatory
    • Sjorgren’s syndrome
    • Sarcoidosis
    • Systemic erythematosus lupus
  • Trauma
    • Hematoma
    • Foreign body
  • Tumor – rare
    • Benign
    • Malignant
    • Other – bony tumors, vascular malformations
  • Other
    • Sialolithiasis – salivary stones
    • Anatomic abnormalities
    • Cystic fibrosis
    • Juvenile recurrent parotitis – a non-obstructive, non-suppurative parotitis
    • Metabolic disorders
  • Problems with adjacent structures may look like parotitis
    • Dental
    • Dermatological
    • Cold panniculitis

Questions for Further Discussion
1. Where are all the salivary glands located?
2. What causes dental caries?
3. What causes facial pain? A review can be found here
4. At what age do teeth exfoliate? A review can be found here

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Salivary Gland Disorders and Mumps.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Ellies M, Laskawi R. Diseases of the salivary glands in infants and adolescents. Head Face Med. 2010;6:1. doi:10.1186/1746-160X-6-1

Taji SS, Savage N, Holcombe T, Khan F, Seow WK. Congenital Aplasia of the Major Salivary Glands: Literature Review and Case Report. Pediatr Dent. 2011;33(2):113-118.

Jablenska L, Trinidade A, Meranagri V, Kothari P. Salivary gland pathology in the paediatric population: implications for management and presentation of a rare case. J Laryngol Otol. 2014;128(1):104-106. doi:10.1017/S0022215113003514

Inarejos Clemente EJ, Navallas M, Tolend M, Sunol Capella M, Rubio-Palau J, Albert Cazalla A, Rebollo Polo M. Imaging Evaluation of Pediatric Parotid Gland Abnormalities. RadioGraphics. Accessed January 10, 2023. https://pubs.rsna.org/doi/10.1148/rg.2018170011

Gellrich D, Bichler M, Reichel CA, Schrotzlmair F, Zengel P. Salivary Gland Disorders in Children and Adolescents: A 15-year Experience. Int Arch Otorhinolaryngol. 2020;24(1):e31-e37. doi:10.1055/s-0039-1697993

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

What Immunity Do Breast Milk Antibodies Confer?

Patient Presentation
A 6-month-old male came to clinic for his health supervision visit. He had been relatively well except for one ear infection and two upper respiratory infections since starting childcare. His mother was concerned about also giving influenza and COVID vaccines in addition to his regular vaccines.

The pertinent physical exam showed a healthy, smiling infant with growth parameters at the 10% for height and 50% for weight and head circumference. He had some rhinorrhea, and his ears were normal as was the rest of his examination.

The diagnosis of a healthy infant with rhinorrhea was made. The mother stated that as she was breastfeeding that was all of the immunity that the baby needed for influenza and COVID. The pediatrician noted that breast milk was really good at helping prevent some diseases but that the baby needed his own immunity from his own vaccines. “I think it is great that you continue to breastfeed, and the milk does give him some immunity, but it isn’t his own and won’t last long term. I know that his brother is coming in next week. I have some good information about the vaccines and immunity that I can give you. Then you and Dad can talk and we can have another discussion next week,” she offered. The patient’s clinical course revealed that the mother was willing to do influenza vaccine the following week for both children but still declined the COVID vaccine as she wanted to do more research into it.

Discussion
Breast milk (BM) has many benefits including its primary role providing appropriate nutrition for newborns and infants, which includes making those nutrients more bioavailable. While BM does not provide all of the nutritional needs over the entire year or two of breastfeeding, complimentary foods are important for furnishing additional nutritional needs along with helping development of appropriate taste and texture acceptance, along with oral-motor skills. Complimentary foods are recommended by the American Academy of Pediatrics when the infant is developmentally ready which is commonly after 6 months of life. Breastfeeding and BM are also associated with other decreased health risks such as diabetes, obesity, allergies, and sudden infant death syndrome. It also helps prevent infections especially in the gastrointestinal tract directly but others more indirectly.

A review about how much a newborn should eat, can be found here.

Learning Point
Maternal antibodies pass to an infant in two basic ways:

  • Transplacental transfer
    • Mainly IgG passing directly into the fetus and newborn’s serum
    • Half-life is 21 days so this immunity wanes over the first year
    • Provides systematic immunity
    • Protects against a variety of infections including influenza, pertussis, and tetanus
    • Transplacental IgE may play a role in allergic response
  • BM antibodies
    • Is secretory IgA (the main antibody with a half-life of days to a couple of weeks), IgM and some IgG
    • Provides important mucosal immunity, but does not provide systemic immunity as mucosal surfaces including the gut close very soon after birth
    • “…BM antibodies [are important] in the protection of newborns against neonatal infections, seeding the gut microbiome, and training tolerance toward mucosal antigens…”
    • The precise role as to how the BM antibodies assist in these roles is not totally understood but appears to be through a complex variety of ways including passive immunity within the gastrointestinal tract

Data for Coronavirus 19 (COVID-19) is being generated. There is data that supports high rates of IgA and IgG production in BM after maternal COVID-19 immunization including the initial dose. Data regarding its persistence over time within BM, especially after full maternal immunization, and its actual functional impact on disease prevention in infants still remain open questions.

As maternal transplacental and BM antibodies wane over time, infants should receive their own immunizations to retain their own immunity.

Questions for Further Discussion
1. What are contraindications to breastfeeding? A review can be found here
2. How much breast milk does a lactating woman make? A review can be found here
3. What are the advantages of breastfeeding for the mother?
4. What advice do you give to mothers’ partners to help support them breastfeeding?

Related Cases

To Learn More
To view pediatric review articles on thse topics from the past year check PubMed – Breast Milk and
PubMed – Immunization.
Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Breastfeeding, Vaccines and Childhood Vaccines.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Atyeo C, Alter G. The multifaceted roles of breast milk antibodies. Cell. 2021;184(6). doi:10.1016/j.cell.2021.02.031

Perl SH, Uzan-Yulzari A, Klainer H, et al. SARS-CoV-2-Specific Antibodies in Breast Milk After COVID-19 Vaccination of Breastfeeding Women. JAMA. 2021;325(19):2013. doi:10.1001/jama.2021.5782

Kalbermatter C, Fernandez Trigo N, Christensen S, Ganal-Vonarburg SC. Maternal Microbiota, Early Life Colonization and Breast Milk Drive Immune Development in the Newborn. Front Immunol. 2021;12:683022. doi:10.3389/fimmu.2021.683022

Whited N, Cervantes J. Antibodies Against SARS-CoV-2 in Human Breast Milk After Vaccination: A Systematic Review and Meta-Analysis. Breastfeed Med Off J Acad Breastfeed Med. 2022;17(6):475-483. doi:10.1089/bfm.2021.0353

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa