Diphallia

Also called Diphallasparatus, penile duplication (PD) or diphallic terata is a rare developmental disorder where a male infant is born with two or more penises. The first reported case was in Bologna; Italy (1609), by Johannes Jacob Wecker, a Swiss female physician and philosopher.

It occurs in 1 out of every 5.5 million males worldwide, and so far only about 100 cases of diphallia have been recorded.

 

Diphallia, Picture credit mamamia.

Diphallia is usually accompanied by other congenital abnormalities like Vertebral (spinal bifida), renal (exstrophy of the bladder, bladder/urethra duplication), hindgut (exstrophy of the cloaca), anorectal (colon and rectosigmoid duplication), skeletal (pubic symphysis diastasis) skeletal muscle abnormalities (ventral hernia) heart muscles abnormalities, etc.

Risk factors/Causes

It is thought diphallia occurs in the fetus between the 23^rd to 35^th day  (3rd to 6th week) of gestation(pregnancy) when an injury; chemical stress, or malfunctioning “homeobox genes” hamper proper function of the caudal cell mass of the foetal mesoderm as the urogenital sinus separates from the genital tubercle and rectum to form the penis.

The embryologic explanation of diphallia is quite obscure, because at no time during normal development is the genital tubercle a paired structure. Reptiles such as snakes and lizards possess a double penis. Moreover, diphallia could represent a teratoid structure or a form of incomplete twinning. Still, none of these explanations is adequate and the wide spectrum of the anomalies cannot be explained by a single hypothesis.

Signs and symptoms

It is believed that no cases are identical as signs and symptoms vary for each patient. It varies from a small accessory penis or duplication of the glans to complete penile duplication.

Generally, it is characterized with two or more penis, both are situated side by side and has equal size, urine passes through both penis, some patient in one penis only, other patient neither of the penis. Some patient is sterile or incapable to produce semen, but the penis will erect if stimulated.

The duplication may be orthotopic or ectopic. Division of the penis may be sagittal or frontal and symmetric or asymmetric, in shape and size.

The urethra shows a range of variations, from functioning double urethras to complete absence of the urethra in each penis. The majority have a single corpus cavernosum in each organ. The meatus may be normal, hypospadias, or epispadia, and the scrotum may be normal or bifid. The testes are normal, athropic, or undescended.

Types

Various classifications for diphallia exist. One classifies it into four types while a recent classification is into two

Classifying Diphallia into four types:

• ·         Diphallia of the glans alone,
• ·         bifid diphallia,
• ·         Complete diphallia.
• ·         Pseudodiphallia.

Classifying diphallia into two types (widely accepted):

• ·         True diphallia; partial duplication or complete duplication.
• ·         Bifid phallus; partial duplication or complete duplication.

True complete diphallia refers to complete penile duplication, each with two corpora cavernosa (body of the penis) and a corpus spongiosum (spongy tissue surrounding the male urethra within the penis). Here both penises are capable of simultaneous erection and ejaculation.

True Partial diphallia; here the duplicate penis is smaller or rudimentary, it corresponds to the term “pseudodiphallia,”. Here the rudimentary or smaller penis may not be capable of erection and ejaculation)

Bifid phallus; when only one corpus cavernosum (body of the penis) is present in each penis. Normal erection in both penises occurs here).

         Complete bifid phallus; when the degree of separation is complete to the base of the shaft

         Partial bifid phallus; when the separation is just to the glans (Tip/cap of the penis).

Diagnosis

Ultrasound is used to help confirm the diagnosis. It detects the number of corpora cavernosum or corpora spongiosum and their accompanying abnormalities. Improved interpretation of anatomical structures has been made possible with the advent of magnetic resonance. Imaging also help in decision making during treatment.

Treatment

Several factors are considered during the treatment of diphallia, these are; medical, ethical and aesthetic factors.

Treatment of diphallia depends on the type of diphallia and the associated anomalies. During treatment of diphallia, it is necessary to individualise each case and also aim at preserving or achieving erectile function, urinary continence, urinary stream and cosmesis.

Surgical Excision of the duplicated noncommunicating penis is reported in many bodies of literature. Penis reconstruction by joining the corporal bodies in each penis in a patient with true complete diphallia has also been reported in some literatures.

Complications

Congenital defects, sterility, erectile dysfunctions, prostatitis, etc.

Prognosis

Infants born with diphallia and its related conditions have a higher death rate from various infections associated with their more complex renal or colorectal systems.

References

R. Mukunda, P. S. Bendre, R. G. Redkar, and S. Hambarde, “Diphallus with anorectal malformation-case report,” Journal of Pediatric Surgery, vol. 45, no. 3, pp. 632–634, 2010

V. C. Varghese and T. U. Sukumaran, “Penile duplication,” Indian Pediatrics, vol. 41, no. 11, p. 1166, 2004

H. S. Bhat, S. Sukumar, T. B. Nair, and C. S. M. Saheed, “Successful surgical correction of true diphallia, scrotal duplication, and associated hypospadias,” Journal of Pediatric Surgery, vol. 41, no. 10, pp. e13–e14, 2006.

M. M. Aboodi and H. A. Al-Jadeed, “Accessory pseudophallus with accessory pseudoscrotum detected during antenatal sonographic scanning,” Journal of Ultrasound in Medicine, vol. 24, no. 8, pp. 1163–1166, 2005.

A. Mirshemirani, F. Roshanzamir, S. Shayeghi, and L. Mohajerzadeh, “Diphallus with imperforate anus and complete duplication of recto-sigmoid colon and lower urinary tract,” Iran Journal of Pediatric, vol. 20, no. 2, pp. 229–232, 2010

S. Priyadarshi, “Diphallus with ectopic bowel segment: a case report,” Pediatric Surgery International, vol. 21, no. 8, pp. 681–683, 2005.

K. Gyftopoulos, K. P. Wolffenbuttel, and R. J. M. Nijman, “Clinical and embryologic aspects of penile duplication and associated anomalies,” Urology, vol. 60, no. 4, pp. 675–679, 2002.

S. Acimi, “Complete diphallia,” Scandinavian Journal of Urology and Nephrology, vol. 38, no. 5, pp. 446–447, 2004.

M. E. Torres, P. J. C. Sanchez, T. A. Aragon, T. V. Camacho, and G. A. Colorado, “Diphalia,” Revista Mexicana de Urología, vol. 69, no. 1, pp. 32–35, 2009.

Wikipedia “Diphallia” Wikipedia 2016.

M. C. de Oliveira, R. Ramires, J. Soares, A. P. Carvalho, and F. Marcelo, “Surgical treatment of penile duplication,” Journal of Pediatric Urology, vol. 6, no. 3, pp. 257-e1–257-e3, 2010

P. Tirtayasa, R. Prasetyo, and A. Rodjani “Diphallia with Associated Anomalies: A Case Report and Literature Review,” Case Reports in Urology Vol. 2013, Article ID 192960, 2013.

Illness Anxiety Disorder Or Hypochondriasis

Illness anxiety disorder (IAD) formerly called Hypochondriasis, Hypochondria, Healthy Anxiety, Illness Phobia or Somatic preoccupation is an overwhelming fear that you have a serious disease based on the misinterpretation of one or more normal bodily signs, even when Doctors can find no evidence of the illness. The disorder is not about the presence or absence of illness, but the psychological reaction.

The term Hypochondrium can be traced back to 400BC when it was used by Hippocrates to describe a region of the abdomen. Back then, it was believed that Hypochondriasis was due to malfunctioning of organs in that region of the abdomen.

This condition is seen in 4-20% of the general population, and in 45% of people with severe psychiatric illnesses. It occurs more in women than men. It occurs at any age, but commonly seen between 36 to 57 years.

 

 picture credit; Anxiety org.

Features

Symptoms of Illness anxiety disorder are not under voluntary control and cannot be explained by physiological causes. 

They include;

Excessive fear, preoccupation or worry over having a serious illness.

The fear persists despite appropriate medical evaluation and reassurance.

The belief is not of delusional intensity and is not restricted to a concern about appearance.

The fear causes clinically significant distress or impairment.

The preoccupation lasts for at least 6 months.

The preoccupation is not explained better by another mood, anxiety, or somatoform disorder.

Physical symptoms are not present or if present, only mild. If another illness is present, or there is a high risk for developing an illness, the person’s concern is out of proportion.

High level of anxiety and alarm over personal health status.

Excessive health-related behaviors (e.g., repeatedly checking body for signs of illness) or shows abnormal avoidance (e.g., avoiding doctors’ appointments and hospitals).

Frequently making medical appointments for reassurance — or, avoiding medical care for fear of being diagnosed with a serious illness.

Avoiding people, places or activities for fear of health risks.

Worrying excessively about a specific medical condition or your risk of developing a medical condition because it runs in the family.

Having so much distress about possible illnesses that it become hard for you to function.

Constantly talking about health and possible illnesses.

Frequently searching the Internet for causes of symptoms or possible illnesses.

The absence of physical findings, after series of medical examinations, supports the diagnosis of hypochondriasis.

Risk Factors/ Causes

Overprotective parents or caregivers,

Family history of hypochondriasis,

Belief,

Excessive health related Internet use,

Mental disorders (Psychosomatic disorders and obsessive compulsive disorders),

Epidemics, pandemics and statistics of outbreaks gives Hypochondriacs an illusion that they have the disease,

Major Stress,

Child abuse/illness,

Neurochemical deficit,

Pathogenesis

Some theories have postulated that neurochemical deficits are responsible for this disorder, such deficits may explain why symptoms overlap, why the disorders are commonly comorbid, and why effective treatments parallel one another (eg, selective serotonin reuptake inhibitors [SSRIs]).

Neurochemicals Implicated in this condition include neurotrophin 3 (NT-3), serotonin (5-HT), etc.

Diagnosis

There is no laboratory or radiologic investigation for the diagnosis of Hypochondriasis or Illness Anxiety Disorder.

Thus diagnosis can only be done can be done using the ICD-10 or the DSM-IV criteria.

The DSM-IV defines hypochondriasis according to the following criteria:

    A. Preoccupation with fears of having, or the idea that one has, a serious disease based on the person's misinterpretation of bodily symptoms.

    B. The preoccupation persists despite appropriate medical evaluation and reassurance.

    C. The belief in Criterion A is not of delusional intensity (as in Delusional Disorder, Somatic Type) and is not restricted to a circumscribed concern about appearance (as in Body Dysmorphic Disorder).

    D. The preoccupation causes clinically significant distress or impairment in social, occupational, or other important areas of functioning.

    E. The duration of the disturbance is at least 6 months.

    F. The preoccupation is not better accounted for by Generalized Anxiety Disorder, Obsessive-Compulsive Disorder, Panic Disorder, a Major Depressive Episode, Separation Anxiety, or another Somatoform Disorder.

The newly published DSM-IV replaces the diagnosis of hypochondriasis with "illness anxiety disorder".

The ICD-10 defines hypochondriasis as follows:

    A. Either one of the following:

            A persistent belief, of at least six months' duration, of the presence of a maximum of two serious physical diseases (of which at least one must be specifically named by the patient).

            A persistent preoccupation with a presumed deformity or disfigurement (body dysmorphic disorder).

    B. Preoccupation with the belief and the symptoms causes persistent distress or interference with personal functioning in daily living, and leads the patient to seek medical treatment or investigations (or equivalent help from local healers).

    C. Persistent refusal to accept medical advice that there is no adequate physical cause for the symptoms or physical abnormality, except for short periods of up to a few weeks at a time immediately after or during medical investigations.

    D. Most commonly used exclusion criteria: not occurring only during any of the schizophrenia and related disorders (F20-F29, particularly F22) or any of the mood disorders (F30-F39).

The Illness Attitude Scale (29 items, 15 min, English only) is used for detection and to assess severity.

The Whitely Index of Hypochondriasis (14 items, 5 min, >14 languages) is used for detection, for rating severity, and for measuring change per interventions.

Differential Diagnosis

Other conditions that should be considered when diagnosing Illness Anxiety Disorder or Hypochondriasis are;

Schizophrenia,

Somatic Symptom Disorders,

Body Dysmorphic Disorders,

Conversion Disorders,

Personality Disorders

Depression,

Anxiety Disorders,

Delusional Disorders.

In this case, the following screening tools are useful in distinguishing Illness Anxiety Disorder from the rest;  

    The Health Anxiety Inventory (HAI) (long version; short version of 14 items, 5 min) reliably distinguishes patients with hypochondriasis from patients with anxiety disorders or healthy controls.

    The Somatoform Disorders Symptom Checklist (65 yes-or-no items, 20 min, >5 languages) screens for hypochondriasis, somatization disorder, BDD, and others.

Treatment

Depending on the severity, patients with illness anxiety disorder may require hospital admission, but mild cases may not.

The following methods are recommended for the management of Hypochondriasis;

Education & Reassurance; here the patient is made to understand the manifestations of hypochondriasis and reassurance is given consistently.

Psychotherapy; using Cognitive behavioral therapy.

Psychosurgery in severe cases,

Lifestyle; Patients with illness anxiety disorders/ hypochondriasis are advised to avoid alcohol, caffeine and nicotine. Regular exercise and quality sleep is also recommended here.

Drugs; this is only recommended in addition to the above methods to prevent complications or reduce symptoms in some cases. The drugs used include; Benzodiazepines (Alprazolam), Antidepressant (Sertraline, Paroxetine, Fluoxetine, Venlafaxine, clomipramine, Imipramine, Citalopram, etc.), Antipsychotics (Pimozide, Risperidone, Olanzapine) or Beta adrenergic receptor blocker (Propranolol). Multivitamin supplements (Vitamin A, B, C, E and D.)

Complications

Social complications; Family and marital issues, Unemployment, bankruptcy from overuse of expensive laboratory or medical facilities,

Complications from investigation procedures,

Suicidal thoughts/ Suicide

Disability

Costly medical bills

Complications from testing procedures

Disability and unemployment

Marital or family problems

Prognosis

It has a good prognosis, especially in patients with high economic status. Sometimes the symptoms last from months to years with long quiescent periods

1/3^rd of the patients improve significantly.

Most children with hypochondriasis recover before adolescence or by early adulthood

References

Ehrlich, S., 2014. Hypochondriasis. University Of Maryland Medical center.

Mayo Clinic, 2015. Illness Anxiety Disorder. Mayo Clinic.

Neziroglu, F., 1998. Hypochondriasis: A Fresh Outlook on Treatment. Psychiatric times.

Olatunji, B., Deacon, B. & Abramowitz, J., 2009. Is hypochondriasis an anxiety disorder? The British Journal of Psychiatry.

Warwick, H., 1998. Cognitive therapy in the treatment of hypochondriasis. Advances in Psychiatric Treatment, 4, pp.285–295.

Xiong, G., 2013. Hypochondriasis. Medscape.